This issue contains the abstracts of the 8th European Neuro-Ophthalmology Society Meeting (EUNOS) held from May 26th to May 29th 2007 in Istanbul. The Editorial Board had decided to publish the abstracts in order to present the wealth of neuro-ophthalmological topics dealt with in Europe, especially since the European Community has extended to countries in East Europe, and since it is much easier for colleagues from former socialist countries and the former Soviet Union to join the meetings and to present their findings and ideas. This issue shall help to strengthen contacts between neuro-ophthalmologists world-wide, especially with those colleagues who did not have many chances to join the network of clinical neuro-ophthalmologists when the iron curtain was sill in place.
The meeting is devoted to the functional as well as to the structural aspects of neuro-ophthalmology, to paediatric neuro-ophthalmology, and important but rarely treated major topic as well as to the development in treating common diseases such as optic neuritis. Additionally, the relation to neighbouring fields shall be strengthened by the emphasis on functional imaging, ocular motor disorders and genetics as well as rehabilitation.
In particular, the key note lectures by
Creig Hoyt, “Brain Injury and the Eye in Children”
Tulay Kansu, “Visual and Neurological aspects of Behçet's Disease” Jonathan Trobe, “How to Write a Medical Journal Article that is Going to be Rejected” and
Eberhart Zrenner, “On the Restitution of Visual Percepts in Blind Patients by Subretinal Microelectrodes”
will help strengthen the field, point out particular problems in neuro-ophthalmology and to improve communication. The Editorial Board has asked the Key Note Lecturers to provide an article in one of the next issues of neuro-ophthalmology on their topic, and certainly all presenters are invited to submit their papers to neuro-ophthalmology.
A most interesting programme has been assembled with altogether 30 abstracts of oral presentations, and 162 abstracts of posters and 8 symposia. It is very gratifying to see how quickly the neuro-ophthalmological community is growing and we are especially happy that many speakers from overseas have joined the meeting for lively discussions.
Istanbul is an area that is predestined to build bridges between East and West. It is officially in both Europe and Asia. Its history leans strongly to Christianity and to the Muslim world according to the historical order, and its numerous traditions of cultural diversity provide a special congress atmosphere that favours the interpersonal exchange of ideas and perspectives and practical experience. This spirit shall be carried forward by this particular issue to those who are not able to attend the meeting and we hope that it will be useful to them to link into the new developments arising in European neuro-ophthalmology.
We are especially grateful to the local scientific committee and to the international scientific committee listed on the following page, namely to Tulay Kansu and to Robert McFadzean, and especially to those who have helped organize a meeting that is marked by warm hospitality, high quality scientific exchange and friendship.
Istanbul, March 1, 2007
Pinar Aydin O'Dwyer
Congress Chairperson
Eberhart Zrenner
Chief Editor
Section I: Oral Presentation
I.1. Imaging-Oral Presentations
I.1.1 (o2)
Patterns in Neuroimaging misreading of neuro-ophthalmic patient
Ruth Huna-Baron1 and Mathy Bakon2
1 Goldschleger Eye Institute, 2Department of Imaging, Sheba Medical Center Tel-Hashomer, Israel
Objective To identify patterns in cases where the neuroimaging findings have been missed or misread.
Patients and Methods All neuroimaging studies copies of consecutive patients, from 1994–2003, where the findings and the report did not correlate according to the authors have been collected. Only studies where the findings are the etiology of the complaints or clinical signs have been included, meaning that missed incidentaloma's have been excluded. Details concerning age, gender, medical history, symptoms, clinical findings, referring physician, report, further neuroimaging studies, delay of diagnosis, location of lesion, and final diagnosis and treatment were documented. Copies of scans and reports were saved.
Results Twenty patients with missed or misinterpreted findings in their MRI and CT scans were evaluated. The presenting symptom was loss of vision in 6 (30%) cases. Five patients complained about diplopia (25%), 5 experienced headache or eye pain (25%), 2 (10%) ptosis and 2 were asymptomatic.
Most of the cases (55%) were referred by ophthalmologists, 6 (30%) by neurologists, 1 by a neuro-ophtalmologists and 1 by an urologist and another by a pediatrician. Twelve (60%) patients were referred to CT without contrast, 1 due to allergy, 6 to CT with contrast and 4 to MRI. Among the most common missed findings were sellar and parasellar lesions (50%). The pathology was not detected in 17 cases and misdiagnosed on 3 cases. Misreading caused delay or incorrect treatment in 15 patients.
Conclusions Most of the patients were referred to CT with out contrast, which is usually ordered as the initial imaging study of the CNS because it is widely available and it is less expensive. As from the results the majority of patients have been referred by an ophthalmologist or pediatrician and urologist who are not updated about neuroimaging technique and are not familiar with brain scans. They relay on the reports. However, they should be aware of the importance of the referral letter that should contain detailed history, findings and specify the diagnostic question. We should encourage them that if the results do not correlate with their clinical judgment the neuroradiologist should be contacted or refer the patient to a neuro-ophthalmologist.
I.1.2 (o3)
Stereotactic radiotherapy for inoperable anterior visual pathway related neoplasia. Late visual outcome in a patient serie
Anne Wiencke, Jens Kiilgaard, Marianne Juhler, Hans C Fledelius
Departments of Ophthalmology and Neurosurgery, Rigshospitalet, Copenhagen University Hospital, Denmark
Purpose To report and discuss visual parameters in a series of chiasm-related tumors after a follow-up of at least 18 months (n=49) with regard to (a) the effect of SRT on the basic disorder, and (b) the anterior optic pathway's tolerance of the therapy.
Methods As main parameters to compare visual acuity and visual field findings prior to the radiotherapy and after an observation time of 18–72 months. Kinetic Goldmann perimetry and computerized static evaluation(Octopus)were scheduled for the current visual field assessments, but individual deviations from protocol had to be accepted, in particular where patient co-operation was poor. The basic main diagnoses were meningioma, pituitary adenoma and craniopharyngioma as histologically verified at previous neurosurgical intervention.
Results A total of 43 patients had no significant change in ophthalmic status, whereas 6 further lost vision. In the 4/6 such patients without evidence of tumor progression, sequelae to the SRT proper are to be considered.
Conclusions Generally, the SRT therapy proved effective and well tolerated despite the close anatomical proximity of focus of radiotherapy to the anterior visual pathway. The patients with pre-existing severe visual loss appeared at greater risk of (further) losing vision due to SRT.
I.1.3 (o9)
A clinically with a visual field test not detectable subjective diffuse diminished light sensation may be due a coagulum in cerebral vessels.
Hilmi Or
Private Practice, Istanbul, Turkey
Aim To show that clinically with ophthalmological examination and visual field testing not detectable subjective diffuse diminished light sensations should be evaluated for cerebral problems.
Material and Methods A patient (a 26-years-old woman) who had been followed up for 14 years for other reasons came with the symptom of diffuse diminished light sensation in both eyes. She had a successful amblyopia therapy in her left eye in childhood. She had contact lenses due to high anisometropia of +3.5 D. Her visual acuity was 1.0 in both eyes. With a high c/d ratio in both eyes and having a intraocular pressure of 20 mmHg she was a glaucoma suspect patient. Her visual field examination was made since years regularly. A new ophthalmological examination showed normal findings and a new visual field test revealed no scotomas. The patient was sent to neurological examination. The cranial MR ordered by neurology revealed a coagulum in the vessels of sigmoid fossa. After 10 days with a started Coumadin therapy by neurology, the subjective diffuse diminished light sensation in both eyes subsided.
Results A clinically with ophthalmological examination and visual field testing not detectable subjective diffuse diminished light sensation may be due a coagulum in cerebral vessels.
Conclusion Light sensation diminishing problem should be clinically evaluated for cerebral reasons after the possible ocular etiologies eliminated.
I.1.4 (o15)
The Quantitative Evaluation of Optic Disc Morphology in Patients with Papilledema of Pseudotumor Cerebri and Pseudopapilledema of Optic Nerve Head Drusen by Heidelberg Retina Tomograph
Şansal Gedik1, Ahmet Akman1, Leyla Erkanli˙1, Sibel Oto1, Erdal Kara2, Yonca A. Akova1
1Başkent University Faculty of Medicine, Department of Ophthalmology, Ankara, Turkey; 2Anadolu University Faculty of Open Education, Ankara, Turkey
Objective One of the main diagnostic difficulties of neuroophthalmologic disorders is differentiation of papilledema (PE) from pseudopapilledema (PPE). In this study, we evaluated optic disc topography and visual field parameters of patients with PE and PPE and investigated the role of Heidelberg Retinal Tomography (HRT) in differential diagnosis.
Patients and Method The study consisted of 15 PPE patients with visible optic nerve head drusen (ONHD; group 1), and 10 patients with PE due to pseudotumor cerebri (PTC; group 2). All patients in group I and II underwent the same ophthalmologic examination.
The Humphrey Field Analyzer (HFA; Zeiss Humphrey Systems, CA) central 30-2, SITA standard program was used for perimetric examination. OD morphology of each patient was assessed by HRT III by the same experienced technician. Data including the patient's age and sex, global indices for HVA (such as the mean deviation [MD] and the pattern of standard deviation [PSD], fixation loss, false-positive and false-negative errors, the duration of the test), stereometric parameters for HRT (linear cup/disc ratio, rim area, rim volume, mean retinal nerve fiber layer (RNFL) thickness) were recorded. Only the right eye parameters have been taken into consideration for statistical analysis.
Results Fifteen patients, with a mean age of 34.78 ± 19.39 years (range: 16–70) and 10 patients with a mean age of 34.44 ± 10.88 years (range: 22–53) were included in group I and II, respectively. The HFA results achieved from the patients are as following: The mean MD value of −4.19 dB (SD ± 2.83) and −8.2 dB (SD ± 7.9), the mean PSD value of 3.59 dB (SD ± 1.7) and 5.56 dB (SD ± 4.3) for groups I and II, respectively. The mean linear cup/disc ratio, rim area, rim volume and RNFL thickness were 0.9/0.35, 1.90mm2/1.17 mm2, 0.70 mm3/0.29 mm3, 0.26 mm/0.27 for groups I/II, respectively.
Conclusion Heidelberg retinal tomography (HRT) is a non-invasive confocal scanning laser ophtalmoscope that measures the topography of the optic nerve head and peripapillary retina, in vivo. This method can be useful in differentiation of PPE of ONHD from PE of PTC.
I.1.5 (o17)
Functional MRI-Perimetry: a Novel Technique for Simultaneous Neural and Behavioral Mapping of the Visual Fiel
G. Szatmáry1, L.R. Kozák2,3, Z. Vidnyánszky2
1Department of Ophthalmology, Semmelweis University; 2MR Research Center, Szentágothai Knowledge Center; 3Institute for Psychology, Hungarian Academy of Sciences, Neurobionics Research Group, Hungarian Academy of Sciences, Péter Pázmány Catholic University and Semmelweis University
Background Visual field defect is one of the most important and sensitive sign of a neuro-ophthalmic disorder. An important and presently unresolved question concerns the plastic brain processes that are triggered by these diseases. Functional MRI is a non-invasive technique for in vivo evaluation of neural activity within the central nervous system. However, presently there are only few methods developed for the assessment of visual field defects by fMRI.
Objective Our aim was to develop and validate a functional MRI (fMRI) perimetry method that would allow simultaneous mapping of perceptual and neural visual field deficits, and assessment of neural plasticity processes underlying spontaneous and visual restoration (medication, visual rehabilitation) induced functional recovery.
Methods Longitudinal, interventional, case-controlled pilot study of a patient with optic neuritis evaluated with fMRI-perimetry and correlated with the result of 24-2 SITA Standard automated perimetry.
Results Performance on fMRI-perimetry closely corresponds to the performance indices obtained by standard automated perimetry (SAP). We found that performance on fMRI-perimetry: blood oxygen level dependent (BOLD) activation patterns, closely correlates with pattern deviation (PD) performance as assessed by static automated perimetry. In addition, BOLD activation patterns were found to be different for simulated and pathological scotomas. Moreover, we observed close correspondence between decreased overall retinotopic activity with distance from the fovea and performance indices obtained by both automated and fMRI-perimetry techniques.
Conclusion FMRI-perimetry is a promising novel technique for the voxel-based quantification of the extent of neural visual field defects, and may even be more sensitive than SAP for the assessment of residual activity behind absolute scotomas. This refined fMRI protocol may be incorporated in future clinical trials as a more sensitive outcome measure to assess drug effects and correlate it with clinical functional status and disability.
I.1.6 (o24)
An Investigation of Ganglion Cell Loss in Retrogeniculate Hemianopia using Optical Coherence Tomography (OCT)
Panitha Jindahra1, Aviva Petrie2, Gordon Plant1
1The National Hospital for Neurology and Neurosurgery, Queen square, London; 2Biostatistics Unit, UCL Eastman Dental Institute, Grays Inn Road, London WC1X 8LD
Background Trans-synaptic retrograde degeneration (TRD) of retinal ganglion cells following retrogeniculate visual pathway lesions were observed in animal studies and congenital homonymous hemianopia (CHH) in human. But the studies in acquired lesions (AHH) have been controversial.
Aim To study the peripapillary retinal nerve fibre layer thickness (RNFT) in patients with homonymous hemianopia.
Method We measured RNFT in microns by using OCT. Four quadrants in each optic disc were performed, namely nasal, temporal, superior, and inferior, each divided into 3 sectors. There were 12 cases of AHH (duration 4 months-67 years, age 30–76 years), 7 cases of CHH (age 17–59 years), and 11 controls (age 23–56).
Results In eyes with temporal hemianopia, there were significant differences in the mean RNFT between AHH and controls (−22.86 microns, p < 0.001), and between CHH and controls (−28.85 microns, p < 0.001), but not between AHH and CHH (+5.97 microns, p=0.03).
In the eyes with nasal hemianopia, there were significant differences in the mean RNFT between AHH and controls (−19.67 microns, p < 0.001), between CHH and controls (−28.37 microns, p < 0.001), and between AHH and CHH (+8.71 microns, p=0.002).
Mean RNFT of crossed/uncrossed fibre defect eyes in controls, is near to one (range 0.94–1.09) in each sector. In both AHH and CHH, the ratios were less than one in the nasal quadrant, superonasal sector, inferonasal sector, and mid-temporal sector. The ratios were greater than one in the remaining sectors. These findings demonstrated a band atrophy pattern in the crossed fiber defect eyes. There were significant differences in the mean RNFT ratio between CHH and controls in sectors of every quadrant, but a few sectors between AHH and controls, and between AHH and CHH.
Conclusion This study revealed that there was thinning of RNFT both in acquired and congenital homonymous hemianopia, with a greater degree in CHH. The atrophy was in the areas corresponding to the damage in the occipital lesions, which can be explained by TRD. This study is, therefore, the first direct confirmation of TRD in acquired damage to the human visual pathway.
I.2. Motility and Pupils—Oral Presentations
I.2.1 (o8)
The Pupillographic Sleepiness Test in patients with multiple sclerosis with and without fatigu
J. Vilisaar, G. Niepel, R. Langley, C.M. Bradshaw, E. Szabadi, C.S. Constantinescu
University of Nottingham
Introduction The pupillographic sleepiness test (PST) is a useful measure of the level of sleepiness, as excessive sleepiness has been associated with increased, quantifiable fluctuations in pupil diameter. Fatigue is a common symptom affecting 80% of multiple sclerosis (MS) patients. No objective measures of fatigue currently exist. Excessive sleepiness was also shown to correlate with fatigue severity scores (FSS) in MS patients, and drugs used in diseases with excessive sleepiness may also be useful in the treatment of MS-related fatigue.
Methods We compared 14 MS patients with significant fatigue (FSS > 5) with 6 MS patients with no or low fatigue (FSS < 2.9). PST was performed, recording pupil diameter oscillations in the dark over a period of 11 min. Measures derived from PST included total power of fluctuations (TPF) and pupillary unrest index (PUI). Other objective measures of arousal included the critical flicker fusion frequency (CFFF) and choice reaction time (CRT). Subjective level of alertness was measured by the Epworth Sleepiness Scale (ESS) and a battery of visual analogue scales (VAS), providing global indices of “alertness”, “anxiety”, and “contentedness”. Mann-Whitney test was used to compare values obtained in the two groups with a significance criterion of p < 0.05. Spearman's correlation analysis was used to analyze the relationship between different measures.
Results On subjective ratings of alertness (ESS, VAS), the fatigue group rated themselves as significantly more sleepy than the non-fatigue group. However, there were no significant differences between the groups on objective measures of arousal (PST, CFFF, and CRT). There was a significant positive correlation between ESS and FSS scores; there was no correlation between subjective and objective measures of alertness.
Conclusions Patients with fatigue rated themselves as more sleepy than patients without fatigue, and measures of sleepiness and fatigue were strongly correlated. This difference between the two groups, however, was not reflected in the objective measures of alertness (i.e., measures derived from the PST, CFFF, and CRT). Although the PST does not appear to provide a direct objective measure of MS fatigue, its response to fatigue-relieving treatments may be a useful measure of therapeutic benefit. This possibility is currently being investigated.
I.2.2 (o22)
Prevalance of nystagmus: The Leicestershire nystagmus surve
N Sarvananthan, M Surendran, EO Roberts, S Jain, S Thomas, N Shah, FA Proudlock, JR Thompson, RJ McLean, C Degg, G Woodruff, I Gottlob
University of Leicester, Leicester, UK
Introduction The prevalence of nystagmus is unknown. Previous estimates have ranged between 1 in 20 000 to 1 in 1000.
Purpose An epidemiological study was carried out to estimate the prevalence of nystagmus in Leicestershire (population 925 000) between August 2001 and August 2004.
Methods Three sources of data were obtained. (1) A hospital based survey recruited patients from hospital ophthalmologists, neurologists, paediatricians, opticians and general practitioners; (2) registration forms and records of all patients registered as blind and partially sighted within the county; (3) patient recruitment from the education services. Patients who attended the hospital survey had detailed ophthalmological assessments, eye movement recordings and electrodiagnostics. Capture-recapture statistical analysis using log-linear models was used to estimate the prevalence of nystagmus.
Results The hospital survey recruited 238 patients (124 male and 114 female) with nystagmus. The registration forms and/or hospital records of 3358 out of 5885 blind and partially sighted individuals were reviewed and 414 patients (242 males, 172 females) with nystagmus were identified. The education services provided information about 193 individuals (82 males, 111 females) with nystagmus.
The commonest forms of nystagmus were idiopathic infantile nystagmus (50 patients) in the hospital survey, nystagmus secondary to low vision (145 patients) amongst visually impaired individuals and albinism (41 patients) in the education services group. Amongst children with neurological nystagmus, most were associated with syndromes such as Downs syndrome or septooptic dysplasia, or congenital neurological anomalies such as congenital hydrocephalus or microcephalus.
Capture–recapture analysis estimates the prevalence of nystagmus to be 24 per 10 000 population.
Conclusion The prevalence of nystagmus is higher than initially predicted. This information is important in health resource planning and allocation.
I.2.3 (o26)
Digital infrared pupillometry for comparing cocaine with apraclonidine testing when investigating Horner's syndrom
Shwe-Tin, G T Smith
The Great Western Hospital, Wiltshire, UK
Introduction Is cocaine testing really the gold standard for confirming oculosympathetic paresis that gives rise to Horner's syndrome?
Purpose To compare the pupillary response to 4% cocaine with 1% apraclonidine, using digital infrared pupillometry.
Methods The effect of 4% cocaine on pupil diameter in 17 patients with anisocoria was measured with the Procyon P2000SA pupillometer. Eight patients with no aniosocoria served as controls. At least 72 h later the same patients were tested with 1% apraclonidine. Palpebral aperture and enophthalmos were measured and any imagingreviewed.
Results All control patients tested negative with cocaine and apraclonidine. Eight of seventeen anisocoria patients tested positive with both cocaine and apraclonidine, i.e., oculosympathetic paresis. Five of seventeen tested negative with both drugs and had no other signs of oculosympathetic paresis, i.e., simpleanisocoria. Four of seventeen tested positive with cocaine and negative with apraclonidine, two had no other signs of oculosympathetic paresis, i.e., simple aniosocoria. Two had mild ptosis but normal radiological investigations and could not be definitively categorised as either oculosympathetic paresis or simple anisocoria. Based on the categorised patients, the sensitivity of both drugs was 100%, the specificity was 71% with cocaine and 100% with apraclonidine.
Conclusion This is the first study to take advantage of the accuracy, reproducibility and simplicity of digital infrared pupillometry to directly compare the effects of 4% cocaine with 1% apraclonidine on allpatients tested. Our results suggest both drugs are equally sensitive, but apraclonidine generates fewer false positives. Apraclonidine should replace cocaine for the pharmacological testing of suspected oculosympathetic paresis.
I.2.4 (o29)
Internuclear Opthalmoplegia and Readin
F.A. Proudlock, I. Gottlob, S.J. Farooq
University of Leicester, RBT Kilpatrick Clinical Science, Leicester Royal Infirmary, Leicester, United Kingdom
Background Internuclear ophthalmoplegia (INO) is a condition resulting from lesions to the medial longitudinal fasciculus and is characterized by an adduction deficit on the side of the lesion and a dissociated nystagmus on abduction of the contralateral eye. On clinical presentation we have observed that patients often report that reading is easier if one eye is closed. We have investigated reading in INO patients under monocular and binocular viewing conditions.
Methods Eye movements were recorded from 13 patients with INO during a reading task with either right eye open, left eye open or both eyes open. The adduction deficit was quantified by plotting adduction velocities against abduction velocities from either eye to saccadic targets at −20°, −10°, 0, 10°, 20°. Subjects were classified as having unilateral INO if the abduction: adduction slope was >0.7 in one eye, and bilateral INO if the slopes were <0.7 in both eyes. One subject, recorded from twice, had bilateral INOon first visit and improved to unilateral INO on the second visit. Instability of fixation was also measured in primary position.
Results Six volunteers were classified as unilateral and 8 subjects as bilateral INO (one volunteer repeated). Three volunteers showing either severe bilateral adduction deficit or vertical eye movement instability (nystagmus) in primary position, found reading almost impossible and took >40 s to read a line. Four subjects with unilateral INO read more quickly with affected eye than the unaffected eye. They found it relatively easy to read with botheyes open but preferred monocular reading. Five subjects with bilateral INO read at similar speeds with either most affected or least affected eye open but worse with both eyes open. Two subjects, who had poor visual acuity in one eye due to either optic neuritis or amblyopia, found reading with both eyes open as easy as monocular reading.
Conclusions We found that most subjects with INO could read better during monocular viewing compared to binocular viewing due to incomitant eye movements. Possibly occlusion of one eye, botulinum injection or surgery could be used as potential therapies to assist reading.
I.2.5 (p78, o32)
Smoking cannabis reduces congenital idiopathic nystagmus (CIN)?
Archana Pradeep, Shery Thomas, Eryl Roberts, Frank Proudlock, Irene Gottlob
Ophthalmology Group, University of Leicester, UK
Introduction Smoking cannabis has been described to reduce acquired pendular nystagmus in MS but its effect on congenital nystagmus is not known.
Purpose To establish whether congenital nystagmus (CN) can be suppressed by smoking cannabis.
Methods A 19-year-old male with congenital horizontal nystagmus presented in clinic after smoking 10 mg cannabis. He claimed the main reason for smoking cannabis is to improve his vision. At the next clinic appointment he had not smoked cannabis for 3 weeks. Full ophthalmological examination and eye movement recordings were performed at each visit.
Results Visual acuities improved by 3 logmar lines in left eye and by 2 logmar lines in right eye on smoking cannabis. Nystagmus intensities were reduced by 26% in primary position and 44, 11, 10, and 40% at 20° eccentricity to the right, left, elevation, and depression respectively after smoking cannabis.
Conclusion Cannabis might be beneficial to treat congenital idiopathic nystagmus. Further research to clarify the safety and efficacy of cannabis in patients with CIN administered for example by capsules or spray would be important.
I.3. Optic Neuropathies—Oral Presentations
I.3.1 (o5)
The Long-term Risk of Clinically Definite Multiple Sclerosis after Optic Neuritis: Final Follow Up of the Optic Neuritis Treatment Trial Cohor
Michael Brodsky1, Robin L. Gal2, Mariya Dontchev2
1University of Arkansas; 2Jaeb Center for Health Research
Objective To evaluate long-term visual function outcomes and probability of developing multiple sclerosis (MS) >15 years after an initial episode of acute optic neuritis.
Methods Between 1988 and 1991, 454 patients with acute optic neuritis were enrolled into the Optic Neuritis Treatment Trial (ONTT) and completed baseline testing. Recently completed exams represent more than 15 years of follow up, and included tests of visual acuity, contrast sensitivity, full-threshold visual fields, neurologic examination and assessment of quality of life using the National Eye Institute Visual Function Questionnaire and Neuro-ophthalmic Supplement.
Results Visual acuity in the affected eyes was >20/20 in 72%, <20/20 to 20/40 in 19%, <20/40 to 20/200 in 6%, and <20/200 in 3%. Visual function was generally worse in patients with MS than in those without MS. The NEI-VFQ quality of life scores were slightly lower (worse) in all subscales in the study patients than in a comparative reference group, as was the composite score for the Neuro-ophthalmic supplement. Among patients eligible for prospective follow up to assess risk of developing MS, the cumulative probability of CDMS was 45%: 24% in the 191 patients with no baseline brain MRI lesions, 57% in the 44 with one lesion, 57% in the 26 with two lesions, 72% in the 49 with three to six lesions, and 76% in the 42 with more than six lesions. Among patients with CDMS completing the exam (N=113) or patients who died from MS (N=4), 66% had an EDSS score <3, 21% 3 to <6, and 13% >6.
Conclusions Most patients retain good to excellent vision 15 years after an initial episode of optic neuritis. The long-term risk of CDMS after optic neuritis is strongly related to whether one or more lesions consistent with demyelination are present on brain MRI at the time of presentation. Even with two or more baseline brain MRI lesions, about one-third of patients will not have another demyelinating event meeting criteria for CDMS in the 15 years after the optic neuritis episode. When CDMS is present, most patients retain good neurologic function 15 years after the episode of optic neuritis.
I.3.2 (o6)
Optic neuropathy associated with inflixima
Marcel PM ten Tusscher
University Hospital Antwerp, Belgium
Purpose To describe the 5-year follow up of two patients with optic neuropathy associated with the use of infliximab.
Methods Two of three patients diagnosed as having bilateral anterior optic neuropathy occurring after a third dose of infliximab (BMJ 2003; 326: 579) were checked five years later.
Results Progressively deteriorating visual fields were noted and in one patient even vision loss was much more severe.
Conclusions Infliximab, an anti-TNF antibody, has been reportedly associated with vision loss and visual field defects as well as exacerbation of demyelinating disease. The pathophysiological mechanism involved is unclear. Deterioration of visual fields and visual acuity over five years with no other neurological symptoms is not likely consistent with demyelinating disease.
I.3.3 (o11)
A Single Centre, Non Randomized Interventional Study of Intra-Arterial Tissue Plasminogen Activator Fibrinolysis Versus Conventional Therapy for the Treatment of Central Retinal Artery Occlusio
Andrew W. Lee, Celia S. Chen, Rebecca S. Gottesman, Robert J. Wityk, Mona N. Bahouth, Phillipe Gailloud, Kieran Murphy, Eric Aldrich, Neil Miller
Departments of Cerebrovascular Neurology, Interventional Neuroradiology and Neuro-Ophthalmology, Johns Hopkins University School of Medicine.
Hypothesis Local intra-arterial fibinolysis (LIF) with tissue plamisnogen activator (tPA) administered in aliquots is associated with improvement in visual acuity (VA) in subjects with central retinal artery occlusion (CRAO), compared with patients who did not receive tPA.
Background CRAO due to platelet-fibrin thrombi or emboli results in visual loss from optic nerve and retinal ischemia with a poor chance of spontaneous recovery. We report the results of a single center, non randomized interventional study of subjects with CRAO who underwent either LIF therapy administered in aliquots or conventional treatment.
Design/Methods Consecutive subjects with CRAO from 2001-2006 were included. VA was documented by standard methods. Subjects underwent either conventional therapy of paracentesis and breathing carbogen (90% CO2/10% oxygen mixture) alone or a combination of conventional therapy and LIF by selective ophthalmic artery catheterization, followed by administration of tPA in 3 mg aliquots. The primary outcome was improvement of VA by 1 line or more on Snellen chart or its equivalent. Fisher's exact test and multivariate logistic regression with likelihood ratio testing were used to compare outcome in the two groups.
Results Each group included 21 subjects. 76% of subjects in the tPA group had VA improvement, compared with 33% in the standard therapy group (p=0.012, Fisher's exact). Multivariate logistic regression adjusting for gender, history of prior stroke/TIA, and history of hypercholesterolemia, showed that patients who received tPA were 36 times more likely to have an improvement in VA (p=0.0001) and 5 times more likely to have a final VA of 20/200 or better (p=0.03). Use of additional conventional therapies and baseline VA did not significantly change this point estimate. Two groin hematomas occurred in the tPA group but there were no intraocular or intracranial hemorrhages.
Conclusions/Relevance LIF with tPA administered in aliquots is associated with an improvement in VA compared with conventional therapy and had few side effects.
I.3.4 (o13)
A novel primate model of non-arteritic anterior ischemic optic neuropathy (pNAION)
Celia S. Chen, Mary A. Johnson, Bernard J. Slater, Neil R. Miller, Steven L. Bernstein
Neuro-Ophthalmology and Orbital Unit, Wilmer Eye Institute, Johns Hopkins Hospital, Baltimore, Maryland, USA Department of Ophthalmology, Anatomy and Genetics, University of Maryland, Baltimore, Maryland, USA
Introduction Nonarteritic anterior ischemic optic neuropathy (NAION) is an optic nerve stroke and is the leading cause of sudden optic nerve-related vision loss in adults over the age of 50. In this disease, axonal infarction results in loss of retinal ganglion cell function and death. There are no effective treatments, partly because the pathophysiology is not fully established. A rodent model of NAION, using a novel photoembolic mechanism, has been developed, but the considerable differences between rodent optic nerve and the highly structured optic nerves of primates make it difficult to predict human responses to treatment for optic nerve ischemia. We aim to determine whether NAION can be induced in non-human primates (pNAION).
Method A novel primate photoembolic model was designed in Rhesus monkey. Rose Bengal was injected intravenously at dose of 2.5 mg/kg and photoactivated with YAG-green diode (535 nm) laser at the optic nerve head with varying exposure time through a special designed contact lens for Rhesus monkey eyes. Following induction, the clinical, electrophysiological and histological changes were characterized.
Results In pNAION, optic nerve swelling is visible beginning 1 day post induction, and resolves within 2 weeks, with optic disc atrophy present by 4 weeks. Deep and surface vascular angiography of the optic nerve head circulation suggests that pNAION does not affect the choroidal circulation. Electrophysiology shows changes consistent with a primary ischemic optic neuropathy. There was relative preservation of retinal ganglion cell function until 1 week post infarction. The electrophysiological findings are consistent with histopathology showingsignificant reduction in retinal ganglion cell numbers, especially in the late stages of pNAION. Marked axonal loss and demyelization are seen, in the anterior optic nerve, with relative sparing of the optic nerve posteriorly.
Conclusion We have developed a novel, reproducible primate model of NAION (pNAION) that closely resembles human NAION clinically, electrophysiologically and histopathologically. Our model should make it possible to evaluate the specific pathophysiological changes associated with human NAION. It will also provide an excellent resource for evaluating neuroprotective and other strategies for treating optic nerve ischemia.
I.3.5 (o14)
Efficacy of Dorzolamide 2% for the Treatment of Nonarteritic Anterior Ischemic Optic Neuropath
Inés Contreras, Susana Noval, Gema Rebolleda, Francisco J. Muñoz-Negrete
Ophthalmology Department, Ramón y Cajal Hospital, Madrid, Spain
Purpose To determine whether topical dorzolamide is useful for treating nonarteritic anterior ischemic optic neuropathy (NAION).
Methods A nonrandomized, case-control prospective study was performed; twenty-three patients diagnosed with NAION were offered treatment with topical dorzolamide. The 15 patients who received dorzolamide and the 8 patients who refused treatment were followed for 6 months. A complete ophthalmologic evaluation, including measurement of Snellen visual acuity (VA), visual fields (standard automated perimetry, strategy SITA 24-2), and the retinal nerve fiber layer (RNFL) thickness using optical coherence tomography, was performed at baseline and 6, 12, and 24 weeks after presentation.
Results Differences between the treated patients and controls in VA, visual field mean deviation (MD), and RNFL thickness between the 6-month and the baseline visit were evaluated. There were no significant differences in VA, visual field MD, or RNFL thickness change between the groups. The percentage of RNFL thickness loss at the 6-month visit was 41.06% in the control group compared with 41.13% in the dorzolamide group (p=0.875,Mann-Whitney test).
Conclusions Treatment with dorzolamide does not appear to modify the poor anatomical and functional visual prognosis of NAION.
I.3.6 (o21)
Intravitreal Triamcinolone Acetonide Injection for Acute Nonarteritic Anterior Ischemic Optic Neuropath
Özlem Barut Selver, Aylin Yaman, Meltem Soylev Bajin, A. Osman Saatci
Department of Ophthalmology Dokuz Eylul University School of Medicine, Izmir Turkey
Purpose To examine the efficacy of intravitreal triamcinolone acetonide injection in the management of nonarteritic anterior ischemic optic neuropathy (NAION).
Methods Four patients who recieved a single injection of triamcinolone acetonide (4 mg) comprised our study group. The diagnosis was made by clinical examination and visual field test. Optical coherence tomography was performed where appropriate. The time between visual loss and intravitreal injection ranged between 4 days and 10days.
Results All of the patients had subjective and objective increase in visual acuity. No side effects including intraocular pressure rise were observed during our follow-up.
Conclusions Intravitreal triamcinolone injection may limit the damage and be helpful in eyes with a short history of visual loss due to ischemic optic neuropathy.
I.3.7 (o27)
Clinical and Prognostic Features of Bilateral and Unilateral Recurrent Optic Neuriti
Asli Kurne, Gül Yalcin Cakmakli, Rana Karabudak, Pínar Aydin, Tülay Kansu
Hacettepe University, Faculty of Medicine, Department of Neurology Mesa Hospital, Department of Ophthalmology
Aim Though various systemic diseases may cause recurrent optic neuritis, literature focuses mainly on multiple sclerosis (MS). The ONTT and CHAMPS trials acquaint us with prognosis and management of recurrent optic neuritis (RON) including probability of developing MS. Our aim is to determine the clinical and prognostic features of RON spectrum and to compare the prognostic factors for differential diagnosis.
Subjects & Methods Medical records of 467 patients, with a clinical diagnosis of idiopathic optic neuritis, treated in our center between 1985 and 2007 were studied. Among them 66 cases with bilateral and/or unilateral RON were the focus. Seven cases who have been followed up with the diagnosis of bilateral optic neuritis (BON), between years 1979–1985, in our center are added to the original group. Cases diagnosed with Leber's hereditary optic neuropathy, systemic or intracranial neoplasm's and toxic causes were excluded. Patients were classified as either; recurrent optic neuritis or bilateral simultaneous optic neuritis. These two groups were classified independently for age of onset, gender, number of recurrences, characteristics of optic neuritis (pain, visual acuity, response to the treatment), concomitant diseases, follow up period and MRI features.
Results Forty eight patients had recurrent unilateral optic neuritis and 25 had bilateral simultaneous optic neuritis. The patients were classified according to the final diagnosis. The subgroups in the RON group was; MS, chronic relapsing inflammatory optic neuropathy (CRION), endocrinopathy and functional visual loss and MS, collagen tissue diseases, neuromyelitis optica (NMO), postinfectious optic neuropathy in Bilateral optic neuritis (BON) group. Longest mean follow up duration was 9.8 years in the recurrent unilateral MS group. Patients with rapid succession of severe ON events are more likely to develop a generalized demyelinating disease. MS patients having BON BON, OR DO THEY MEAN RON? have limited and late response to corticosteroids when compared to the unilateral group. Differential diagnosis between multiple sclerosis and vasculitis is crucial as the treatment algorithm differs according to the diagnosis.
Conclusion The prediction of the prognosis of patients with the recurrent optic neuritis and the required treatment mainly depends on correct diagnosis. Besides MS, patients with recurrent optic neuritis are also at risk of collagen tissue diseases, neuromyelitis optica and CRION.
I.4. Optic Nerve, Chiasma, and Central Nervous System (CNS)—Oral Presentations
I.4.1 (o7)
Parent Artery Closure or Preservation in Endovascular Treatment of Aneurysm Induced Vision Loss?
Mark J. Kupersmith, Navraj S. Heran, Joon K. Song, Yasunari Niimi, Katsunari Namba, David J. Langer, Alejandro Berenstein
Inn at Roosevelt Hospital and New York Eye and Ear Infirmary
Objective Neurosurgeons and interventional neuroradiologists prefer preservation of the artery associated with an intracranial aneurysm, but it is undecided whether this is applicable in the therapy of aneurysms that cause anterior optic pathway compression (AOPC). To report results of endovascular treatments of AOPC.
Methods We retrospectively reviewed all of our patients with unruptured ophthalmic segment aneurysms with AOPC treated with endovascular coils. The outcomes were vision function of affected eyes, using visual acuity expressed in logmar notation and visual field grade (1–5, normal–blind), aneurysm and parent artery occlusion, neurological status, and treatment complications.
Results Seventeen patients (16 women), age range 38–83 years, had 28 affected eyes. In affected worse eyes mean baseline visual acuity and visual field score were 0.8, SD 0.8 and 3.2, SD 1.4. For first procedures, 16/17 aneurysms had endosaccular coiling with ICA preservation; and 1 aneurysm was trapped with ICA occlusion. Afterwards, patients were followed for a mean of 2.9 years (range: 1 month–11.2 years). One patient died from subarachnoid hemorrhage (SAH) after 1 month. Vision worsened in 6 patients, stabilized in 4, and improved in 6. Twelve patients had 13 subsequent procedures including endovascular ICA occlusion in 7, recoiling in 5, and optic nerve decompression in 1. Vision improved in 83% after ICA occlusion. Another patient died of SAH 5 months afterrecoiling. The final vision improved in 8 patients (50%), stabilized in 4 (25%), and worsened in 4 (25%). In the eyes with worse vision at baseline, the mean final acuity and field score were 0.8, SD 1.2 and 2.8, SD 1.6.
Conclusions Coil endosaccular treatment, with ICA preservation, of ophthalmic segment aneurysms with chronic AOPC may not benefit vision. Our results suggest that currently, endovascular trapping with ICA sacrifice appears to result in good vision, clinical, and aneurysm closure outcomes.
I.4.2 (o16)
Chiasmal lesion and visual inattention for temporally located symbols at visual acuity testin
Hans C Fledelius, Hanne G Roed
University Eye Dept, Rigshospitalet, Copenhagen University, Denmark
Purpose To present on a short video the lackful performance on a visual chart of a female patient with lesion to the chiasm, when tested for monocular visual acuity.
Case story A 52-year-old female visually managed worse after surgery for a pituitary adenoma with suprasellar extension. Her 1.0 acuity right eye could give the full line on the chart, symbol for symbol. The 0.4 acuity left eye could catch only the uttermost nasal symbol and ignored those on the rest of the line. She knew they were missing, but could not find them. Her reading has remained impaired and slow, word for word only, even when closing the poorer eye. On the tangent screen binocularly there is no central drop-out. At monocular testing there isbitemporal visual field defect, to midline in the better eye and exceeding the mid-vertical in the poorer eye.
Conclusion A video presentation was chosen for one out of the 52 ‘chiasmal’ subjects we have observed in our clinic with ‘nasal seeing’ or ‘temporal blocking’ at visual acuity testing. With the behavior observed in occasional patients, the observation should direct the attention to chiasmal pathology.
I.4.3 (o19)
Transient Visual Obscurations in Papilloedema. Observations based on a case series of 31 first eye optic nerve sheath decompressions for idiopathic intracranial hypertensio
J. Acheson
National Hospital for Neurology and Neurosurgery
Objective To evaluate effect of optic nerve sheath decompression (ONSD) on transient visual obscurations (TVO) in papilloedema due to idiopathic intracranial hypertension (IIH). To draw conclusions on likely pathogenesis of TVO's.
Methods A retrospective case review of 31 patients all with IIH diagnosed according to modified Dandy criteria (Digre 2001) undergoing first eye optic nerve sheath decompression by a single surgeon. Follow-up 12–24 months with later data if available not included. Visual function scoring after Wall and George (1991).
Result Mean Snellen fraction acuity in the operated eye improved from 0.48 to 0.65 and mean visual field score improved from 3.2 to 2.5. Eleven out of 31 cases (34%) required subsequent shunting, 5 for progressive visual failure and 6 for uncontrolled headache. Eight (26%) patients experienced prominent TVO's prior to ONSD: this symptom completely resolved in all cases. Six of these cases were in the group who did not require shunting, but 2 were in cases requiring shunting for problems associated with continuing uncontrolled raised intracranial pressure.
Conclusion ONSD appears to effectively relieve TVO's even in cases where intracranial pressure remained high and further CSF diversion surgery was required. This data provides further evidence for a local cause for TVO's within the optic nerve head, and not intracranially.
I.4.4 (o25)
Spontaneous Improvement of Optic Nerve Gliom
Riri Silvia MANOR, MD, and Nitza COHEN, MD
Rabin Medical Center, Dailinson Campus & Schneider Children Medical Center, Petah Tikva
Background Our understanding of the natural history of optic nerve glioma has been refined since Cameron Parsa et al. reported thirteen cases of spontaneous regression of large clinically symptomatic optic pathways gliomas in patients with or without neurofibromatosis.
Material During the last 16 years we had the opportunity to observe and to document by serial neuroimaging a case of a unilateral intraorbital optic nerve glioma without N-F 1 signs. Sixteen years ago, a healthy 4-year-old boy was brought to examination because of the presence of a unilateral exophthalmos, decreased vision to count fingers from 1.5 meters only and a pale edematous optic nerve head. CT and MRI examinations exposed an intra-orbital optic glioma pressing the eyeball.
Results During the years of follow-up, the visual acuity improved to 6/30, and this year it was found to be 6/10! There was a considerable improvement of the visual field as well, but the tumor size remained unchanged during the 16 years of follow-up. Moreover, in contrast to the striking visual achievements, there has been an increased enhancement of the tumor. The cellular mechanism that may produce visual function improvement with or without the shrinkage of these low-grade glioma has not yet been understood.
Conclusion Chemo-therapy and/or radio-therapy treatment of those tumors is often overdone. Rather, a close follow-up is indicated. We should always keep in mind the possibility of optic nerve glioma spontaneous improvement.
I.4.5 (o30)
Papilloedema in Sleep Apnea: Unnecessary Screening?
Silvia Muñoz1,3, Carme Monasterio2, Jorge Arruga1,3
1Department of Ophthalmology, Hospital de Bellvitge, Barcelona, Spain; 2Sleep Disorders Unit, Department of Pneumology, Hospital de Bellvitge, Barcelona, Spain; 3Department of Ophthalmology, Institut Català de Retina, Barcelona, Spain
Background Sleep apnea has been related to papilloedema as responsible of nocturnal hypoxemia and hypercapnia resulting in intracranial hypertension. Systemic manifestations of elevated intracranial pressure may be mild or absent because hypertensive peaks overlap apnoeic periods, thus when the patient is sleeping. Some serial cases of intracranial hypertension with papilloedema in male patients have been reported, affecting both overweight and non obese individuals and attributed to this sleep disorder.
Objective To investigate the prevalence of papilloedema in patients recently diagnosed of sleep apnoea and set up the guidelines for an ophthalmologic management if necessary. To detect asymptomatic intracranial hypertension caused by sleep apnoea.
Methods Forty-eight patients prospectively underwent an ophthalmologic examination after being diagnosed of obstructive sleep apnea by polisomnography in the Sleep Disorders Unit. Complete examination was performed (visual acuity, colour vision, IOP, eye movements, anterior segment, and posterior segment). Additionally an automated perimetry was performed if unexplained RAPD or a suspicious glaucomatous opticdisk was seen.
Results There were 35 men and 13 women whose mean age was 60. Mean visual acuity was 0.9 in both eyes. Mean IOP was 17.4 RE/ 15.8 LE. Body mass index (BMI) was obtained in 39 patients; BMI was over 26 in 97%. Main vascular risk factors (blood hypertension, diabetes, dyslipemia) were frequently combined: 54% had one, 23% had two or three. One patient had previous history of glaucoma. Fifteen patients (31%) had eye and perimetry findings consistent with glaucoma. Optic disk morphology was classified in four groups: normal without cupping (50% eyes), normal with cupping (14.5%), abnormal pallor, cupping or both (30% eyes), and oblique (5% eyes). Two eyes had myelinated peripapillary fibres. Among the group no patient had papilloedema.
Conclusion A high prevalence of glaucoma was detected, but papilloedema was not. Therefore sleep apnoea patients should be screened for glaucoma. Size of the sample is a limiting factor and may have biased glaucoma results. The most prevalent type of optic disk was the crowded one (50% eyes). It might explain why sleep apnea patients are prone to develop non ischemic optic neuropathy.
I.5. Genetics and Pediatric Neuro-Ophthalmology—Oral Presentations
I.5.1 (o1)
A Dynamic Visual Function Consequence of Infantile Nystagmus Syndrome is Being “Slow to See”: Model Predictions and Patient Data Identify Stimulus Timing as its Caus
L.F. Dell'Osso and Z.I. Wang
Daroff-Dell'Osso Ocular Motility Laboratory, LSCDVA Medical Center and CASE Medical School; Departments of Neurology and Biomedical Engineering, Case Western Reserve University; Cleveland, OH, USA
Objective In addition to static measures of visual function, we wished to investigate the dynamic properties of Infantile Nystagmus Syndrome (INS) that leads to patients' complaints that they are “slow to see.” For instance, which factors influence latency of the initial voluntary saccade (Ls) and latency to target acquisition (Lt)?
Methods We used our behavioral Ocular Motor System (OMS) model to simulate saccadic responses (in the presence of INS) to target jumps at different times within a single INS cycle and at random times during multiple cycles. The model predicted that the time within an INS cycle that a target jumped affected Lt. The responses of 4 INS subjects with different waveforms were compared to the model's predictions. Infrared reflection was used for 1 INS subject, high-speed digital video for 3. We studied both large and small target-step stimuli and evaluated the following factors: stimulus time within the cycle (Tc), normalized Tc (Tc%), initial orbital position (Po), saccade amplitude, initial retinal error (ei), and final retinal error (ef). The ocular motor simulations were performed in MATLAB Simulink environment and the analysis was performed in MATLAB environment using OMLAB software. Both the OMS model and OMtools software are available from http://www.omlab.org.
Results Our data analysis showed that, Ls was a fixed value that is typically higher than the normal saccadic latency. For Lt, Tc% was the most influential factor for each waveform type. The OMS model's predictions accurately simulated the human data. The main refixation strategies employed by INS subjects made use of slow and fast phases and catch-up saccades, or combinations of them. These strategies helped the subjects to foveate effectively after target movement, sometimes at the cost of increased target acquisition time.
Conclusions Saccades, whether foveating or braking, disrupted the OMS' ability to accurately calculate saccade amplitude and refoveate. Idiosyncratic variations in the prolongation of Ls occurred among INS subjects. Our OMS model simulations demonstrated this emergent behavior; this robust model can be used to both predict and reinforce data analysis in future research.
I.5.2 (o4)
Infantile Esotropia with Nystagmus: A Treatable Cause of Oscillatory Head Movements in Childre
Michael C. Brodsky and Kenneth W. Wright
University of Arkansas for Medical Sciences, Little Rock, Arkansas and the Cedars-Sinai Medical Center, and the Keck School of Medicine, University of Southern California, USA
The rare association of head oscillations with infantile esotropia and nystagmus has gone unrecognized. We examined three patients who had infantile esotropia, nystagmus, and unexplained head shaking or head nodding. Strabismus surgery restored ocular alignment and produced resolution of the head shaking in all cases. In one case, head shaking accompanied recurrence of the esotropia and again resolved following surgical realignment of the eyes. Head shaking or head nodding can rarely be associated with infantile esotropia and nystagmus. In this setting, surgicalrealignment of the eyes may produce simultaneous resolution of the head oscillations.
I.5.3 (o18)
Comprehensive mtDNA Sequence Analysis Reveals Novel Mutations and Distinct Features in Turkish Patients with Leber's Hereditary Optic Neuropath
Çetin Kocaefe1, Roland Heilig2, Gölge Acaroğlu3, Feyza Önder4, Tülay Kansu5, Meral Özgüç1
Institutions: 1Hacettepe University Faculty of Medicine Dept of Medical Biology, Ankara, Turkey; 2Genoscope, Centre National de Sequencage, Evry, France; 3Social Security Eye Hospital, Neuro-ophthalmology & Orbital Disease Unit, Ankara, Turkey; 4Istanbul Haseki Hospital Eye Clinic, Ankara, Turkey; 5Hacettepe University Faculty of Medicine, Dept of Neurology, Neuro-ophthalmology Unit, Ankara, Turkey
We have investigated the mitochondrial DNA characteristics of 30 patients diagnosed with Leber's hereditary Optic Neuropathy (LHON) by sequencing analysis following a definitive clinical diagnosis. The identified nucleotide variations have been compared to the mtDNA sequence of 85 unaffected employed used as ethnically matched control data and also been compared to the Uppsala mtDNA sequence database.
The analysis revealed the presence of 9 previously identified sequence variations in 18 patients. The primary LHON mutation G11778A is the most frequent nucleotide change observed in 10, A4917G in 4 patients. The G12192A, G3316A, C3497T, T4336C, C4640A, A10006G, T14484C mutations are observed in singleton patients. 21 novel nucleotide changes have been identified where 8 of them are accompanying the previously reported mutations and 13 novel nucleotide changes are observed in 12 remaining patients. Of the 13 novel nucleotide changes, 8 are in ATPase subunits 6 and 8.
The remaining 5 variations are observed in ND1, ND5, COX1 and CytB genes. Additionally, 6 variations reside in highly conserved regions of rRNA and tRNA genes. All of the observed novel mutations are in the homoplasmic state and apart from one Cox1 mutation, none observed in 85 control individuals.
Turkish population diverges from the European Caucasians with a lower prevalence of the canonical primary mutations. Three nucleotide variations of T4336C, A10006G and G12192A have been previously reported to be associated with Parkinson's disease, encephalopathy and cardiomyopathy respectively. Their occurrence in Turkish LHON patients suggests a phenotypic variation in the clinical presentation of the mitochondrial impairment. The reported data will aid for further genetic testing for the presence of other mtDNA alterations and for the establishment of a fullmolecular diagnosis in LHON patients.
I.5.4 (o20)
A novel missense mutation, p.R229G, in the FRMD7 gene causes idiopathic congenital nystagmus linked to Xq26-q27
Y. Kaplan1, I. Vargel2, T. Kansu3, B. Akin4, E. Rohmann5, S. Kamaci6, E. Uz7, T. Ozcelik7, B. Wollnik5, N.A. Akarsu4
1Department of Neurology, Gaziosmanpasa University, Tokat, Turkey; 2Department of Plastic and Reconstructive Surgery, Hacettepe University Medical Faculty, Ankara, Turkey; 3Department of Neurology, Hacettepe University Medical Faculty, Ankara, Turkey; 4Gene Mapping Laboratory, Pediatric Hematology Unit, Hacettepe University Medical Faculty, Ankara, Turkey; 5Center for Molecular Medicine Cologne (CMMC), University of Cologne, Cologne, Germany; 6Department of Orthodontics, Hacettepe University, Faculty of Dentistry, Ankara, Turkey; 7Department of Molecular Biology and Genetics, Bilkent University, Ankara, Turkey
X-linked idiopathic congenital motor nystagmus (NYS1) is the most common form of nystagmus. Two distinct loci, one on Xq26-q27 and the other on Xp11.4 were previously reported for NYS1. Recently, FRMD7 (FERM domain-containing 7) mutations were identified in the Xq26 linked families. We report an extensive NYS1 pedigree including 162 individuals of whom 28 are affected. The mode of inheritance is clearly X-linked demonstrating 27% penetrance in female obligate carriers. Genetic linkage analysis confirmed the Xq26 locus and further mutation screening identified a novel missense c.686C>G mutation in exon 8 of the gene. This change causes a substitution of a conserved arginine at amino acid position 229 in the functionally important FERM-C domain of the protein by glycine (p. R229G). The following points support the disease-causing nature of this mutation: (a) it co-segregates with the disease in the family; (b) a Turkish control population screen excludes non-synonymous polymorphism possibility (c) it is located in the FERM-C domain in the vicinity of the previously described missense mutations. In this family, we observed an individual who is born to two affected parents, therefore, homozygote for NYS1. No phenotypic difference is documented between homozygote and heterozygote states of this disorder. We also detected skewed X-inactivation in the affected females of our family suggesting that X-inactivation patterns might influence the manifestation of the disease in females. Little is known about the function of FRMD7 and to the best of our knowledge its inactivation status has not been studied. Further investigations on the X-inactivation status of FRMD7 might help to understand the irregular pattern of inheritance NYS1 (Support: Hacettepe University Research Foundation, 02 01 101 010).
I.5.5 (o28)
A New View of Neuromyelitis Optica (Devic's Disease)
Shelley Ann Cross
Mayo Clinic, Rochester, MN, USA
New research is providing the basis for a revised understanding of Neuromyelitis Optica (NMO). NMO, or Devic's disease, has been characterized by the presence of optic neuritis that is usually bilateral, simultaneous and severe, myelopathy with longitudinally extensive spinal cord imaging abnormalities, and no brain MRI abnormalities typical of multiple sclerosis (MS). There is often rapid progression to debility and death. These features have been used to distinguish NMO from MS. Researchers at the Mayo Clinic have identified an antibody marker of NMO that binds selectively to the aquaporin-4 water channel and may play a causative role in the disease. This marker has beenfound in Japanese patients with opticospinal MS, prompting the suggestion that NMO and Japanese opticospinal MS are the same disorder. The NMO antibody predicts the frequent relapse of optic neuritis and myelitis. It is also found in some patients with Sjogren's syndrome and lupus erythematosis implicating a close relationship between NMO and other auto-immune disorders. Because this antibody is found in patients with brain MRI abnormalities atypical of MS, the diagnositc criteria for NMO have been revised to allow the inclusion of patients with these MRI abnormalities. The distinction between NMO asnd MS is critically important because these two disorders respond differently toimmune modulatory therapy.
I.5.6 (o31)
Topographic Parameters of the Optic Nerve Head in Hereditary Optic Atroph
Golge Acaroglu, Ufuk Elgin, Bayazit Ilhan, Tulay Simsek, Solmaz Ozalp, Orhan Zilelioglu
Ulucanlar Ankara Eye research Hospital
Aim Patients with Autosomal Dominant Optic Atrophy (ADOA) share a distinct clinical appearance to their optic discs. This study is aimed to clarify the morphological features of the optic nerve head in presumed ADOA patients by comparing the topographical optic disc parameters with normal individuals.
Material and Methods Thirteen patients, 6 of which belonging to the same pedigree were identified in a prospective observational study from 2003 to 2006. Clinical data were collected by a single observer and the diagnosis was made on clinical grounds with special emphasis on the described optic disc morphology, along with reduced vision, reduced color vision, paracentral visual field defects, normal intraocular pressures and familyhistory. Confocal scanning laser ophthalmoscopy was used in order to perform quantitative topographic measurements of the optic nerve head. Measurements were compared to age and sex matched normal individuals. Two tailed T Test was used for statistical comparisons.
Results The disc area (2.73 ± 0.43 mm2 vs. 2.17 ± 0.43 mm2) and the cup area (0.98 ± 0.4 mm2 vs. 0.42 ± 0.18 mm2) were significantly larger than normal (p=0.000 for both). The cup volume (0.29 ± 0.17 cmm vs. 0.85 ± 0.61 cmm) was significantly smaller than normal (p=0.000). C/D area ratio (0.35 ± 0.12 vs. 0.2 ± 0.08) and linear C/D ratio (0.59 ± 0.09 vs. 0.44 ± 0.11) were bigger than normal (p=0.000 for both). Mean and maximum cup depths were significantly bigger (p=0.000 and p=0.002). Some patients' discs showed localized retinal nerve fiber layer defects corresponding to the visual field defects.
Conclusion Disc topography of eyes with ADOA was quantitatively characterized by bigger discs with bigger cups. Color vision, visual field characteristics and genetic analysis should be assessed accordingly to make diagnosis of ADOA certain in such patients.
Section II: Posters
II.1. Orbit—Poster Presentations
II.1.1 (p30)
Silent period of levator palpebrae; Relationship with ARAS and nucleus of Levator palpebrae.
Hilmi Uysal1, Bari˙n Selçuk2, Murat Ersöz2, Müfit Akyüz2
1Akdeniz University Hospital, Neurology Department, Antalya, Turkey; 2Ankara Physical Medicine and Rehabilitation Education and Research Hospital of Ministry of Health, Ankara, Turkey
Aim Opening and closing eyes are managed by levator palpebrae (LP) and orbicularis oculi (Oo) muscles. When an eye is opened Oo muscle is suppressed and when an eye is closed LP muscle is suppressed conversely. Nucleus of LP is in the mesensephalone as a part of oculomotor nucleus. In daily EMG practice LP muscle activity is not recorded routinely. Functions of LP muscles are a component of Glasgow coma scale. There must be a direct relationship between ARAS and nucleus of LP in the brainstem.
Materials and Methods In this study we have explored electrophysiological clues related to this opinion. We have examined patients with total peripheral facial palsy because the only thing we wanted to ascertain was LP muscle activity. With needle EMG electrodes we recorded LP muscle activity by inserting the middle of the supraorbital margin with great care for the upper globe of the eye and by monitoring the muscle activity in electromyography. After that we asked patients to open their eyes and while eyes were open we stimulated the median nerve with a 0.5–1 msec. stimuli, followed by stimulation of the contralateral supraorbital region. We have recorded reflex activity of the LP muscle separately when eyes were open and closed.
Results Seven patients (3 females and 4 males) were examined. Mean age was 37 ± 15 years. All patients have total peripheral type facial palsy. With median nerve stimulation there is a silent period beginning 53 ± 4 msec (mean ± SD) ending in 78 ± 3 msec. in activity of LP muscle. Mean of silent period was 25 ± 4 msec, however, this was not observed in the Oo muscle when median nerve is stimulated in normal side.
Conclusion So we propose that this silent period is a sign of relationship between ARAS and LP muscle. Even if this pattern is an inhibitor type, we think that there may be a polysynaptic reflex relationship excitatory type and it would be suppressed during sleeping.
II.1.2 (p42)
Visual field loss caused by excessive blepharochalasis due to solar elastosis
Yasemin Ozdamar, Golge Acaroglu, Huseyin Ustun, H. Muzeyyen Astarci, Pergin Atilla
Ministry of Health, teaching hospitals: Ulucanlar Eye Hospital and Ankara Hospital, Ankara, Turkey
A 65-year-old white male presented for evaluation of bilaterally “droopy” lids. He reported difficulty seeing around. The condition had been present for at least five years. Gross inspection of the lids revealed prominent folds of excess skin in the upper lids. His eyelids and forehead region skin was thickened and poorly adherent to the underlying tissue. Superficial yellowish papulae were present.
The patient underwent bilateral upper lid blepharoplasty. Visual fields improved immediately. Pathologic examination revealed solar elastosis in the specimens.
II.1.3 (p63)
Orbital Granulositic Sarcoma
Kadircan H. Keskinbora, Mithat Ulay
Acibadem Bakirkoy Hospital, International Hospital/Yesilkoy
Background Orbital granulocytic sarcoma is a localized tumor composed of cells of myeloid origin. Histological diagnosis can be difficult in patients with poorly differentiated orbital tumors and no evidence of systemic leukaemia.
Case Presentation In this poster, a case of six years old male patient with orbital granulocytic sarcoma is presented. In the ophthalmological examination, the child had left proptosis and his left eye was displaced upside. The eye movements were normal. The right and left visual acuity were 1.0 and 0.4 consecutively. Ocular tensions and fundus examinations were also normal in both eyes. Magnetic resonance scans showed that there was a mass at the bottom of the left orbit which eroded the bone and displaced the eye anteriorly and superiorly. For definite proper diagnosis, incisional biopsy was performed. At the same time, intramedulaar bone marrow biopsy was performed from the iliac bones and sacrum. Bone syntigraphy was normal. No abnormality was found in the bone marrow. In the pathological processing of the orbital mass, Haematoxylin and eosin, Leder, and lysozyme stained sections and immunohistochemical evaluation were performed.
Conclusion Orbital granulocytic sarcoma also called myeloid sarcoma or chloroma is a tumor that affects children and can present with rapidly progressive proptosis. This tumor may develop before, during or after the occurrence of systemic leukemia.
II.1.4 (p74)
Reconstructive surgery for severe cranio-orbito-basal damages
G.G. Shahinian, O.N. Dreval, K.D. Meparishvili, Z. Yu
Chair of Neurosurgery, Russian Medical Academy of Postgraduate Education, Municipal clinical hospital No 67
Objective The increase of penetrating cranio-orbital injury (PCOI) rates and severity of its complications and consequences (basal CSF fistula, meningoencephalitis, endophtalmitis, diplopia, squint) make well timed specialized medical treatment as well as working out of new methods of treatment together with perfection of old ones especially important. This report was designed to analyze surgical tactic in patients with PCOI.
Materials and Methods A total of 128 pts with PCOI was treated from 1999–2006. Investigations included: X-ray of a cranium and zygomatic bone, CT and 3D CT.
Results Patients were divided into 4 groups: Group 1: 9 patients with GCS of 3–4; Group 2: 26 patients with GCS of 5–6; Group 3: 34 patients with GCS of 7–8; Group 4: 59 patients with GCS of 9–10.Operative treatments were performed within a few hours after trauma or up to 10 days. Types of operative interventions: debridement of a cranio-orbital wound, with removal of nonviable bone fragments and foreign body (41 pts); bifrontal craniotomy with closure of craniobasal CSF fistula (24 patients); partial resection of ethmoidal bone cells with plastic of a medial orbital wall using fascia or sintetic dura mater (14 patients); kanthopexy in telecantus (8 patients).
Conclusion Results of our investigation allow us to recommend primary reconstruction of the upper part of facial bones and base of anterior cranial fossa with tough fixation of bone fragments within the acute period between 1 to 10 days after trauma. 3D CT image allows us to determine relations of foreign body with structures of a craniobasal region and choose adequate and most sparing surgical access for there removal, as well as to determine the spreading of a structure damages in a region in order to find out the right tactic of plastic of a base of anterior cranial fossa. Application of basal accesses allows removal of a foreign body successfully, without added brain trauma and damage of big blood vessels with good cosmetic results, and to carry out plastic of a cranial base in basal CSF leakage cases. In PCOI, specialists of different profiles (neurosurgeons, ophthalmosurgeons, cranio-facial surgeons) should be enlisted. Patients of this group must be hospitalized in multi-profile clinics.
II.2. Pediatric Neuro-ophthalmology—Poster Presentations
II.2.1 (p19)
Diagnostic challenge: Visual loss and bilateral optic nerve head swelling in an overweight teenager
A.H. Dahlmann-Noor 1,2, E. Cosgrave1,2, A.S. Ramsay 2, S. O'Sullivan2, A. Parker1, N. Sarkies1, L.E. Allen1, A.J. Vivian1,2
1Addenbrooke's Hospital, Hills Road, Cambridge; 2Eye Treatment Centre, West Suffolk Hospital, UK
Case A 15-year-old girl presented with a 4 day history of central visual loss of the left eye (LE), which had developed over 24 hours.
Significant findings Body Mass Index (BMI) 37 (Obese), Snellen VA right eye (RE) 6/6, LE counting fingers, LE relative afferent pupillary defect (RAPD), Ishihara RE normal, LE 0, bilateral optic nerve head (ONH) swelling and no retinitis/retinal vasculitis. Brain MRI (without gadolinium) was normal. The CSF was assessed, using lumbar puncture method, whereby the pressure was 28 mm H2O. Within the CSF, protein levels and glucose were normal. There were no pathological cells and no growth in the microbial media has been observed.
Initial diagnosis was Idiopathic Intracranial Hypertension (IIH), which was treated using acetazolamide, and sodium bicarbonate.
Review a few days later showed irregular ONH margins and B-scan revealed bilateral ONH drusen (ONHD). Visual field of the right eye was normal however, the left eye had an inferior and central deficit. Visually evoked potentials of the LE was also severely reduced.
Second diagnosis was an anterior ischaemic optic neuropathy secondary to drusen. Further evaluation, including an MR venogram, CSF infusion studies and vasculitis screen were normal. Whereby, systemic medication was discontinued.
There were improvements in the left eye at the two month review, with a visual acuity of 6/12 and normal Ishihara. Although there was red desaturation, left RAPD and a pale left ONH. Retrospective diagnosis was, either an idiopathic or post-infectious optic neuritis.
Discussion The differential diagnosis of optic nerve head swelling and visual loss is complex. IIH is less common in children than in adults. Clinical characteristics and outcome differ before and after puberty, with worse visual prognosis in the latter.
ONHD and IIH can co-exist in children. ONHD cause visual loss through nerve fiber bundle compression, peri-papillary neovascular membranes, or acute ischaemic events. Drusen not visible on fundoscopy (“buried drusen”), which are more common in children, are less likely to cause visual symptoms (5–36%).
Cerebral venous sinus thrombosis (CVST) is a multifactorial disorder potentially associated with significant mortality and morbidity. Clinical manifestations are non-specific and may be subtle. Of patients with presumed IIH, 9.4% may have CVST. MR venography is the diagnostic method of choice.
Optic nerve demyelination in children may be unilateral in 34–58%, with initial severe visual deficit in 2/3 and full recovery in 83%. White matter lesions are seen in 33–55%. Auto-immune processes including interactions between genetic predisposition, environmental factors and infectious pathogens are potential causes.
II.2.2 (p20)
Visual impairment in premature children with hydrocephalus
D. Lilakova, M. Kuba, D. Hejcmanova
Department of Ophthalmology, University Hospital, Hradec Kralove, Czech Republic
Objectives The developments in neonatology have resulted in an increasing number of deliveries of extremely imm Immature or premature?ature infants that are associated with high morbidity. The frequent cause of visual problems is neurological diseases.
The aim of this study was to document the quality of visual functions in premature children with posthaemorrhagic hydrocephalus, which underwent neurosurgical operation.
Patients and methods We have evaluated 8 premature children (16 eyes) with birth weight between 870 and 1380g. These children were born between 26 and 29 weeks of gestation. We evaluated visual acuity, refraction, strabismus and visual-evoked potentials.
Results The visual acuity was in the range of 1.0–0.8 in 37.50% of eyes, 0.7–0.5 in 25.00%, 0.4–0.3 in 6.25%, 0.2–0.1 in 18.75%, less than 0.1 in 12.5% of eyes. 37.50% of the examined children wearglasses; all of them have hypermetropic correction. Strabismus was found in 62.50% of patients, esotropia in 50.00%, exotropia in 12.50%. The subatrophy of optic disc was found in 50.00% of eyes, the cupping of disc was found in 12.50% of eyes. All children have abnormalities of visual-evoked potentials (VEP). 87.50% of children have ocular motor problems.
Conclusions Results of this study confirm an increased prevalence of refractive errors and strabismus in premature children with posthaemorrhagic hydrocephalus. The best corrected visual acuity was normal only in 37.50% of patients. All children have VEP abnormalities.
The results of the study show the necessity of following these patients regularly. Follow up, even in later age, together with the attempt to compensate refractive errors is very important, especially in children with common problems who have frequent vision complication as well.
II.2.3 (p21)
Neurofibromatosis Type 1 with sphenoid bone dysplasia in a teenager
M. Johari, S.S. Fatimah, M.K. Khairidzan.
Department of Ophthalmology International Islamic University of Malaysia & Hospital Tengku Ampuan Afzan, Kuantan, Pahang, Malaysia
Purpose To report the case of Neurofibromatosis Type 1 with sphenoid bone dysplasia.
Case Report We report a case of a rare condition, neurofibromatosis Type 1 with left sphenoid wing dysplasia and left pulsating exophthalmos with a good vision who underwent surgical repair successfully. The 13-year-old boy who was diagnosed to have Neurofibromatosis Type 1, in our clinic, showed a 3 month history of left eye discomfort and gradual proptosis. Clinical examinations revealed, left eye pulsating exophthalmos with unremarkable of fundus background. Orbital CT /MRI scan showed sphenoid bone dysplasia with meniggoencephalocele.
Other ocular and skin findings included left upper lid plexiform neuroma, prominent corneal nerves, lisch nodules, café au lait spot, and cutaneus neurofibroma. This child had no other bony deformity or a family history of neurofibromatosis disease. Subsequently this child underwent titanium mesh repair of the bony defect. The surgery was successfully and his vision preserved.
II.2.4 (p28)
Paediatric unilateral papilledema
N. Martin1, Ch. Wolley-Dod1, S. Muñoz2
1Department of Paediatric Ophthalmology, Hospital Vall d'Hebron, Barcelona, Spain;2 Department of Ophthalmology, Hospital Universitari Bellvitge, Barcelona, Spain
BackgroundHeadache and bilateral optic disc swelling are the prime manifestations of intracranial hypertension. However the lack of them should not rule out this condition.
Case An asymptomatic 7-year-old boy with neurofibromatosis type 1 had been diagnosed of left optic nerve and chiasmal glioma at 36 months of age. He also had multiple unknown bright objects (UBOs), remaining stable on serial neuro-radiological examinations. In a routine follow-up control, visual acuities were 0.8 in both eyes, color vision was normal, as was extrinsic and intrinsic ocular motility. Fundus examination revealed optic disc swelling of the right eye, whereas the left optic disc was normal. On MR imaging an active hydrocephalus due to obstruction of the Sylvian aqueduct was observed. After endoscopic ventriculocisternostomy right optic disc swelling and hydrocephalus resolved.
ObservationsUnilateral papilledema is rare in the paediatric population. In this case, unilateral papilledema was initially attributed to blocking of the cerebrospinal fluid by the glioma at the left orbital apex. As no radiological asymmetries were detected in the dural perioptic space around both optic nerves, the mechanism of unilateral papilledema remains unclear.
Conclusion Intracranial hypertension should be considered in the differential diagnosis of a unilateral optic disc swelling in paediatrics, irrespective of the background pathology of the patient. Also, children may remain totally asymptomatic, so diagnosis is made on the basis of routine follow-up examinations.
II.2.5 (p40)
Ophthalmologic characteristics of the slight closed craniocerebral injury (CCI) of the children
S.K. Guseynova, S.Z. Salmanova, N.Z. Aliyeva
Research institute (Z. Aliyeva) and Baku Children's Neurologic Hospital, Baku, Azerbaijan
Objective With incipientmanifestations of CCI, we observed 128 children. 85 patients had neurologic and neuro-ophthalmologic symptomatology which appeared straight away or during the first 24 h, the others 5–12 days after the injury.
Materials and Methods The clinical presentation of 62 children (1st group) whose condition can be estimated as brain concussion, was characterized by symmetric pupil dilatation, transitory amblyopia, symmetric inefficiency of sixth cranial nerves, increase of optico-vestibular sensitivity (threshold reduction of opticokinetic nystagmus) and increase or perversion of oculocardiac reflex. The electroencephalogram (EEG) detected moderate disorganization of main rhythms.
The ophthalmoscopy detected: venous hyperemia, excavation ectasis of the optic nerve head (ONH), and diffuse foveal reflex. The amplitude of general electroretinogram (ERG) was somewhat reduced. Visual evoked potentials (VEP) revealed a moderate lengthening of peak latency P100, and also size increase of perceptible cells of chess pattern. Almost all children at the premature infancy had continuance of retardation of visual maturation.
33 of the children (2nd group) had slight brain contusion. On EEG the character of á-rhythm reduction and assimilation of given photo stimuli had more distinct asymmetry, by type “worse” on the scalp, contralateral stimulated homonymous hemianopsia.
Results Ophthalmoscopy was the same with the first group; the phenomenon of “excavation ectasis of ONH” in this group wasn't revealed. VEP study detected the delay of impulse conduction, which echoed in asymmetric characteristic of component latency. Further ophthalmologic investigation revealed slight compound hyperopic astigmatism, asymmetric refractive amblyopia, and slight alternating squint.
During the first three days following CCI, arctation of liquor spaces, including ventricle of the brain, combined with the density rise of white substance, mainly around the 3rd ventricle was found. In that period, evidence of the cause of intracranial pressure rise was brain hyperemia not a brain edema. Starting with 3–4 days by the retention of liquor space compression, the density reduction of both white substance, and cortex was observed.
Conclusion Findings of the children with slight degree of CCI are analogous to disorder of neurohumoral control of regulation of central cerebral hemodynamics during the critical period of perinatal encephalopathy.
II.2.6 (p58)
Pilocytic astrocytoma in children and serious consequences due to noncompliance of their parents
M. Hejcmanova1, R. Autrata1, K. Landau2, M.M. Boesch2
Department of Ophthalmology, University Hospital, Hradec Kralove, Czech Republic; 1Department of Ophthalmology, Brno, Czech Republic; 2Department of Ophthalmology, University Hospital, Zurich, Switzerland
The authors report on two cases of pilocytic astrocytoma (grade I) in optic chiasm.
Case 1 In a 9-year-old girl, surgical intervention for suprasellar pilocytic astrocytoma was performed. Postoperative neuroimaging demonstrated that the resection was only subtotal and cranial radiotherapy was recommended which the parents have refused. Regular MRI and ophthalmic examination were proposed. One year after the surgery, repeated MRI showed stable mild hydrocephalus with no influence on visual functions (VA=1,0)and no tumor progression. At age of 12 years the patient presented again, only light perception was detected in the right eye and best corrected visual acuity was 0,7 in the left eye, accompanied by visual field defects. Ophthalmic examination showed chronic papilledema due to progression of tumor compressing optic chiasm. Unfortunately, after total resection of tumor and V-P shunt placement, the visual functions have further worsened.
Case 2 A 10-month-old girl was found to have esotropia and proptosis of her left eye due to tumor located in left optic nerve with extension into optic chiasm.
The patient underwent repeated tumor resections and V-P shunt placement. The radiotherapy after the age of 3 years and further regular neuroimaging controls were refused by parents. The patient was further “treated” by a healer. The girl presented again at 7 years of age with severe proptosis of left blind eye and serious decreased visual acuity in the right eye, headache and vomiting. Neuroimaging revealed progression of tumor into optic chiasm with suspected dissemination into the 4th ventricle. The enucleation of the left eye was performed and the patient receives now palliative chemotherapy.
Conclusion Total surgical resection of low grade gliomas can be curative in 95% to 100% children dependent on location. Even after partial resection, the tumors may stabilize for long periods of time. Regular ophthalmic and neuroimaging follow-ups and co-operation of patients or their parents are crucial for right timing of further intervention.
Preference: Poster
II.2.7 (p94)
Visual prognosis in childhood optic pathway gliomas
A. Dev Borman1, D. Thompson2, D.R. Hargrave3, R.J. Leitch1,2
1Sutton Eye Unit, Epsom and St Helier NHS Trust, Surrey; 2Great Ormond Street Hospital, London; 3Royal Marsden Hospital, London
Objectives Childhood optic pathway gliomas (OPG) represent 0.6–5.1% of intracranial tumors. The natural history is highly variable and there is an association with neurofibromatosis type 1 (NF1). Management decisions for OPG can be complex as tumor progression may be visually asymptomatic. Close neuro-ophthalmic follow-up, employing neuroradiological and neurophysiological investigation is necessary. Treatment decisions require a multi-disciplinary approach.
Aim To review the OPG patients in one eye unit, comparing clinical findings, investigative results and visual outcomes relative to diagnosis and treatment.
Methods A retrospective case note review of 26 patients (52 eyes) with OPGs, seen between April 1996 and December 2006, was performed.
Results Of 26 patients, 13 were male, 18 (69.2%) had NF1 and the median ages at presentation were 5 years 2 months (NF1 group) and 6 years (non-NF1 group).
Monocular visual acuity (VA) was achieved in 25 patients. Eyes were divided into groups: VA worse than 6/36 (‘poor’) and 6/36 or greater (“better”). Of patients in whom monocular VA was available, 28 eyes presented with ‘better’ vision. None of these deteriorated to “poor”. 22 eyes presented with ‘poor’ vision; 3 of these improved to greater than 6/36. Overall, 26 eyes showed improved VA, with corresponding improvement or stability of color vision, visual fields and pupil function. In one patient (in whom only binocular VA was achieved), vision deteriorated from “better” to “poor”.
All patients had radiological confirmation of their tumors. NF1 patients had varied appearances, including extension/multi-focal OPGs. A total of 87.5% of non-NF1 patients had isolated chiasmal OPGs.
Nineteen patients underwent visual electrophysiology (VEP) testing. A range of VEP changes were seen, including changes in patients without clinical evidence of visual pathway involvement/deterioration.
Conclusion OPG patients presenting with “better” vision can expect to have stable or improved vision as their final outcome. Patients with “poor” vision in one or both eyes have a small chance of improving into the better group (13.6%). Electrophysiology can prove visual pathway involvement in the absence of clinical signs and can be a qualitative indicator of vision. This data can help clinicians advice patients and parents of visual prognosis in optic pathway glioma.
II.2.8 (p117)
Sonographic results in neurofibromatosis 1
N. Karabul, R. Brzezinska, R. Schumacher, P. Gutjahr
Pediatric Oncology Department, Johannes Gutenberg University of Mainz
Introduction Neurofibromatosis 1 is an inherited disorder (autosomal dominant disease), characterized by formation of neurofibromas in the skin, subcutaneous tissue, cranial nerves and spinal root nerves. Sonography can help to diagnose it.
Methods We studied the abdomen, retroperitoneum, orbit and peripheral nerves in 84 patients, aged between 2 and 54 years of age. From these, 28 patients had a positive family history,. We used US-vector probes with a frequency of 2.5–8.5 MHz and linear probes of 8–14 MHz.
Results In 31 cases we found pathological results: 4× gliomas of optic nerve; 4× intraabdominal/retroperitoneal plexiform neurofibromas; 2× stenosis of the renal artery; 23 with neurofibromas of peripheral nerves, sensorimotor as well as autonomous.
Conclusions To study patients with Neurofibromatosis 1, sonograpy is a suitable method. We were able to detect glioma of the optic nerve, renal artery stenosis and, in particular, neurofibroma of peripheral nerves if they were larger than 1 mm diameter. Because of the latter, sonography can be used in young patients with positive family history to make early diagnosis.
II.2.9 (p129)
The incidence of optic disk drusen in children with accommodative esotropia
Sibel Oto, Şansal Gedik, Sezin Bayar, Burcu Kahraman, Veysi Öztürk, Yonca A. Akova
Başkent University Department of Ophthalmology
Objective Optic disk drusen (ODD) occur in 0.3–2.4% of the population. However visible drusen are very rare in children, the incidence reported as 0.4%. We aimed to investigate the incidence of ODD in children with accommodative esotropia.
Methods The records of 143 patients diagnosed as accommodative esotropia between January 1996 and December 2006 and who were under 15 years of age when first presented to our clinic were reviewed. The database of ODD patients diagnosed during this time period were also evaluated.
Results Five children (3.5%) in accommodative esotropia group revealed ODD which became visible during the follow-up period. One child was emmetropic and had high AC/A ratio, this child only had a family history of ODD. The other four children had full accommodative esotropia with 4.00 to 8.00 D of hypermetropia. ODD data cumulated during the same time period consisted of 44 patients excluding the accommodative esotropia group and 3 of them were younger than 16 years of age.
Conclusion The incidence of ODD in children with accommodative esotropia were found to be higher than the expected population incidence. Although the general belief is that the refractive error distribution of eyes with ODD parallels with the general population we presumed this high rate of incidence in our group of patients may be due to the presence of hypermetropia. Patients with accommodative esotropia should be monitored for ODD.
II.2.10 (p146)
Upgaze palsy due to hematoma that happen after ventriculoperitoneal shunt overflow
Yusuf Uysal, Ríza Güngör, Güngör Sobaci˙
GATA Department of Ophthalmology, Ankara-Turkey
Objective The dorsal midbrain syndrome of Parinaud usually results from damage to premotor pathways for binocular upwardgaze in the dorsal mesencephalon.
Case This report describes a 9-year-old boy who has all of the essential features suggestive of Parinaud's syndrome, caused by pretectalglioma leading hydrocephalus. Upward gaze palsy was come out after the development of left subdural hematoma, caused by overflow of the shunt. Hematoma was evacuated and shunt was adjusted. Ten months later, there was only binocular upward gaze palsy. The best corrected visual acuities were20/20 and there were optic disc temporal paleness in both eyes.
Conclusion The dorsal midbrain syndrome of Parinaud can happen due to hematoma resulting from ventriculoperitoneal shunt overflow.
II.2.11 (p158)
Optical disc changes in children with myopia on date OCT
A.G. Aubakirova, G.K. Toxanbaeva, Z.A. Jumataeva, A.S. Mustafaeva
Kazakh Eye Research Institute
Purpose To study optical nerve in children with different stages of myopia using optical coherence tomography.
Materials and Methods A total of 46 children with myopia (72 eyes) aged 7–14 years were examined to detect changes in the optic nerve due to myopia.
Examination was performed using OCT–Stratus 3000 (Carl Zeiss) on cycloplegia (propokaine 1%) ratio of disc excavation to vertical and horizontal diameters of disc and volume of neuroglial ring were evaluated. Axial length was measured in all children. Patients were divided into 3 groups: (1) composed of 12 patients (20 eyes) with myopia up to 3.0 diopters and axial length 24.3 ± 0.4 mm; (2) composed of 14 children (26 eyes) with myopia 3.25-6.00 diopters, axial length 25.5 ± 0.7 mm; (3) composed of 20 children (26 eyes) with myopia greater than 6.0 diopters and axial length 26.4 ± 0.2 mm.
Results Examination showed that excavation of 0.3-0.4 detected ophthalmoscopically was found in 37.5% of patients and in all patients using OCT of examination ratio to disc diameter shows a tendency to rise in connection with myopia grades and axial length measurements increase and volume of neuragliae ring was diminished.
In group 1, ratio of examination was 0.417 ± 0.05 to vertical diameter and was 0.502 ± 0.05 to horizontal, neuragliae ring volume–0.9 ± 0.25 (p < 0.05). In group 2, 0.436 ± 0.05 and 0.512 ± 0.01, respectively neuraglial ring volume-0.7 ± 0.17 (p < 0.05). In group 3, vertical diameter increased to 0.531 ± 0.03, neuraglial ring 0.4 ± 0.04 (p < 0.01).
Conclusion examination of optical disc on OCT revealed compression and deformation of glial base of optical nerve and neural fiber layer thinning correlated with myopia and axial length increase.
II.2.12 (p159)
Neurological status in children with strabismus and disbinocular amblyopia
T.K. Botabekova, A.Zh. Aubakirova, G.K. Toksanbayeva, A.D. Tapenbayeva
Kazakh Eye Research Institutå
Purpose to study the neurological status in children with strabismus and disbinocular amblyopia.
Material and Methods There were 78 children between age 2 and 15 years old with strabismus and disbinocular amblyopia of different degree. Among them were 15 children with very high amblyopia, 21 with high amblyopia, 24 with moderate, and 18 with low amblyopia. All children had pathopsychological and neurological observation with echoencephalography along with ophthalmological observation.
Results Pathology of central nervous system in most cases manifest as hypertension syndrome and arises from organic residual lesion of brain (65.8%) and astheno-neurological syndrome (32.4%). The cases of increase of the head circumference were noted to be twice as frequent among children with very high amblyopia than among children with high and moderate amblyopia. Tremor of hands, hyperreflexia were in 60 % of cases and only among children with very high amblyopia.
Two-thirds of children with very high and high amblyopia had obtruding motions and different mimic stereotypes. Such symptoms among children with moderate and low amblyopia were noted in 22.2%. Disorders of coordinator area and “soft” neurological symptoms among children with very high amblyopia were prevalent in 66.7%, among children with high amblyopia in 76.2% and among children with moderate and low degrees of amblyopia were noted twice less frequently. Children with very high amblyopia had intellectual disorders, reduction of mental outlook and abilities for analytical thinking, difficulties with changing the focus of attention, monotony of verbal reaction.
Echographic observation revealed the disorder of liquor circulation among children with very high amblyopia. The increase of third ventricle to 7∼mm was noted among 69.2%, an increase to 5.5–6.0 mm among 30.8% of children with high amblyopia the third ventricle showed an increase to 6 mm. Children with moderate and low degrees of amblyopia who showed an increase of the third ventricle to 6 mm made up 16.7%.
Thus the results of observation show that children with very high amblyopia have a more difficult neurological status, which makes it necessary to use additional correction to their treatment.
II.2.13. (p111)
Features of a visual nerve at children with atrophy of a visual nerve in combination with anomalies of refraction
T.K. Botabekova, A.J. Aubakirova, V.R. Nigmatulina
Kazakh Eye Research Institute, Almaty, Kazakhstan
Background By the data of some authors' anomalies of refraction quite often accompanies with atrophy of a visual nerve. So the study of some parameters of functional activity of the visual analyzer is of interest at various degrees of anomalies of refraction with atrophy of a visual nerve.
Purpose The objective rating of a status of a disk of a visual nerve at anomalies of refraction and accompanying atropy of a visual nerve.
Materials and Methods 217 children with a pathology of refraction in the age of from 3 till 14 years were examined. Accompanying atrophy of a visual nerve was found out at 51 children (23.5%). Among examined children boys was 23 (45%), girls–-28 (55%). Bilateral atrophy of visual nerve was found out at 20 (39.2%) children, unilateral–-at 31 (60.8%). To all children carried out definition of a visual acute, biomicroscopy, electrorethinography research, optical cogherent tomography. The greatest quantity of children with the accompanying diagnosis atrophy of a visual nerve was revealed in the age of 8–9 and 13 years. All patients had been divided into groups depending on a floor, age, kind and degree of anomalies refraction, character of atrophy of a visual nerve.
Results Accompanying to anomalies of refraction atrophy of a visual nerve was found out in 23.5% of cases. Atrophy of a visual nerve more often met at the girls, thus basically was partial atrophy of a visual nerve. Complete atrophy of a visual nerve was diagnosed basically at children with inherent high degree myopia(80%), partial ftrophy of a visual nerve met more often at children with hypermetropy of an average degree (47.5%). By the data of optical cogherent tomography the size of vertical and horizontal diameters of the head of a visual nerve raised according to increase of a degree of myopia, and at increase of a degree hypermetropia was reduced. Volume of nervous fibres decreased with progression of refractive infringements. The average values of electrical sensitivity, electrical lability and critical frequency of merge flashing at myopia and hypermetropia essentially did not differ from each other and depend only on a stage of atrophy of a visual nerve.
II.3. Imaging—Poster Presentations
II.3.1 (p13)
Idiopathic episcleral venous engorgement
Golge Acaroglu, Semiha Eranil, Yasemin Ozdamar, Ferhan Ural, M Yasin Teke
Ministry of Health, Ulucanlar Eye Hospital, Ankara, Turkey
Case Report We report a 64-year-old patient with a history of slowly progressive visual loss in a “red eye” for 35 years. The patient was thoroughly investigated to find an orbital or neuro-ophthalmological etiology. Dilated episcleral vessels may be present as an idiopathic phenomenon (Radius-Maumenee Syndrome). In these patients, the pressure of the episcleral vessels is elevated and therefore the outflow from Schlemm's Canal is reduced.
Conclusion This leads toelevated intraocular pressure and secondary glaucoma.
II.3.2 (p24)
Homonymous hemianopia in multiple sclerosis. Case report with magnetic resonance findings.José Nieto Enríquez
Jesus Torres Blanch
Alicia Galán Terraza, José García-Arumí: Hospital Vall d'Hebrön, Barcelona
Purpose To describe a case of homonymous hemianopia in a 29 year-old lady diagnosed with multiple sclerosis.
Setting Hospital Vall d'Hebrön, Barcelona.Spain
CASE A 29-year-old lady presented to the ER complaining of bilateral visual loss. She could not precisely explain her symptoms and felt her pupils were more dilated than usual. She had been diagnosed with multiple sclerosis 3 years before, after an episode of retrobulbar optic neuritis in her right eye. She was currently under no treatment. Her visual acuity was 20/30 in both eyes and her pupils were normal. She failed to identify 10 out of 16 Ishihara plates in each eye. Funduscopy revealed normal optic discs. An automated visual field test was performed and showed a congruous left hemianopia. Magnetic resonance imaging was performed. T2 sequences revealed multiple hyperintense focal lesions in subcortical and periventricular white matter in both hemispheres. A large lesion with moderate enhancement after gadolinium was found affecting the right capsulo-thalamic region and was identified as responsible for the patient's left hemianopia. A three day regimen of 1g methylprednisol one per day was administered. Two months later, her visual field had returned to normal, as had her visual acuity (20/20) and color vision test with the Ishihara plates. Magnetic resonance imaging three months later found that the lesion had diminished in size and inflammatory activity. The patient is currently under maintenance therapy.
Comment Visual loss in multiple sclerosis is usually caused by optic neuritis, affecting most frequently the retrobulbar region of the optic nerve. However, large demyelinating lesions, sometimes with a mass effect, can also compromise the retrochiasmal visual pathway as in our case, producing a hemianopic field defect. In the ER setting, these cases may require quite a high level of suspicion as visual acuity may be preserved and patients may not be able to explain precisely their deficit. They could be misdiagnosed with bilateral retrobulbar optic neuritis, which is highly infrequent. Also, it may be the presenting sign of multiple sclerosis, as previously described in the literature. High dose intravenous steriod treatment may produce the complete resolution of the visual field defect.
II.3.3 (p31)
The spectrum of retinal arterial occlusions according to clinical features
Aylin Yaman, Gul Arikan, Meltem Fatma Soylev Bajin, Ali Osman Saatci
Dokuz Eylul University, School of Medicine, Department of Ophthalmology, İzmir, Turkey
Aim To demonstrate the classical and unusual types of retinal arterial occlusions together.
Subject and Methods We retrospectively analyzed our records to identify eyes with retinal arterial occlusions examined at the neuroophthalmology and retina units between January 1993 and January 2007.
Results The type of arterial occlusions are classified according to clinical appearance such as: ophthalmic artery occlusion, combined central retinal artery and vein occlusion, hemicentral retinal artery occlusion, central retinal artery occlusion with and without macular sparing, branch retinal artery occlusion with and without macular involvement, branch retinal arterial occlusion and ischemic optic neuropathy, isolated cilioretinal artery occlusion with and without macular involvement, cilioretinal artery occlusion associated with neuroretinitis, or central retinal vein occlusion.
Conclusion Though the classical retinal arterial occlusions can easily be diagnosed ophthalmoscopically, some eyes may have confusing clinical features due to associated combined involvements.
II.3.4 (p34)
Results of the pupillometry during videonystagmography in asymmetric pseudoexfoliation patients
Dr. Fatma Yulek1, Ody Dr. Ozlem Konukseven2, Dr. Hasan Basri Cakmak1, Dr. Nagihan Orhan1, Prof Dr. Saban Simsek1, Assoc Prof Dr. Ahmet Kutluhan2
1First Ophthalmology Department of Ataturk Training and Research Hospital; 2Otorhinolaryngology Department of SB Ankara Ataturk Training and Research Hospital
Purpose Investigating the function of the iris muscle dynamically in patients with asymmetric pseudoexfoliation syndrome by using pupillometry test of electronystagmography.
Methods Patients with clinically detected asymmetric pseudoexfoliation syndrome were included in the study. The pupillometry tests were carried out in the same baseline illumination level at the same time of the day with the pupillometry test of the videonystagmography of the Micromedical System. The system measures the pupillary diameter change in a selected time interval giving an idea about the dynamic pupillary function. We measured the change in pupillary diameter during (1) fixation to an accommodative target at 30 cm to measure accommodative response, (2) light reaction, (3) convergence (from 1 m to 15 cm) induced miosis and finally during the divergence (from 15 cm to 1 m) induced mydriasis. Mann Whitney U test was used for statistical analysis.
Results Fifteen patients with asymmetric PXS were involved in the study (mean age: 71.20Â ± 6.60, min: 57, max: 80, 6 females, and 9 males). The percent of change in pupillary diameter in one second (PCPD) during: (1) accommodation (PCPD: −2.68Â ± 2.24% in eyes without PXS and −2.21Â ± 2.04% in eyes with PXS) (2) light reaction (PCPD: −7.19Â ± 3.52% in eyes without PXS and −5.45Â ± 3.14% in eyes with PXS), (3) miosis with convergence (PCDP: −2.27Â ± 0.96% in eyes without PXS and −2.20Â ± 1.20% in eyes with PXS) and (4) mydriasis with divergence (PCDP: 2.13Â ± 1.06% in eyes without PXS and 1.84Â ± 0.87% in eyes with PXS) were not found to be statistically different between the two groups.
Conclusion Objective imaging methods to detect the earliest clinical signs of the disease such as pupillometry may be of great value for an accurate early diagnosis. Other reports about pupillary functions of PXS patients usually evaluate only pupillary diameters. This videonystagmography apparatus on the use of audiology clinics seems to be not detecting a difference in terms of iris movements in eyes with asymmetric PXS.
II.3.5 (p44)
Neuro-ophthalmologic findings in patients with occipital lobe infarction
Nazife Sefi Yurdakul, Ahmet Maden
Izmir Atatürk Education and Research Hospital, İzmir, Turkey
Purpose To report the neuro-ophthalmologic findings in patients with occipital lobe infarction.
Material and methods Our study included the cases presented to neuro-ophthalmology department with visual complaints due to occipital lobe infarction between June 2003 and January 2006. Complete neurologic and neuro-ophthalmologic examinations were performed; pupillary reactions, eye movements and color visions with Ishihara test were assessed. Central 24-2 SITA standard achromatic perimetry (Humphrey Field Analyzer II), cranial magnetic resonance imaging and carotid-vertebral color Doppler ultrasound were performed.
Results One woman and 4 men with a median age of 58 years (41–64 years) were evaluated. At the initial examination visual acuity was 10/10 ou in 2 patients, and ranged between 1/10 and 8/10 in the others. Color vision was defective in 2 patients and normal in the others. Pupillary light reactions, anterior and posterior segment examinations were normal in all patients. Visual field testing revealed bilateral homonymous hemianopia in 4 patients, and bilateral superior quadranopsia in 1 patient. On radiological examination, 1 patient had parieto-occipital lobe infarctions and 4 patients had occipital lobe infarctions. In 2 patients occipital infarction developed following coronary by-pass surgery. Color Doppler ultrasound revealed 50% stenosis on the right internal carotid, 70% stenosis on the left internal carotid in one of these patients. Of the other patients 2 had hypertension and coronary artery disease, and 1 had high cholesterol level as risk factors. At the last follow-up, 4 out 5 had 10/10 vision while visual field defects persisted.
Conclusions Retrochiasmal visual pathways can be damaged by transient or permanent cerebral ischemia and can cause homonymous hemianopia and quadranopsia. As infarction field heals, visual acuity may improve. Magnetic resonance imaging and visual field examination are important to determine the lesion localization.
II.3.6 (p45)
Ocular manifestations in patients with intracranial tumors
Nazife Sefi Yurdakul, Şeyda Uğurlu, Ahmet Maden
İzmir Atatürk Education and Research Hospital, İzmir, Turkey
Purpose To evaluate ophthalmologic manifestations in patients with intracranial tumours who admitted initially to ophthalmology outpatient clinic with ocular symptoms.
Material and methods The patients who admitted to neuro-ophthalmology department between August 1999 and January 2007 because of ocular symptoms, and found to have intracranial tumour with computed brain tomography or magnetic resonance imaging and neurologic examinations were included. Complete neuro-ophthalmologic examination including visual acuity, pupillary reactions, color vision, ocular motility, biomicroscopy of anterior segment, intraocular pressure measurement and dilated fundus examination was performed. Ocular manifestations and the types and locations of the intracranial tumours were evaluated.
Results The average age of 9 women (60%) and 6 men (40%) was 43.92 ± 11.50 years (range, 20–66 years) at the time of diagnosis. Initial symptoms were transient visual obscurations, visual loss or visual field defect in 12 cases (80%), and diplopia in 3 cases (20%). Neuro-ophthalmologic examination showed normal optic disc, pallor of optic disc, papilledema, and sixth nerve palsy. Visual acuity ranged between normal and acute loss of light perception bilaterally. Neurologic examination and cranial imaging methods determined craniopharyngioma (n =1), meningiomas (n =5; olfactory groove, tuberculum sellae, suprasellar, cranial fossa), and pituitary macroadenomas (n =9). Pituitary macroadenomas were more frequent in women (3 men, 6 women).
Conclusions The ophthalmologist may be the first physician to challenge with clinical manifestations of intracranial tumours that may cause devastating neurologic and ocular complications. Prompt diagnosis can provide institution of early treatment, hence avoidance of complications.
II.3.7 (p47)
A case of improvement of thyroid associated extraocular muscle hypertrophy following Botulinum toxin A injection
Hyosook Ahn, MD, Hyun Taek Lim, MD, Hoonbo Kim, MD, Hyung Hun Kim, MD
Department of Ophthalmology, Asan Medical Center, University of Ulsan, Seoul, Korea
Purpose We are to present a case of thyroid associated ophthalmopathy which showed a significant improvement of extraocular muscle hypertrophy after the injection of Botulinum toxin A.
Case A 45-year-old Asian woman with hyperthyroidism presented with a 3-month history of diplopia and ocular motility restriction. At the time of initial presentation, she had a severe limitation of abduction and a moderate limitation of supraduction and infraduction in the right eye. Prism cover test revealed a 10 prism diopter of esotropia and 8 prism diopter of right hypotropia in primary position. Orbit CT scan showed a prominent hypertrophy of medial and inferior recti muscles in the right side. She was treated by 10 units of botulinum toxin injections to the right medial rectus muscle three times consecutively, over duration of 15 months. After the three times injection of Botulinum toxin, a significant reduction in muscle volume of medial rectus was noticed in orbit CT scan.
Conclusion A Botulinum toxin injection may induce a significant improvement of extraocular muscle hypertrophy in patients with thyroid associated ophthalmopathy, especially when treated repetitively. It may result from rectus muscle atrophy as a long-term effect of Botulinum treatment.
II.3.8 (p57)
The heavy eye phenomenon, a rare condition in a twin
J.T.H.N. de Faber, E. Ganteris-Gerritsen, H. Geene, M. Wefers Bettink-Remeijer
The Rotterdam Eye Hospital, Rotterdam, Netherlands
Purpose to report a rare case of twin sisters with high myopia and heavy eye phenomenon.
Background The heavy eye phenomenon presents as progressive esotropia and hypotropia in high myopia. It appears to be due to an enlarged myopic globe that compresses the lateral rectus muscle against the lateral orbital wall resulting in a strabismus fixus. This condition has been described in both congenital and acquired form but are both very rare. Strabismus fixus is difficult to operate. One of the possible interventions is a Yokoyama procedure.
Methods We present a case of twin sisters with high myopia and heavy eye phenomenon by pictures and magnetic resonance imaging scans (MRI) of this condition and a short summary of the literature.
Conclusion To the best of our knowledge this is the first case in which the heavy eye phenomenon is reported in a twin.
II.3.9 (p66)
Isolated inferior rectus palsy revealing a brainstem cavernous angioma
M. Robert, C. Duncombe-Poulet, E. Emery, F. Viader, F. Mouriaux
University Hospital of Caen, France
Purpose To report a case of isolated inferior rectus palsy secondary to a pontine cavernous angioma.
Observation A 53-year-old man with a history of chronic renal insufficiency treated by dialysis presented with acute vertical binocular diplopia. The neuro-ophthalmic examination revealed a right inferior rectus palsy, without any other abnormality. The patient was seen two weeks later with the results of his check-up (orbital CT-scan and blood tests: TSH, T4, inflammatory proteins and anti-AChR Abs). No abnormality was found and the clinical examination was unchanged.
A brain MRI scan showed a focal lesion in the upper, ventral left paramedian part of the tegmentum pontis, consistent with an hemorrhagic cavernous angioma.
Discussion Brainstem lesions are an exceptional cause of isolated inferior rectus palsy. This is due to the discrete locations of both the inferior rectus subnucleus (in the dorso-lateral part of the oculo-motor nuclear complex) and the inferior rectus fascicle (classically situated much more medially and caudally than the other oculo-motor fascicles).
In this case the MRI-scan was performed quite early, despite the normal neurological and pupil examination and allowed us to modify the anticoagulation protocol of this dialyzed patient. Early imaging of the brainstem may thus be crucial in such clinical situations.
In our patient, the lesion is very anterior and medial, near the right inferior rectus fascicle. The location is intriguing and its significance concerning the precise anatomical location of the inferior fascicle is discussed.
Conclusion MRI-scan exploring the brainstem may be indicated early in isolated inferior rectus palsy, when no cause has been found.
II.3.10 (p68)
Evaluation of structural change of globe and optic nerve in cases with pseudotumor cerebri (PTC)
Aylin Yaman,1 Ziya Ayhan,2 Sinem Gezer,2 Suleyman Men,2 Meltem F Soylev1
1Department of Ophthalmology Dokuz Eylul University; 2Department of Radiology Dokuz Eylul University, İzmir, Turkey
Aim To evaluate structural changes of globe and optic nerve in cases with pseudotumor cerebri (PTC).
Method Ten patients with confirmed PTC (9 females, 1 male; mean age 32.5 ± 11.3) and 10 control cases (7 females, 3 males; mean age 37.6 ± 9.8) were evaluated retrospectively. The radiological evaluation was made on the cranial or orbital MR images retrieved from the PACS archive. On axial images of the orbit transverse diameters of the globe, thickness of the optic nerve and the perioptic cerebrospinal fluid (CSF) space were measured. The sagittal and axial images were evaluated for also posterior scleral flattening, vertical tortiosity of the optic nerve, protrusion of papilla and empty sella. All radiologic images were evaluated by the same neuroradiologist.
Results There was no statistical difference between PTC and control group for transvers/axial diameters of globe, thickness of perioptic CSF space, optic nerve and presence of protrusion of papilla. (Mann–Whitney U test; p > 0.05). Posterior scleral flattening and vertical tortiosity of optic nerve were significantly associated with PTC (Fisher Exact Test; p < 0.05) Although statistically insignificant in Fisher Exact Test (p > 0.05), empty sella was found in 60% of patients with PTC at neuroradiologic images.
Conclusions Although there are many reported signs at cranial and orbital MR images in patients with PTC, only posterior scleral flattening and vertical tortiosity of optic nerve were suggestive of diagnosis of PTC in our study.
II.3.11 (p70)
Pulsatile proptosis
MA. Arcediano1, B.F. Sanchez-Dalmau2, L. Pascual1, M. García-Bach3, J.L. Dolz4
1Ophthalmology, Hospital Mütua de Terrassa, Barcelona; 2Institut Clinic d'Oftalmología (ICOF), Hospital Clínic, Barcelona; 3Neurosurgery, Hospital Mütua de Terrassa, Barcelona; 4Radiology Services, Hospital Mütua de Terrassa, Barcelona
Introduction Pulsalite proptosis can be caused by different etiologies, as distensible venous vascular malformation (orbital varix, lymphangioma, capilar hemangioma), arteriovenous fistulas and congenital absence or destructive lesions of the orbital roof. We report a case of acquired pulsatile proptosis and its etiology, clinical manifestations, and treatment are revised.
Methods Observational case report A 36-year-old man was referred for consultation because of a proptosis in his left eye. On examination, a moderate proptosis was found with displacement of the globe down and outwards. The proptosis was pulsatile and increased with Valsalva's maneuver but not with compression on the ipsilateral jugular vein. A contrast enhanced CT scan with raised jugular venous pressure was made showing an absence of the bony roof of the orbit, mainly in the posteromedial portion, with prolapse of intracranial structures into the superior orbit. An MRI of the brain revealed the presence of an arachnoidal cyst occupying the anterior portion of the left temporal lobe extending to the adjacent orbit and parasellar region; it also produced a displacement of the pituitary stalk and a distortion of the optic nerve along its channel. All these findings confirmed the diagnosis of an orbital meningoencefalocele.
Conclusion Orbital meningoencephalocele is a rare disease of the orbit. Although they mainly occur in the early childhood, we should to keep in mind this diagnosis when approaching a pulsatile proptosis in an adult. Imaging techniques are essential to find out the diagnosis and its etiology. Management is surgical and requires the intervention of both neurosurgeon and the ophthalmologist
II.3.12 (p82)
Jerky seesaw and hemi-seesaw nystagmus: clinico-radiologic correlation
Seong-Hae Jeong1, Young-Mi Oh1, Hyo-Jung Kim1, Eung Kyu Kim2, Jun Lee3, Kyung-Mi Oh4, Kwang-Dong Choi1,5, Seong-Ho Park1, Ji Soo Kim1
1Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, South Korea; 2Pusan Paik Hospital, South Korea; 3Yeungnam University Hospital, South Korea; 4Korea University School of Medicine, South Korea; 5Pusan National University School of Medicine and Medical Research Institute, South Korea
Background The mechanism of acquired jerky seesaw and Hemi-seesaw nystagmus remains unknown.
Objective The aim of this study was to elucidate the mechanism of acquired seesaw and Hemi-seesaw nystagmus by analyzing the patterns of nystagmus and measuring the individual semicircular function.
Methods Ten consecutive patients with acquired seesaw and Hemi-seesaw nystagmus underwent recording of the nystagmus using three-dimensional video-oculography or magnetic search coil technique. In one patient, the vestibulo-ocular reflex from individual semicircular canal was determined by using head impulse maneuver. All the patients had MRI of the brain.
Results In 6 patients, the nystagmus was conjugate torsional with disparate vertical components (seesaw nystagmus) while it was mainly torsional in one eye and mainly vertical in the contralateral eye (hemi-seesaw nystagmus, n=4). Of the 4 patients with Hemi-seesaw nystagmus, the vertical component was upbeat in 3 and downbeat in one. Internuclear ophthalmoplegia (INO) was associated with nystagmus in 6 and it was an isolated finding in 5 of them. Lesions in MRI were located in the pontine tegmentum (n=6), in the territories of the posterior and anterior cerebellar arteries (n=1), in the medial medulla (n=1), and in the paramedian thalamus (n=1). In one patient, no lesion was detected in MRI. In one patient with seesaw nystagmus and INO from pontine lesions, head impulse tests disclosed impaired VOR from the anterior and posterior semicircular canals contralateral to the paretic eye.
Conclusion Acquired jerky seesaw and Hemi-seesaw nystagmus mostly develop in lesions involving the pontine tegmentum where the medial longitudinal fasciculus locates, and commonly accompanies signs of INO. Hemi-seesaw nystagmus may result from damage to the pathway from the contralesional anterior or posterior semicircular canal while the seesaw nystagmus may develop owing to disruption of the pathways from both canals.
II.3.13 (p97)
Correlation of Macular pathology with multifocal Electroretinography (mfERG) recordings in patients with age related macular degeneration (AMD).
R. Karanjia, K. Eng, J. Gale, S. Sharma, M. W. ten Hove
Department of Ophthalmology, Hotel Dieu Hospital, Queen's University
Purpose To examine the sensitivity of mf-ERG to measure changes in retinal electrical activity in response to treatment for AMD.
Methods Fifteen subjects with exudative AMD were recruited over a four month period at a tertiary care centre. The subjects selected were naíve to Avastin (bevacizumab) in the investigated eye and underwent pretreatment testing with mf-ERG and intravenous fluorescein angiography (IVFA). A second mf-ERG test was conducted seven to fourteen days post-treatment. The location and size of the lesion was assessed on the IVFA. A custom overlay was used to approximate the retinal area associated with each waveform using a standard distance of 15° from the fovea to optic disc. The P1 response amplitudes were examined pre and post-bevacizumab for the hexagons corresponding to areas of maximal pathology. Inter-test variability was accounted for by areas without pathology and only pairs of recording where there was no significant difference in responses in these areas between tests were utilized.
Results There was a significant improvement in P1 response amplitude from lesion associated recordings in 60% of those who showed a clinical improvement in visual acuity post-bevacizumab. In four subjects the P1 response from the area of highest fluid accumulation was significantly increase post-treatment, irrespective of clinical outcome (43% ± 13 increase; p < 0.008). In addition, in three patients where the visual acuity showed improvement, the amplitude of P1 in areas where fluid was identified on IVFA was significantly increased relative to baseline (17% ± 5 increase; p < 0.03).
Conclusion This study represents a novel method for assessing an improvement of mf-ERG responses. We were able to detect an electrophysiological change which correlates with improvements in visual acuity post-bevacizumab treatment. This study demonstrates that mf-ERG is a useful modality for identification of localized electrical disturbances in the macula caused by pathologic processes such as AMD. Furthermore, mf-ERG provides a potential method for assessing treatment outcome over time. The ability of mf-ERG responses to predict changes in visual acuity warrants further investigation.
II.3.14 (p98)
Optimization of environmental conditions through electrophysiological analysis of pattern visual evoked potential recordings
R. Karanjia1, D. Brunet2, M. W. ten Hove1
1Department of Ophthalmology, Department of Medicine, Queen's University, Kingston, Canada; 2Division of Neurology, Department of Medicine, Queen's University, Kingston, Canada
Purpose To explore the influence of environmental conditions on pattern VEP recordings.
Methods Fourteen subjects with no known ocular pathology were recruited for the study. All recordings were conducted using ISCEV protocol with check sizes of 16 × 16, 64 × 64, and 128 × 128. In an attempt to optimize the recording conditions, VEP recordings were preformed in both the seated and recumbent positions. Comparisons were made between recordings using either LCD or CRT displays and recordings obtained in silence or with quiet background music. Paired recordings (in which only one variable was changed) were analyzed for changes in P100 latency, RMS noise, and variability. Recording sequences were varied to negate subject fatigue and same subject recordings from two separate sessions were used as internal controls.
Results Baseline RMS noise of 60 recording pairs demonstrated a significant decrease in the variability during the first 50 msec for recumbent position compared to the seated position (p < 0.05) for check sizes of 16 × 16 and 128 × 128. Analysis of the one hundred data points which comprised the initial 50 msec revealed a significant decrease in the amount of variability in the recumbent position of 57% of the pairs versus only 10% favoring the seated position. Analysis of VEPs recorded using LCD monitors demonstrated a significant increase (>25 msec) in the P100 latency when compared to CRT recordings in the same subjects. Similarly the use of a low contrast CRT monitor also increased the latency of the P100 response. The addition of background music did not affect the amount of RMS noise during the first 50 msec of the recordings.
Conclusion This study demonstrates that the use of the recumbent position increases patient comfort and improves the signal to noise ratio. In contrast, the addition of background music to relax the patient did not improve the recording signal. Furthermore, the study illustrates the importance of avoiding low-contrast visual stimulation patterns obtained with LCD or older CRT monitors as they lead to higher latencies resulting in false positive recordings. These findings are important when establishing or modifying a pattern VEP recording protocol.
II.3.15 (p100)
Quadrantanopic visual field loss as the initial presentation of temporal lobe cavernous angioma
Randhir Chavan, Bal Manoj
Royal Wolverhampton Hospitals Nhs Trust, Wolverhampton,United Kingdom
Purpose To report the case of a quadrantanopic visual field loss as the only finding at initial presentation of a temporal lobe cavernous angioma.
Methods Observational case report. A 14 year old boy presented with a 3-day history of a patch of letters missing while reading. He had no headaches or any neurological symptoms. Ophthalmologic and neurologic examinations revealed no abnormalities except for a right superior homonymous quadrantanopia. Magnetic resonance imaging (MRI) was arranged for the following week. Unfortunately the boy presented in A&E on the MRI day with headache, nausea & vomiting along with visual hallucinations.
Results MRI showed a 3 × 4 cm well-defined heterogenous mass in the medial cortex of left temporal lobe. T1 images suggested recent hemorrhage within it. It was also obstructing the temporal horn of the lateral ventricle. A provisional diagnosis of left temporal lobe hematoma was entertained and urgent left temporal craniotomy was performed with partial evacuation of hematoma. All his neurological symptoms including headache and hallucinations resolved and subjectively his field defect was reduced to only a small patch postoperatively. Subsequent T2 weighted MRI showed typical reticulated “popcorn-like” lesion with a peripheral low signal rim of hemosiderin suggesting cavernous angioma in the temporal lobe. In view of the angioma being deeply situated in a critical region with no further signs of hemorrhage so far a conservative approach was contemplated.
Conclusion The treatment of symptomatic cavernous angiomas is controversial. Particularly if the lesions lie in critical brain regions as in our case, observation is usually recommended as the operative risk is often more than the risk of re-bleeding or growth. This case is exceptional in that most cavernous angiomas do not become symptomatic before the third decade of life and rarely attain such a large size. Also initial presenting sign was only a superior quadrantanopic visual field defect. To the best of our knowledge this is the first reported case of a cavernous angioma presenting in pediatric age group initially only with a field defect.
II.3.16 (p104)
Evaluation of visual function in multiple sclerosis patients
Victoria Pueyo, M. Carmen Egea, F. Javier Fernandez, Noemi Guerri, Carmen Almárcegui, Jesus Martin, Francisco M. Honrubia.
Ophthalmology, Neurophysiology, Neurology Departments, Miguel Servet University Hospital, Zaragoza, Spain
Purpose To evaluate the visual function of patients with relapsing-remitting Multiple Sclerosis (MS) and its correlation with functional disability.
Patients and methods One hundred fifty eyes from 50 patients with MS and 25 healthy age- and sex-matched controls were included in this study. All patients underwent neurologic assessment and complete ophthalmologic examination (visual acuity, color vision, visual field, visual evoked potentials, optical coherence tomography and scanning laser polarimetry).
Results RNFL thickness measured by means of Optical coherence tomography (OCT) and Scanning laser polarimetry (GDx) was significantly reduced in eyes of MS patients compared with age- and sex-matched controls (RNFL average thickness 92 nm versus 104 nm; NFI 24 versus 16; p < 0.05). This difference was greater in patients with MS and optic neuritis history (RNFL average thickness 84 nm; NFI 30) but there was also observed in eyes with no history of optic neuritis (RNFL thickness 94 nm; NFI 21). There was good correlation between functional and structural assessments of visual function. However, no correlation between RNFL measurements and functional disability was observed.
Conclusions Optical imaging technologies, such as Optical coherence tomography and Scanning laser polarimetry, are able to detect axonal loss in the optic nerve of patients with MS, even in cases with no history of optic neuritis.
II.3.17 (p105)
Comparison of functional and structural technologies in optic neuritis diagnosis and follow-up
Victoria Pueyo, Noemi Güerri, F. Javier Fernandez, M. Carmen Egea, Esteban de Vicente, Francisco Rabinal, Francisco M. Honrubia.
Ophthalmology and Neurophysiology Departments, Miguel Servet University Hospital, Zaragoza, Spain
Purpose To evaluate the ability of some functional and structural technologies to detect changes in time in eyes affected by optic neuritis.
Patients and methods Ten eyes with acute optic neuritis were evaluated and followed-up during 6 months. They were compared with ten sex- and age-matched healthy eyes. All patients underwent a complete ophthalmologic examination (visual acuity, color vision, visual field, optical coherence tomography, and scanning laser polarimetry) in the acute moment, in one month and in six months. Visual evoked potentials were also performed at the beginning. The functional and structural parameters obtained in the optic neuritis group were compared with healthy eyes and their changes in time were evaluated.
Results Eyes with acute papillitis showed increased RNFL thickness measured by OCT in superior quadrants compared with healthy eyes (151 nm vs. 131∼nm) and significant differences in MD visual field and VEP latency. Only functional abnormalities were initially observed in eyes with posterior optic neuritis. In 6 months of follow-up, significantly decreased RNFL thickness measured by means of OCT (69 nm in papillitis and 83 nm in posterior optic neuritis) and GDx was observed in eyes with either papillitis or posterior optic neuritis.
Conclusions Structural analysis of the RNFL by means of Optical coherence tomography and Scanning laser polarimetry is useful in optic neuritis diagnosis and follow-up.
II.3.18 (p115)
Sixth nerve palsy secondary to dolichoectasia basillary artery
A. Bures1, B.F. Sanchez-Dalmau1, L. Pascual2, A. Aranda1, A. Adán1
1Institut Clinic d'Oftalmologia. Hospital Clinic, Barcelona; 2Hospital Mutua de Terrassa
Introduction Dolichoectasia (elongation, dilatation and tortuosity) of the basilar artery can produce cranial nerve dysfunction either by compression or ischemia.
The trigeminal and facial nerve are the most commonly affected nerves in basilar dolichoectasia but other cranial nerve palsies have also been described in the literature.
We present two cases of isolated sixth nerve palsy secondary to dolichoectatic basilar artery.
Methods: Observational case report
Case 1 A 68-year-old male presented with an acute onset horizontal binocular diplopia due to a left sixth nerve palsy. Past medical history was significant for systemic hypertension and hypercholesterolemia and the palsy was initially oriented as microvascular. After three months the palsy showed no signs of recovery and neuroimaging was arranged.
Neuroimage showed dilatation and tortuosity of basilar artery around midbrain and compression of the root of the left abducens nerve.
Case 2 A 73-year-old female presented with a 10-month history of progressively worsening horizontal binocular diplopia. Ophthalmologic examination revealed a left sixth nerve palsy. Medical history was otherwise non-significant.
Neuroimage was arranged and the brain MRI showed dolichoectasia of the basilar artery.
DiscussionBasilar artery dolichoectasia is an uncommon cause of cranial nerve palsy, but has to be included in the differential diagnosis of isolated sixth nerve palsy.
Microvascular etiology is the most frequent cause of sixth nerve palsy but other causes have to be excluded when there are no vasculopathic risk factors or when the palsy shows worsening or no signs of recovery 8 weeks after onset.
Computed tomographic angiography (CTA) and magnetic resonance angiography (MRA) are the preferred techniques to identify dolichoectatic vessels.
Clinical course may be variable and surgical treatment of the dolichoectasia is usually not recommended in cranial mononeuropathies since the risk of surgery outweighs its benefits.
II.3.19 (p116)
Reflex blepharospasm associated with non-dominant parietal lobe infarct
Canan Togay Işíkay, Başak Bolluk, Nurhan Yílmaz, M. Cenk Akbostanci˙
Ankara University Faculty of Medicine, Department of Neurology, Turkey
Blepharospasm is involuntary bilateral eye closure produced by spasmodic contractions of the orbicularis oculi muscles. When it occurs only in response to certain stimuli, such as sudden visual threat or auditory or tactile stimuli, the term “reflex blepharospasm” (RB) is used. RB is seen in premature infants and patients with various Parkinsonian syndromes, and a variety of ocular disorders. RB in stroke patients with non-dominant temporoparietal lobe lesions was first defined by Fisher. We present two cases of RB associated with acute stroke due to non-dominant parietal lobe infarct.
Case 1 A 76-year-old woman was admitted with acute left sided weakness. She had chronic atrial fibrilation and several strokes in medical history without residual deficits. Neurological examination revealed dysarthria, facial paresis, hemiparesis, and Babinski sign on the left, and bilateral RB predominantly on the right eye. Cranial CT showed acute infarct in the right parietal lobe, and multiple chronic infarcts elsewhere.
Case 2 A 70-year-old woman was admitted with acute left-sided weakness. Neurological examination revealed facial paresis and mild hemiparesis on the left. Cranial CT showed right parietal infarct. This patient deteriorated on the fifth day of admission; she became hemiplegic, and developed RB. Spontaneous eye opening and eye opening to sudden visual threats and verbal commands were normal in both patients. RB was observed only to tactile stimulation (when we tried to open the patient's eyes), and disappeared on the eighth day of the presentation.
RB, which is a sign that can be easily overlooked or misinterpreted as incooperation to the neurological examination should be remembered in the case of ischemic stroke due to non-dominant parietal lobe infarct.
II.3.20 (p121)
Art photography: difference of light drawing in photography and light drawing and perception in eye makes from artificial imaging an “art”
Hilmi Or
Private Practice, Istanbul, Turkey
Aim To show similarities and differences between photographic (photo-graphic=light drawing) and ocular light drawing and perception in order to be perceived as an art.
Material and methods: The optical image drawing in photographic equipments and used techniques in art photography are compared with the optical image anatomy and physiology of the eye and image perception in brain. The author is ophthalmologist and has also the title “Artist of International Federation of Photography” (AFIAP).
Results: The similarity in photography and the eye in light-drawing and perception is the psychological factor of the “known” and “usual”. Due to differences in sharp areas of the image, depth perception, motion imaging and perception, color perception, light adaptation, entoptic phenomenon and other factors between the eye and photographic equipment there is non-similar light-drawing and perception between the eye and photographic equipment. These factors are discussed in detail.
Conclusion: Differences of the imaging in eye with the perception in brain and imaging in photography let photography be not a image of the reality but an “art”.
II.3.21 (p123)
Intermittent uniocular mydriasis associated with ipsilateral aberrant vascular loop
Abbott J, Matthews TD
Birmingham Neuro-ophthalmology Unit, UK
Method Case report supplemented by MRI images.
Findings A 39-year-old with long history of recurrent headaches associated with photophobia and nausea developed intermittent left mydriasis as a feature of these episodes. The neurological symptoms and signs resolved within 12 h on each occasion. MRI scanning revealed an atypical vessel immediately adjacent to the left oculomotor nerve.
Conclusion Mydriasis has been reported in association with migraines and any radiological etiology is usually excluded by normal imaging. This is the first case, which we are aware of to show a possible structural cause for intermittent mydriasis.
II.3.22 (p133)
Multifocal ERG in neuro-ophthalmic practice: distinguishing bilateral optic neuropathy from clinical occult maculopathy
Antonella Boschi1, Isabelle Ingster-Moati2, Kristian Klemp3, Bernadette Snyers1, Michel Paques4, Joseacute; Sahel4, Phuc LeHoang5, D Milea3,5
1Cliniques Universitaires St Luc, Brussels, Belgium; 2Université Paris 7 and Ophtalmology Department, Hôpital Necker, Paris, France; 3Glostrup University Hospital, Copenhagen, Denmark; 4CHNO XV-XX and Fondation Ophthalmologique Rothschild, Paris; 5Pitié-Salpêtriére Hospital, Paris, France
IntroductionBilateral painless visual loss associated with color vision defects and central scotomas is usually suggestive of a bilateral retrobulbar optic neuropathy, if ocular fundi are normal. However, in a small number of cases it can be difficult to differentiate between a bilateral optic neuropathy at an early stage and a clinically occult maculopathy. The aim of this study was to asses the use of multifocal ERG (mfERG) in this setting.
Patients In this retrospective series, we describe 9 patients, initially referred for a bilateral optic neuropathy of unknown origin, with ophthalmoscopically normal fundi. In some of these cases, an additional non-organic, functional cause was also sometimes suspected. In most of the cases, a variable combination of the following examinations were initially performed, with no conclusive results concerning the origin of the optic neuropathy: fluorescein angiography, optical coherence tomography, full-field ERG, VEP, neuroimaging, cerebrospinal fluid analysis.
Results In each patient, N1, P1, and N2 implicit times and amplitudes of the first order mfERG were recorded. Local responses and concentric ring averaged responses were measured and compared to age-matched normal subjects. Results showed reduced amplitudes for N1, P1, and N2 in the foveal, perifoveal and parafoveal areas in these patients compared to normal subjects, indicating dysfunction of the macula and integrity of the visual pathways.
ConclusionClinically occult maculopathies can initially mimic a bilateral optic neuropathy at its early stages, since they can present with variable onset of visual loss, bilateral central scotomas and no ophthalmoscopic changes, occurring at any ages, even in absence of family history. Electrophysiology and more specifically mfERG is the definitive diagnostic criterion for cone dystrophies and other occult maculopathies. The early use of mfERG could avoid useless and expensive neuroimaging procedures.
II.3.23 (p134)
Clinical and radiological comparisons of thyroid orbitopathy with and without optic neuropathy
Naciye Aktas, Golge Acaroglu, Yasemin Ozdamar, Jale Karakaya, Bayazit İlhan, Tulay Simsek, Orhan Zilelioglu
Ministry of Health, Ulucanlar Eye Hospital, Ankara
Purpose To compare the clinical and radiological parameters in thyroid orbitopathy (TO) with and without optic neuropathy.
Material-Methods Clinical findings and orbital magnetic resonance images (MRI) were reviewed. Dysthyroid optic neuropathy (DON) patients who had axial and coronal orbital MRIs were extracted from the archives of our neuroophthalmology department and consecutive TO patients without optic neuropathy were prospectively enrolled in the study. Statistical comparisons were made utilizing SPSS software, evaluating each orbit independently. Comparisons were made in terms of intraocular pressure, degree of exophthalmos, clinical activity score and motility disturbance. Radiological muscular indices were calculated from orbital MRIs.
Results DON patients were significantly older (p: 0.03). There were 15 orbits with; and 45 orbits without optic neuropathy. Statistically significant differences were found in the two groups for all parameters studied. Orbits with DON seemed to have more pronounced inflammatory activity (p: 0.00), and contained more exophthalmic globes (p: 0.013) with higher mean intraocular pressures (p: 0.001); however, the presence of established glaucoma was not statistically confirmed (p: 0.208). Globe motility disturbances (p: 0.000 and p: 0.010) and larger extra ocular muscle volume/orbital volume ratios were present in orbits with DON, as demonstrated by significantly greater muscular indices (p: 0.022 and p: 0.007). Strong statistical significance of horizontal motility disturbance and axially calculated horizontal orbital index incorporating medial rectus muscle were noteworthy.
Conclusion This study suggests that TO patients having aforementioned characteristics are more likely to have coexisting or incipient optic neuropathy; therefore should be closely monitored.
II.3.24 (p147)
Ocular and systemic findings after intravascular treatment of spontaneous cavernous dural arteriovenous malformations
M. Amaro, MD, A. Sulzbacher, MD, M. Lourdes Vieira, MD, J. Campos, MD, A. Castanheira-Dinis, MD
Lisbon Institute of Ophthalmology (Instituto de Oftalmologia Dr. Gama Pinto-IOGP) and Neuroimaging Department of Hospital Santa Maria, University of Lisbon, Portugal
Introduction Cavernous dural arteriovenous malformations (CDAVM) are abnormal shunts and connections that occur within the cavernous sinus. A CDAVM results in high pressure arterial blood entering the low pressure cavernous sinus. Most CDAVM are of spontaneous origin and unknown ethiology, representing approximately 12% of all dural shunts. Depending on the shunt flow, arterial pressure and venous drainage, hemodynamic consequences will emerge, mainly those concerned with ocular manifestations. Treatment is recommended for intolerable bruit, progressive visual loss, and for proptosis.
Material and MethodsA retrospective database review and transversal non-comparative neuro-ophthalmological study of patients with the diagnosis of spontaneous CDAVM that were submitted to intravascular treatment and follow-up by the Neuroimaging Department at Santa Maria Hospital-University of Lisbon between 2000 and 2006.
Some months after the endovascular treatment we performed neuro-ophthalmologic examinations and epidemiologic studies as well as blood analysis to identify pre-thrombotic risk factors in order to obtain information about the probable etiology.
Results We evaluated 7 patients who had been submitted to endovascular treatment ranging from ages 52–64; average age 58.4 years, (42.8% females and 57.1% males). A complete angiographic resolution of the fistula was obtained in all cases. Six patients had cardiovascular risk factors, such as: diabetes, hypertension and hypercholesterolemia.
Neuroophthalmologic abnormalities after embolization consisted of high intraocular pressure (4 patients), constricted visual fields (6 patients) and electrophysiologic disturbances presented as a diminished amplitude of flash and pattern PEV (5 patients). Six patients had blood analysis confirmation of pre-thrombotic risk factors.
Conclusion Although CDAVM closure was obtained after endovascular treatment, neurophthalmological signs have been persistant for several months, which unveils the importance of the ophthalmological follow-up as well as the control of systemic risk factors.
II.3.25 (p149)
Occipital and cerebellar arteriovenous malformations: Neuroophthalmologic manifestations
M. Lourdes Vieira, MD, A. Sulzbacher, MD, M. Amaro, MD, J. Campos, MD, A. Castanheira-Dinis, MD
Lisbon Institute of Ophthalmology (Instituto de Oftalmologia Dr. Gama Pinto-IOGP) and Neuroimanging, Department of Hospital Santa Maria, University of Lisbon, Portugal
IntroductionThe occipital and cerebellar localizations of intracranial vascular malformations (AVMs) cause a variety of visual disturbances, as a mode of presentation or after neurosurgical treatments.
Material and MethodsThe sample of our study is based on a population of 107 patients with retrochiasmatic AVMs, treatment and follow-up by the Neuroimanging Department of Santa Maria Hospital at University of Lisbon between 2000 and 2006.
We performed a retrospective review and transversal non-comparative neuro-ophthalmologic study of 11 patients with occipital and 10 patients with cerebellar arteriovenous malformations.
We investigated some months after the treatment, the ocular and visual neuro-ophthalmologic disturbances and epidemiologic studies, in order to obtain information about ocular and visual findings.
Results Eleven patients with occipital AVMs with symptoms of intracranial bleeding (five patients), seizures (four patients), headaches with differential diagnosis, migraine (four patients), and four with eloquent visual disturbance; The group with cerebellar AVMs were ten with symptoms of intracranial hematoma (nine patients), asymptomatic (one patient).
All the patients had been treated with embolization before surgery and/or radiosurgery. The final results of AVMs in the occipital area showed as a main aspect, visual field loss, associated with some cortical disturbances such verbal alexia and hemiacromatopsia; the cerebellar AVMs all of them having had oscillopsia, that has been reversible; there remain four cases of oculomotor palsies.
Conclusion In the occipital AVMs the visual incapacities were prominent. The differential diagnosis between headaches and migraines are important to attain an early diagnosis and adequate treatment. The visual field improvement can spontaneously occur when the loss was secondary to intracerebral bleeding, or it can occur after surgical excision. The cerebellar AVMs showed less visual impairment, the four oculomotor palsies were not disabling.
II.4. Myopathies, Motility Disorders—Poster Presentations
II.4.1 (p8)
Vertical smooth pursuit in congenital idiopathic nystagmus (CIN)
Archana Pradeep, Rebecca Mclean, Frank Proudlock, Irene Gottlob
Ophthalmology Group, University of Leicester
Purpose In most CIN, horizontal pursuit is abnormal. We wanted to compare and assess the vertical pursuit function in subjects with idiopathic nystagmus and controls.
Methods Horizontal and vertical eye movements at 250 Hz were measured in patients and controls with a target velocity of 20 degree/sec and amplitude of +/−20. Gain was measured in along the vertical axis.
Results Pursuit gain were 0.74 (SD 0.20) and 0.70 (SD 0.14) in nystagmus subjects and 0.72 (SD 0.22) and 0.65(SD 0.15) in controls for elevation and depression respectively. Consequently there is no significant difference in vertical smooth pursuit gain between idiopathic nystagmus subjects and controls.
Conclusion We found the pursuit gain for vertically moving target is similar in nystagmus subjects and controls.
II.4.2 (p12)
Self-paced repetitive saccades and saccadic latencies in predictive saccadic tracking
H. Tegetmeyer
University of Leipzig, University Eye Hospital, Liebigstr. 10-14, D-04103 Leipzig, Germany
Objectives To determine the frequency of self-paced repetitive saccades between stationary visual targets and to correlate the findings with the latencies of repetitive saccades to alternating targets in a square wave tracking paradigm.
Methods Six normal subjects made voluntary repetitive horizontal saccades of 20° between stationary targets. They were instructed to minimize fixation periods and to switch the gaze position as soon as the target letter was discerned. In a second task, subjects followed horizontal target jumps between two light points projected at +/−10° on either side of the midline with interstimulus intervals (ISI) of 500 and 1000 ms, respectively.
Results All subjects performed at least 100 consecutive self-paced saccades at a nearly constant rate with mean intersaccadic intervals between 410 and 460 ms.
The analysis of saccadic latencies in response to repetitive target jumps revealed broad distributions of latencies with peaks between 200 and 300 ms (reactive saccades) for the 1000 ms ISI. With ISI of 500 ms, however, considerable narrower latency distributions with peaks at 100 ms or less (predictive saccades) were found.
Conclusions Repetitive saccadic tracking at constant ISI is obviously improved if the ISI are in the range of the intersaccadic intervals during forced self-paced repetitive saccadic movements. Time strategies of self-paced saccadic scanning offer probably and internal time reference which can be used for the generation of repetitive predictive saccades.
II.4.3 (p14)
Horner syndrome after radiofrequency palatoplasty
Golge Acaroglu, Yasemin Ozdamar, Kuddusi Teberik, Fikret Ileri
Ministry of Health, Ulucanlar Eye Hospital, Ankara, Turkey
We report a 42-year-old woman with an interesting etiology of Horner syndrome. She developed miosis and ptosis immediately after radiofrequency palatoplasty surgery for snoring. This is an unreported complication of this type of surgery which is probably induced by a stretch injury of the cervical symphatetics; therefore it is not a direct complication but a possible effect of neck hyperextension during surgery.
II.4.4 (p15)
It is not what you have already diagnosed?
Golge Acaroglu, Yasemin Ozdamar, Seda Mutevelli, Orhan Zilelioglu
Ministry of Health, Ulucanlar Eye Hospital, Ankara, Turkey
We report a 52-year-old woman who presented with diplopia and face turn to left. The patient was diagnosed with a right 6th nerve palsy. Medical investigations including neuroimaging were done which were all found negative and she was referred to the neuro-ophthalmology department for follow-up. A thorough clinical examination revealed symblepharon formation at a previous pterygium surgery site (medial aspect of left conjunctiva). Restriction of the ocular movements can mimic ocular motor palsies.
II.4.5 (p23)
Isolated Ophthalmoparesis in Anti-GQ1b Antibody Syndrome
Seung-Han Lee1, Gun-Han Lim2, Sang-Jun Park3, Hyung-Kyun Oh4, Ji Soo Kim5
1Department of Neurology, Chonnam National University Medical School, 2College of Medicine, Chosun University, 3Cheomdan Medical Center, 4Kwangju Veterans Hospital, 5College of Medicine, Seoul National University
Background Anti-GQ1b antibody has been found Guillain-Barré syndrome (GBS) with ophthalmoplegia, Miller-Fisher syndrome (MFS), Bickerstaff's brainstem encephalitis, and acute ophthalmoparesis. The aim of this study was to determine the clinical features of isolated ophthalmoparesis in anti-GQ1b antibody syndrome.
Subject and Method We retrospectively recruited 34 patients with anti-GQ1b antibody. The patients were classified into GBS with ophthalmoplegia, MFS, and isolated ophthalmopareis according to their neurological manifestations.
Results Isolated ophthalmoparesis was observed in 8 (23.5%) of the 34 patients with anti-GQ1b antibody. Most patients (6/8, 75.0%) suffered from antecedent illnesses, such as upper respiratory infection (5) and diarrhea (1). External ophthalmoparesis was present in all the patients and included mixed horizontal-vertical (n =6), pure horizontal (n =1), and pure vertical gaze palsy (n =1). Binocular involvement was common (75.0%). Other findings included ptosis (n =4) and internal ophthalmoplegia (n =4). The patients were treated with intravenous immunoglobulin (5/8) or steroids (3/8), and most of them improved within 6 months.
Conclusion Isolated ophthalmoparesis commonly occurs in anti-GQ1b antibody syndrome and manifests as various combinations of external and internal ophthalmoplegia. Anti-GQ1b antibody should be sought in patients with acute ophthalmoparesis of undetermined etiology.
II.4.6 (p35)
Abducens palsy following herpes zoster ophthalmicus
Fatma Yulek1, Nurullah Ogasil1, Emine Kalkan Akcay1, Aysegul Kocak Alti˙ntas1, Saban Simsek1, Karabekir Ozcan2
1First Ophthalmology Department of SB Ankara Ataturk Training and Research Hospital; 2Radiology Department of SB Ankara Ataturk Training and Research Hospital, Ankara, Turkey
Purpose To report a case of abducens palsy following herpes zoster ophthalmicus.
Methods Retrospective chart review of a patient with abducens palsy with zoster scars on the left side of the forehead.
Case A 57-year-old male patient presented with abducens palsy on the left eye with a duration of 35 days. His medical history included diabetes mellitus and hypertension both controlled by medical therapy and a herpes zoster attack that had started 10 days before the appearance of palsy. The zoster attack presented with acute onset of pain, a vesicular cutaneous eruption and herpes zoster ophthalmicus on the left side. There was left abduction limitation with 80% loss of the normal value. The ocular examination was otherwise completely normal and there were no signs of iritis.
Result Five days after the admission of the patient the cranial magnetic resonance imaging with gadalinium enhancement revealed no pathology related to the cranial nerves and the lateral rectus muscle power had spontaneously approached to 60%.
Conclusion Microangiopathic sixth cranial nerve palsy, affecting the lateral rectus muscle, is not a rare event, but herpes zoster ophthalmicus complicated by motor nerve palsies is an unusual finding. Herpes zoster may cause peripheral neuropathy or may precipitate a sixth cranial nerve palsy due to arteritis on the basis of vascular changes caused by hypertension and diabetes.
II.4.7 (p56)
Treatment of Aberrant Facial Nerve Regeneration and Ptosis with Botilinum Toxin A and Levator Resection
Emine Kalkan Akcay, Nurullah Caği˙l, Nagehan Uğurlu, Alparslan Anayol, Aysegül Kocak Alti˙ntas, Saban Simsek
Ankara Ataturk Education and Research Hospital Department of Ophthalmology, Ankara, Turkey
Purpose To assess the treatment with Botilinum Toxin A injection and levator resection in the patient with ptosis and orbicular synkinesia after facial nerve palsy.
Methods Interventional case report. The patient with involuntary lid closure during movement of the mouth due to aberrant regeneration of the facial nerve in addition to aponeurotic ptosis was treated with injections of 25 unit BTX-A into the orbicularis oculi. Levator resection was performed to right eye. Position of eyelid and presence of orbicular synkinesia were evaluated after treatment.
Results The synkinesia of patient disappeared with BTX-A treatment in 1 week. In addition, ptosis ameliorated after levator resection. Orbicular synkinesia was not detected in 3th and 6th month controls. Minimal fasciculations during chewing was observed at 9th and 12th month controls, which did not necessitate additional treatment. Complications like lagophtalmus, hyperlacrimation, exposure kerathopathy, diplopy, lid-lag, malposition of eyelid, and weakness were not seen after treatment. Good functional and cosmetic results were obtained.
Conclusion Consecutive application of Botulinum Toxin A and levator resection yielded successful cosmetic and functional result in aberrant regeneration of the facial nerve and aponeurotic ptosis.
II.4.8 (p62)
Horner Syndrome Following Venous Catheterization for Chemotherapy
Kadircan H. Keskinbora
Acibadem Bakirkoy Hospital
Aim Percutaneous venous catheterization is an important way for applying repetitive parenteral chemotherapeutic drug administration in cancer patients. In this poster, a case who developed Horner's syndrome at right side (Ptosis, myosis, and enophthalmic expression of the right eye) following venous catheterization in the right internal jugular vein is presented.
Materials and Methods Thirty-eight-years-old male patient whose diagnosis is anaplastic large cell lymphoma has been planned to receive chemotherapy. It was very hard to find peripheral veins for intravenous injection. So, it had been decided to apply jugular central venous catheterization on the right internal jugular vein. After one day of application of the jugular venous catheterization, myosis and ptosis were recognized at the right eye of the patient. The patient has also experienced hoarseness in addition to the findings of Horner's syndrome. No evidence of mass lesions in the neck or pulmonary apex could be determined. After removal of the catheter, the hoarsenesss was resolved gradually. The case has been followed periodically (monthly) for 6 months. Although the signs of the Horner's syndrome has been reduced, they persisted till now.
Conclusion Horner's syndrome should be considered as a possible complication of percutaneous internal jugular vein catheterization.
II.4.9 (p71)
A novel method of measuring orbicularis oculi strength
Martin W. ten Hove, Michele Melanson, Ashlin Alexander
Departments of Ophthalmology and Neurology, Queen's University Kingston ON
Purpose Paresis of the orbicularis oculi is a well-recognized clinical sign in patients with myasthenia. Currently, clinical assessment of the strength of the orbicularis is limited to manually prying apart the eyelids while asking the patient forcefully close them. This crude technique offers no reliable way to quantify the weakness. We developed a practical noninvasive instrument to measure the maximal force that the orbicularis oculi is capable of generating.
Methods A surgical eyelid speculum (Katena) was modified to operate by means of a small “frictionless” pneumatic cylinder. Under topic anesthetic, the speculum was inserted between the upper and lower eye lids such that complete eye lid closure was possible. The patient was instructed to keep the eyelids forcibly shut while the pressure inside the cylinder was gradually increased by inflating a standard sphygmomanometer until the speculum force overcame the patient's maximal effort. The maximal pressure at the time of lid opening was recorded by the sphygmomanometer. This instrument was validated by measuring the maximal closing strength of control subjects as well as subjects with a proven diagnosis of myasthenia gravis.
Results Six subjects with generalized myasthenia and 10 age-matched control subjects were tested (age range 22–63; 2 males, 14 females). The median maximal closing strength for the normal group was significantly different from the myasthenic group (128.3 vs. 74.17 mmHg; p < 0.05). Facial EMG recordings also showed a similarly significant difference between the two groups (3.80 vs. 0.915mV, p < 0.05). Repeatability was assessed by performing the test three times with 1 minute rest between measurements. The mean inter-test scores variance for the control group was 5.7 and 4.5 mmHg for the myasthenic group. All subjects tolerated the testing device well and there were no injuries to the ocular surface or eyelids.
Conclusions The orbicularis oculi pressure sensor (OOPS) is a novel valid method to quantify orbicualris oculi strength. The instrument is simple to construct, easy to operate, safe and reliable. It offers a new way to diagnosis and follow patients with conditions that cause weakness of the orbicularis oculi.
II.4.10 (p77)
Perceptual fading during voluntary and involuntary eye movements
F.A. Proudlock, A.Y. Jorgensen, I. Gottlob
University of Leicester, RBT Kilpatrick Clinical Science, Leicester Royal Infirmary, Leicester, United Kingdom
Purpose Perceptual fading (PF) is a filling-in related phenomenon in which a static target in the visual field fades from view after a certain fading time (FT). PF is reset by microsaccadic eye movements; however, PF has not been investigated during nonfixation eye movements. We compared PF during voluntary eye movements in normal volunteers and in patients with involuntary eye oscillations caused by nystagmus. FT of targets during saccadic and pursuit eye movements in normal volunteers was compared to equivalent retinal movement of targets during steady fixation. FT was compared at the null point to other eccentricities in patients with nystagmus.
Methods Eleven normal volunteers performed a PF task consisting of following an oscillating fixation cross of either sine wave (pursuit task) or square wave waveform (saccadic task) while viewing a static peripheral spot of low contrast (1.5× threshold). This was compared to FT during a static fixation cross and the peripheral spot moving. Also, FT during both fixation cross and peripheral target moving synchronously was compared to a static cross and target. Targets at horizontal and vertical eccentricities were compared and eye movements recorded. FT was also compared in five volunteers with nystagmus at five horizontal eccentricities when filling-in a static target.
Results During pursuit, FT was significantly longer for the static cross + moving target condition compared to the moving cross + static target (p=0.04) and moving cross + moving target (p=0.001). In contrast, during the saccadic trial, FT was similar for static cross + moving target, and, moving cross + static target tasks, but both were lower than the moving cross + moving target task. FT loosely resembled nystagmus intensity in the five subjects with nystagmus.
Conclusions We describe the filling-in of moving targets when stimuli are applied at low contrasts. For equivalent target movements on the retina in normals, FT was less during voluntary eye movements compared to when the target moves relative to the eye. This was only during pursuit but not the saccadic task. Interestingly, patients with involuntary eye movements could also fill–in targets although responses were more variable than normals.
II.4.11 (p80)
Periodic alternating nystagmus and multiple other ocular motor manifestations of Chiari malformation
Scott D.Z. Eggers
Mayo Clinic, Rochester, MN, USA
Aim Chiari malformation is a disorder of caudal descent of the hindbrain through the foramen magnum. Various ocular motor and vestibularmanifestations have been associated with Chiari malformation.
Case Report A patient found to have a Chiari I malformationpresented with complaints of oscillopsia, diplopia, and progressive gait ataxia. Examination revealed periodic alternating nystagmus, downbeat nystagmus, acquired esotropia, alternating skew deviation, impaired pursuit and vestibulo-ocular reflex (VOR) suppression, and cross-coupling of the horizontal VOR. Management involved suboccipital craniectomy and upper cervical laminectomies, medial rectus recession and base out prisms for esotropia, and baclofen to abolish the PAN and dampen the downbeat nystagmus. Numerous ocular motor manifestations referable to vestibulocerebellar dysfunction may occur in the setting of Chiari malformation, sometimes all in asingle patient.
Conclusion This case exemplifies the importance of a systematic approach in identifying each of them to facilitate the appropriate diagnosis and specific treatments.
II.4.12 (p90)
Long-term management of sixth nerve palsy
Akar Serpil, Özbek Asli˙, Gökyigit Birsen, Güngel Hülya, Yi˙lmaz Ömer Faruk
Beyoglu Eye Education and Research Hospital, Istanbul, Turkey
Purpose to report the long-term management of sixth nerve palsy.
Methods A total of 74 of 113 cases were acute sixth nerve palsy and 39 cases were chronic sixth nerve palsy. Six (5%) were bilateral and 107 (95%) were unilateral. Twenty of 74 acute sixth nerve palsy cases was performed Botulinum toxin A or Fresnel prism. Nineteen of 39 chronic sixth nerve palsy cases had strabismus surgery alone and 8 surgeries with Botox. Outcome was classified at time of last follow-up (45 cases were examined). Success was defined as no diplopia in primary position at distance fixation. Partial success was defined as no more than 10 PD esotropia despite diplopia. The follow up period was 2 years.
Results Overall, 39 (86%) of the patients were classified as successes, 3 (7%) as partial successes, and 3 (7%) as failures. 7% of all patients had more than one surgery.
Conclusions Botulinum toxin A and the use of Fresnel prism can be used for to relieve symptomatic and to prevent medial rectus muscle contracture in acute stage. Our reported good surgical success rate in chronic sixth nerve palsy. It can need additional strabismus surgery or the use of prism for face turn or small residual deviations in the management of chronic sixth nerve palsy.
II.4.13 (p93)
Fourth nerve palsy after Trigeminal Neuralgia treatment with Radiofrequency Rhizolysis
Huban Atilla, Necile Erkam, Yücel Kanpolat
Department of Ophthalmology and Neurosurgery, Faculty of Medicine, Ankara University, Turkey
Case Report We report a patient with fourth nerve palsy after Radiofrequency Rhizolysis. Sixty-eight-years-old female patient who had Radiofrequency Rhizolysis previously for three times to treat resistant trigeminal neuralgia, had diplopia after the recent procedure.
She had 16–18 PD left hypertropia that was increasing on right gaze and left head tilt, 8 PD of left hypertropia with Maddox rod test and less than 10° of excyclotropia with double Maddox test. Her motility examination showed underaction of left superior oblique muscle. Fresnel prism was prescribed (8 PD base down on left eyeglasses) and at third month control, her complaints improved and she was able to fuse with minimal head position without prisms.
Conclusion Abducens nerve palsy as a complication of Radiofrequency Rhizolysis was reported and accepted to be related to mislocation of the needle. Fourth nerve palsy was a rare complication and spontaneous improvement that was detected in our case indicated a reversible damage of fourth nerve that was caused by indirect effect of the procedure due to close proximity of the nerves in the cavernous sinus.
II.4.14 (p95)
Bilateral adie's pupils in systemic amyloidosis
P. Fahmy1, H. Kjaerbo2, C.B. Andersen3, D. Milea4
1Department of Ophthalmology, Naestved Hospital, Denmark; 2Department of Ophthalmology, Roskilde Hospital, Denmark; 3Department of Pathology, Rigshospitalet University Hospital, Denmark; 4Department of Ophthalmology, Glostrup University Hospital, Denmark
Purpose To report occurrence of bilateral Adie's tonic pupil as a consequence of systemic autonomic dysfunction in a patient with primary AL amyloidosis.
Methods A 59 year-old male patient with pathologically proven amyloidosis causing polyneuropathy, renal and cardiac involvement was referred for ophthalmic assessment of isolated bilateral mydriasis.
Results Unequal mydriasis was present, resulting in anisocoria. Both pupils were unresponsive to light, but light-near dissociation was found, with tonic mild contractions of the pupils after the near-response. Slit-lamp examination disclosed an oval shape of the pupils, most marked on the right side, but no sign of an associated intraocular condition, i.e., synaechie. The eye movements were normal as well as the remainder of the ophthalmic examination, showing no intraocular involvement of the systemic disease. After diluted pilocarpin 0.125% instillation, marked bilateral miosis was found, as a result of denervation supersensitivity and being suggestive of bilateral Adie's tonic pupils.
Conclusion Bilateral Adie's tonic pupils can occur in patients with systemic peripheral polyneuropathies, such as in amyloidosis with autonomic dysfunction. In these cases, since the bilateral isolated iridoplegia is a result of a probable peripheral insult of the cilliary ganglions or nerves, no further invasive investigations of the central nervous system are needed.
II.4.15 (p101)
Extreme torsion: sensory adaption to iatrogenic monocular torsion induced by macular translocation surgery
L.S. Mengher, G.S. Uppal, L. da Cruz, J.P. Lee and J.F. Acheson
Neuro-ophthalmology & Strabismus Service; Moorfields Eye Hospital, City Road, London. EC1V 2PD United Kingdom
Introduction Despite anatomically successful 360° macular translocation surgery (MT360) and counter rotation surgery for exudative age related macular degeneration (AMD), symptom of torsion remain a major obstacle to achieving full visual rehabilitation. In a consecutive series of 27 cases of MT360 we examined the incidence and magnitude of iatrogenic torsion following MT360 and subsequent counter rotation surgery.
Methods and Patients A prospective interventional case series of 27 consecutive patients (17 females, 10 males) with a mean age of 76 (57–95) years and subfoveal choroidal neovascular membranes (CNV) secondary to AMD underwent MT360 with silicone oil tamponade and subsequent counter-rotation surgery at Moorfields Eye Hospital, London between May 2003 and 2006. MT360 was performed by a single surgeon (LDC) and counter rotation surgery by two surgeons (JPL and JA).
Results At an average follow-up of 12.2 months (04–36) the ETDRS distance acuity improved from a mean preoperative logMAR acuity of 0.88 (0.18–2.10) to 0.68 (0.12–1.80) (p < 0.03) postoperatively. During the same follow up period the residual torsion after counter rotation surgery decreased from 37° (15–60) to 11° (0–40).
After MT360 surgery and despite silicone oil tamponade, 95% of the patients experienced extreme torsion. Following counter rotation squint surgery, review at 3 months stage, 75% were aware of torsion. At 12.2 month assessment 50% were still symptomatic; of which 40% were adversely affected by torsion in the activities of daily living.
Conclusions Despite good anatomical and visual outcome after MT360, functional recovery was limited by torsion. This debilitating symptom occurred in 20% of the cohort and represent a formidable obstacle to successful visual rehabilitation. Strategies need to be developed in order to optimize visual function.
II.4.16 (p162)
Etiological eveluation in cranial nerve (cn) 3rd palsy and efficiency in the treatment phases
Birsen Gokyigit, Serpil Akar, Asli Ozbek, Vedat Kaya, Mehmet Caki˙r
Beyoglu Education and Research Eye Hospital, Istanbul, Turkey
Aim The aim of this study is evaluation of etiology in CN 3rd palsy and the investigation of the efficiency in the treatment phases.
Materials and Methods The files of 50 patients (18 women, 32 men) in our hospital's strabismus department were retrospectively investigated.
The youngest of the patients was 6 and the oldest was 82 years old. We noted that right eye in 15 patients, left eye in 24 patients and both eyes in 9 patients were affected and 31 patients had ptosis.
Results Illnesses began 1 day to 42 years prior to our examination of the patients. Nine patients had the problem since birth, in 13 patients it started following trauma, in 4 patients following convulsion and 1 patient following brain operation. The reason for palsy in 14 patients was determined as systematic illness of whom 6 had diabetes, 4 had high blood pressure and 4 of whom had both. Three patients had cerebral disease, 1 patient myasthenia and 1 patient sinusitis. We could not determine a specific reason in 4 patients. The median follow-up of the patients was 14.3 months.
Besides taking under control of the systematic illnesses, Botilinum toxin A injection was applied to lateral rectus of the effected eyes of 7 patients during the first 2 months. Full recovery was noted in 5 of these patients during 6 month follow-up. Surgery was done in 18 patients whose cases were older than 6 months. Except for 3 of these patients, deviation in primer position was below +10 pd. and the results did not change in longer follow-ups. Un-effected 3 patients had congenital CN 3rd palsy.
Conclusion We concluded that congenital total CN 3rd palsy had strong resistance to surgical treatment.
II.4.17 (p172)
Paroxysmal Ocular Tilt Reaction due to Mesodiencephalic Lesion
Sun-Young Oh, Byoung-Soo Shin, Man-Wook Seo, Kwang-Dong Choi, Ji Soo Kim
Department of Neurology, Chonbuk National University Hospital
Mesodiencephalic lesions usually produce a tonic contralesional ocular tilt reaction by involving either the riMLF or INC. Lesions of the INC produce an ipsilesional torsional nystagmus and lesions of the riMLF cause a contralesional torsional nystagmus. Here, we describe four patients with paroxysmal ipsiversive OTR concurrently with contralesional or ipsilesional torsional nystagmus. Patients are also associated with vertical gaze palsy, saccadic slowing and decreased amplitudes of unilateral torsional nystagmus. These findings may be explained by paroxysmal irritation of the ipsilesional interstitial nucleus of Cajal and/or rostral interstitial nucleus of the medial longitudinal fasciculus. In this paper, we are attempt to differentiate the effects of INC abnormality and riMLF as a cause of paroxysmal ocular tilt reaction from mesencephalic lesions on different oculomotor parameters under clinical observations.
II.5. Idiopathic Intracranial Hypertension (IIH)—Poster Presentations
II.5.1 (p6)
Visual functions in benign intracranial hypertension
N. Eliseeva, N. Serova, S. Gasparyan, M. Shifrin
The Burdenko Neurosurgery Institute, Moscow, Russian Federation
Aim The visual functions and optic disc appearances were studied in 49 patients with a diagnosis of benign intracranial hypertension (BIH).
Materials and Methods Age of the patients ranged from 4 to 52 years (median 39). There were 5 patients under 18 years of age. The male to female ratio was 1:11.5. Obesity was found in 25 (51%) of the patients. There were 15 (30.6%) patients with venous sinus thrombosis causing BIH. In patients under 18 years of age the predisposing conditions were viral infection and otitis media. MRI demonstrated an empty sella in 27 (55.1%) patients.
Results The patients had early (2 eyes), moderate (14 eyes) severe (23 eyes) and chronic (10 eyes) stages of papilledema. Visual acuity was disturbed in 23 (46.9%) patients in one or both eyes at the initial visit. 41% of the eyes had visual acuities between 0.2–0.8 and 10% of the eyes had visual acuities less than 0.05. Visual acuity loss occurred only in eyes with severe and chronic papilledema. Automated perimetry revealed enlargement of the blind spot, inferior nasal defects, peripheral constriction, temporal island. 43 patients were on follow-up during the study. Medical therapy was successful in 27 patients. Lumboperitoneal shunting became necessary in 16 patients if visual function worsened and increased CSF pressure persists despite medical treatment. Visual acuity deterioration was stationary in 9 (20%) patients, improved in 8 (18%) patients, decreased in 6 (14%), patients. Visual field defects were stationary 18 (41%) patients, improved in 12 (27%) patients, worsened in 7 (16%) patients.
Conclusions Severity of visual disturbances correlates with the stage of papilledema. Automated perimetry is the most useful method to reveal visual disturbances and follow up patients with BIH. Lumboperitoneal shunting is necessary in cases of progressive visual loss.
II.5.2 (p16)
Benign Intracranial Hypertension associated with Sulphasalazine Treatment
Eser Basak Sevgi, Gul Yalcin Cakmakli, Tulay Kansu, Kubilay Varli
Hacettepe University, Faculty of Medicine, Department of Neurology, Ankara, Turkey
Purpose To report a patient who developed headache and papilledema under sulphasalazine treatment for ulcerative colitis.
Background Sulfapyridine bound to 5 Aminosalicylate (5 ASA) is named as Sulphasalazine. Headache is a known side effect of sulphasalazine, but intracranial hypertension (IH) due to sulphasalazine treatment has not been reported before. Two previous cases of benign IH reported in the literature have been associated with Mesalasine treatment, which is another product of 5 ASA.
Case description 25-year-old female patient was admitted to the hospital with the complaint of headache and blurred vision. She had been put on sulphasalazine treatment for ulcerative colitis three weeks previously. Neurological examination revealed 20/200 visual acuity on both sides, peripheral constriction in visual fields, bilateral papilledema, abduction deficit, and peripheral facial palsy on the left. Cranial MR and MR venography were found to be normal. LP revealed an opening pressure of 300 mm H2O. Diagnosis of ulcerative colitis was confirmed by intestinal biopsy. Sulphasalazine was discontinued and the patient continued with azathioprine for ulcerative colitis and acetazolamide for IH. Three weeks later, her examination was normal and LP revealed an opening pressure of 180 mm H2O.
Conclusion There is a likely relationship between the administration of sulphasalazine and the onset of IH symptoms. Early diagnosis of IH is important in patients with ulcerative colitis receiving 5 ASA treatments to prevent visual complications.
II.5.3 (p33)
Treatment of idiopathic intracranial hypertension: Topiramate versus acetazolamide, an open-label study
Nee Çelebisoy, Figen Gökçay, Hadiye Şirin, Önder Akyürekli
Ege University Medical School Department of Neurology, İzmir, Turkey
Aim Idiopathic intracranial hypertension (IIH), is the clinical syndrome of raised intracranial pressure without clinical, laboratory or radiological evidence of intracranial pathology. There have been no sufficiently large studies of any treatment modality in IIH. The carbonic anhydrase inhibitor acetazolamide is usually accepted as the first-line drug in treatment and it acts by reducing the CSF production. Topiramate is a relatively new anticonvulsant which is also a carbonic anhydrase inhibitor. This open-label study was planned to assess the efficacy and tolerability of topiramate in IIH and to compare it with acetazolamide.
Materials and Methods A total of 41 patients diagnosed with IIH were randomly assigned to treatment with either acetazolamide or topiramate from January 2002 to January 2007. Snellen visual acuities, opthalmoscopy and automated perimetry testing was made every second week in the first month increasing to one per third month to judge the treatment efficacy. The results of the third, sixth and twelfth months were taken into consideration.
Results There were 36 women and 5 men with ages ranging from 16 to 51 years (mean 32.5, SD 9.5). One patient was excluded as the visual fields deteriorated very rapidly within the first week when she was using topiramate 50∼mg/d and a lumboperitoneal shunt was performed. The results of 20 patients on topiramate were compared with the results of the 20 patients on acetazolamide. Treatment of topiramate was administered at doses ranging from 50 mg to 100 mg daily. Daily doses of acetazolamide ranged from 500 mg to 1000 mg. When the visual fields at the end of the third, sixth an twelfth months of the two groups were compared it was seen that no statistically significant difference was present. The most frequent side effects in the acetazolamide group were fatigue and tingling of the hands and feet. The most common side effects in the topiramate group were distal paresthesias, concentration difficulties and weight loss. None of the patients in either group discontinued because of adverse events.
Conclusion These results suggest that topiramate is efficacious and well-tolerated in the treatment of IIH. Further study in a larger, placebo-controlled, double-blind trial is recommended to confirm these results.
II.5.4 (p37)
The cases of benign intracranial hypertension
Guseynova Sima; Kazimova Banovsha
(Z. Aliyeva) Scientific Research Institute of Ophthalmology, Baku, Azerbeijan
Aim Benign intracranial hypertension syndrome is a disease based on an increase of intracranial hypertension without signs of a space-occupying lesion of the brain or any other obvious causes of hypertension. Rarely the disease is followed by “empty” turkish saddle syndrome. Mainly young, overweight females develop benign hypertension syndrome. Also children can develop the above mentioned syndrome as a result of steroids withdrawal.
Materials and Methods There was a patient, young female, suffering from obesity, who was referred to the SRI of eye diseases named after Z.Alieva, diagnosed with “bilateral optical neuritis”. The patient was referred by the neurosurgical clinic, where she was under treatment regarding her headaches.
Results At the moment of the admission to SRI of the eye diseases, the patient was complaining of decreased bilateral vision. Headaches were not noted. During an examination: Visual acuity of OD–0, 7; OS–0, 4 (n/c). Field of vision on white, red and green colors is remained on the small areas of temporal parts: OD–in a superior temporal quadrant, OS–in an inferior temporal quadrant. Ophthalmoscopy assessment showed bilateral congested disks of the optic nerves at the stage of beginning atrophy.
MRI examination detected insignificant edema of the brain where the sizes of brain ventricles were normal. During spinal puncture intracranial pressure was 240 mm Hg. In spite of steroid and dehydrating therapy vision acuity decreased to 0, 01 and 0, 03 within 2 weeks. Field of vision remained within 5–7°, red and green colors were recognized. Against a background of the repeated lumbar punctures and intracranial pressure decrease to 130 mmHg, visual acuity of both eyes increased up to 0,05–0,06. Field of vision did not expand. On the follow-up examination, secondary optic atrophy was observed.
Conclusion In summary, we remind to the ophthalmologists and neuro-ophthalmologists about a probability of the pseudotumor of the brain in the cases with bilateral congested disks optic nerves.
II.5.5 (p38)
Ophthalmic manifestation of the “Empty” Turkish saddle syndrome
Guseynova Sima, Ibragimova Nurana, Kazimova Banovsha
(Z. Aliyeva) Scientific Research Institute of Ophthalmology, Baku, Azerbeijan
Aim “Empty” Turkish saddle syndrome is one of the insufficiently known problems of neuro-ophthalmology.
Materials and Methods Due to implementation of the noninvasive method of MRI, the opportunities to study this pathology have greatly increased. Turkish saddle on MRI is always filled up by liquor and contains the remains of hypophysis. Sometimes chiasmic and optic nerves can intrude into it. Ventricular system of the brain is not expanded and subarachnoid space is in norm.
Clinical presentations of the “empty” Turkish saddle are the combination of the various visual, neurological, and endocrine impairments.
Results There were 17 female patients, aged between 15 and 45, under our observation, with verified (on MRI) empty Turkish saddle syndrome. Of these, six patients had been diagnosed with benign intracranial hypertension.
Subjective complaints mainly resulted in an ulterior headache, including the pain in the depth of orbital cavity, short-term “misting”, diplopia, and visual defocusing.
Persistent reduction of visual acuity within 0.2–0.8 took place in 10 cases, the 8 patients have been identified with changes of visual fields (bitemporal defects, increased blind spots, concentric narrowing). Additionally, 12 patients had bilateral edema of the optic nerves at varuous intensities, and 3 patients had optic atrophy. Only 1 patient was diagnosed with bilaterally increased intraocular pressure, up to 28 mm Hg. In all cases, during spinal puncture, the increase of intracranial pressure existed.
Conclusions According to the available data, ophthalmic impairments are concerned with pathogenetic factors, which determine the general clinical presentation of the “empty” turkish saddle syndrome and caused by the intracranial hypertension, dysfunction of the vegetative nervous system and by the impairment of blood supply of the chiasmatic part of optic tract.
The research of the ophthalmic manifestations of the “empty” turkish saddle syndrome arises big interest for the ophthalmologists in cases of the visual impairment where there is no other ophthalmic pathology. At the same time, detection of an optic nerve edema has significant importance for the timely diagnostics of intracranial hypertension and for those caused by complications from the vision point of view.
II.5.6 (p67)
Idiopathic intracranial hypertension in a woman with schizophrenia
A. Tzoukeva, N. Deleva, I. Dimitrov
First Clinic of Neurology, “Sveta Marina” University Hospital, Varna, Bulgaria
Background Idiopathic intracranial hypertension (IIH) or benign intracranial hypertension is a neurological syndrome characterized by elevated intracranial pressure. Though the origin seems to be multifactorial, most cases remain idiopathic. This uncommon disorder occurs primarily in obese women aged 10–50 years in association with endocrine and metabolic dysfunction, with systemic diseases or treated with multiple medications. For some drugs, the association between these exogenous substances and the development of IIH is well documented. For others however a causal relationship is uncertain.
Purpose To report a case of IIH in a 43-year-old woman with schizophrenia treated with risperidone, demonstrating the typical clinical picture with special attention to etiology.
Methods and Results The patient was diagnosed with schizophrenia 10 years ago. One month after initiation of treatment with risperidone she noted weight gain (30 kg for 5 years), and another 3 years later she began to complain of menstrual irregularities, impaired glucose tolerability, headache, nausea with vomiting. Transient visual obscurations were experienced from 2–3 month. IIH was diagnosed by revealing bilateral asymmetric papilledema, normal magnetic resonance imaging and elevated intracranial pressure. Risperidone was discontinued, treatment with furosemide and mannitol was initiated, and papilledema was completely resolved in 2 months.
Discussion IIH is a rare disorder, associated with several conditions and risk factors: obesity, weight gain, tetracycline antibiotics, vitamin A and other retinoids. Numerous drugs, including psychotherapeutics, have been associated with IIH, but a causal relationship is only supported by single cases. In our case, a woman with schizophrenia treated for 5 years with risperidone, developed IIH with a typical clinical picture. Weight gain is a well-known adverse side effect of risperidone, and at the same time it is associated with greater risk of IIH. It could be discussed that IIH in the described case was mainly associated with weight gain, secondary to prolonged use of risperidone, and not with risperidone itself.
Conclusion The role of adverse effects of risperidone should be considered when IIH occurs in patients with schizophrenia, treated with the above-mentioned drug.
II.5.7 (p87)
Pseudotumor cerebri: Analysis of 38 cases
Derya Tosun, Erdem Yaka, Fethi Idiman
Dokuz Eylul University Department of Neurology, İzmir, Turkey
Aim Pseudotumor cerebri (PTC), also known as “benign” intracranial hypertension or idiopathic intracranial hypertension (IIH) appears with the clinical signs of elevated intracranial pressure. The etiology is unclear but generally it can be associated with disorders such as obesity, endocrinologic abnormalities, empty sella, intracranial venous sinus thrombosis, chronic otitis media and there is no specific diagnostic tools for PTC.
Materials and Methods In this study, we assessed the demographic features, clinical signs and symptoms, treatment and follow-up periods of 38 IIH patients in our neuroophthalmology unit.
Results A total of 33 of the patients were female and 5 of them were male of whom the average ages were respectively, 32.1 and 31.4. The duration of the complaints were 6 days to 10 years (average 17.4 months) when they were accepted into the department. The most common complaint, headache and/or ocular pain, occurred in 84.2% of the patients. Visual disturbances occurred in 60.5 % of the patients. The second most common reported complaints were vertigo, nausea, diplopia and tinnitus. The morbidity of visual deficits was documented in 48.6 % of the cases that separated from mild to severe respectively, 13.1% and 26.3%. Visual field deficits reported as 60% that generalized constriction (30.9) and enlarged blind spot (14.2 %) were the most common forms. Papilledema occurred in 70% of the patients, most of which were bilateral (64.8 %). There were no neuroradiologic abnormalities demonstrated nearly in 50 % of the cases during evaluation. Respectively, slit-like ventricules, empty sella, venous sinus thrombosis and optic hydrops were revealed.
Conclusion In our cases,respectively, endocrinologic abnormalities, obesity, hypertension, administering oral contraception's, venous thrombosis, vasculitis, seizure were associated with diseases. When follow-up documentations were reviewed, we assessed that only two patients had severe visual functional impairment, while others recovered totally or nearly total.
II.5.8 (p108)
Pseudotomor cerebri: is it a “benign” disease?
Golge Acaroglu, Yasemin Ozdamar, Murat S. Songur, Bayazit Ilhan, Seyhan S. Ozkan
Ministry of Health, Ulcanlar Eye Hospital, Ankara, Turkey
Purpose To evaluate the residual functional visual impairment in patients with pseudotumor cerebri (PTC) after the conclusion of therapy.
Material-Methods Medical records of patients treated and followed at our neuro-ophthalmology department from 1997 to 2006 were reviewed. All patients were diagnosed according to modified Dandy's Criteria and treated medically and/or surgically. Thirty patients who had at least 6 months' follow-up after the termination of their therapy were included in the study. Parameters including visual acuity, visual fields, color vision and the status of the optic nerve head were assessed at the final visit to determine the visual sequel.
Results There were 24 women and 5 men with an average age of 36.4 ± 9.9 years (range, 16–55 years). Patients were followed-up for 6 to 84 months (mean, 25.3 months). Mean visual acuity (VA) level was 0.82 ± 0.26 (Snellen) and mean color vision (CV) score was 9.8 ± 3.7 (Ishihara) at the initial examination. Papilledema was present in 47 eyes. Visual field defects were present in 50 eyes, including peripheral constriction in 16 eyes and big blind spots in 14 eyes. Diplopia was observed in 5 eyes. Patients received at least one of the treatment modalities such as acetazolamide, corticosteroids, lumboperitoneal shunt and optic nerve sheath decompression. At the end of the therapy, general health and symptoms of the patients improved, however; majority of eyes were left with at least one visual sequel. Nineteen eyes had varying degrees of optic atrophy which caused mild-to moderate permanent visual acuity and color vision loss. Visual field defects persisted in 31 eyes, mostly in the form of inferior-nasal or other types of arcuate defects.
Conclusion Pseudotumor cerebri is thought to have a benign course if treated. Our study demonstrates that this is only partly true, because even strictly treated and followed patients can end up with visual impairment.
II.5.9 (p122)
Efficiency of rarebit perimetry in the evaluation of patients with pseudotumor cerebri
Şansal Gedik, Sibel Oto, Ahmet Akman, Yonca A. Akova
Başkent University Faculty of Medicine, Department of Ophthalmology, Ankara, Turkey
ObjectiveIdiopathic intracranial hypertension (IIH) is a common syndrome accounting for papilledema in neuroophthalmic practice. In this study, we aimed to compare the efficiency of Rarebit (RB) perimetry and Humphrey Field Analyzer (HFA) in detecting the visual field defects in patients with IIH.
Patients and MethodEighteen patients with IIH who fullfilled the modified Dandy Walker criteria underwent visual field analysis on the same day, in random order; either first with Humphrey Perimetry 30-2, SITA standard program (Zeiss Humphrey Systems, CA) or Rarebit perimetry. Data including the patient's age and sex, global indices for HFA (such as the mean deviation [MD] and the pattern of standard deviation [PSD], fixation loss, false-positive and false-negative errors, the duration of the test), and parameters like mean hit rate (MHR), the number of tested locations with a hit rate of less than 90%, the error number, the time required to complete the test, and the mean reaction time for RB perimetry were recorded. Only the right eye parameters have been taken into consideration for statistical analysis.
Results Eighteen patients, with a mean age of 39.88±16.97 years (range: 12–61) were included. The HFA results, mean MD, PSD, fixation loss, false positive and negative errors and test duration were–-9.38 dB, 6.34 dB, 2.77, 3.83%, 10.16%, 7.6 min, respectively. The mean RB perimetry results mean hit rate, hit rate number 90%, error number, test time and mean reaction time were 65.27%, 53.88%, 0.5, 3.69 min, 0.81 s, respectively.
ConclusionRB perimetry is a reliable, easily performed method, which may be helpful in evaluating visual field defects in patients with IIH.
II.5.10 (p127)
The association between intracranial hypertension and anemia
S.P. Mollan, A. Ball, A. Sinclair, S. Madill, M.A. Burdon, T.D. Matthews
The Birmingham Neuro-Ophthalmology Unit, Birmingham, United Kingdom
Objective To investigate the incidence and clinical features of “idiopathic” intracranial hypertension (IIH) occurring in the presence of anemia.
DesignRetrospective consecutive case note review.
MethodsPatients with clinical diagnoses of intracranial hypertension and anemia presenting to the Birmingham Neuro-ophthalmology Unit, United Kingdom, over a 2-year period were identified. Demographics, etiology and clinical details were recorded and analyzed.
Results Six cases were identified (1 male and 5 females) from 102 new cases presenting with IIH. All 6 had documented microcytic anaemia with clinical evidence of raised intracranial hypertension. There was no evidence of venous sinus thrombosis on MRI and MRV imaging in 5 subjects and CTV in one. On correction of anemia alone, 5 cases resolved. One patient with severe progressive visual loss underwent VP shunt in addition to treatment of their anemia, with good outcome.
Discussion These cases further present an association between anemia and intracranial hypertension. Investigation and treatment of the existing anemia was associated with reversal of visual loss. A clinically significant proportion of cases presenting with signs of IIH have anemia.
II.5.11 (p148)
Unilateral papilledema in pseudotumor cerebri
Fatih M. Mutlu, Samir Musayev
GATA Department of Ophthalmology, Ankara, Turkey
We describe a 12-year-old girl who complained of headache and transient blurring vision in her right eye. Visual acuity was 20/20 in both eyes and there was no pupillary defect. Eye movements and slit-lamp examination were normal. Fundoscopic examination revealed right optic disc edema, whereas the left eye was normal. Visual field test demonstrated enlarged blind spot in the right eye. Neurologic and systemic examination were normal. Laboratory studies were unremarkable. After discarding an ocular or orbital condition, normal magnetic resonance imaging and lumbar puncture with an opening pressure of 250 mm H2O lead to diagnosis of unilateral disc edema due to idiopathic intracranial hypertension (pseudotumor cerebri). Symptoms improved after Acetazolamide treatment. Although increased intracranial pressure usually leads to bilateral papilledema, unilateral papilledema sometimes is a sign of intracranial hypertension. Congenital or acquired factors may alter the patency of the optic nerve sheaths, and maybe result in unilateral papilledema in cases with pseudotumor cerebri.
II.5.12 (p155)
Optic nerve sheath fenestration in idiopathic intracranial hypertension
Feyza Önder, Savaş Özay
Haseki Education and Research Hospital Eye Clinic, İstanbul, Turkey
Purpuse To evaluate the medical and the surgical results of the 12 patients that were diagnosed and followed as cases of idiopathic intracranial hypertension (IIH).
Methods and Materials The clinical properties and the results of the medical and surgical treatment of the 12 patients with IIH were evaluated.
Results The age of the patients were between 20 and 46, 9 females and 3 males. All patients underwent lumbar puncture with manometry and all pressure of cerebrospinal fluid (CSF) of the patients were measured above the level of 300 mm H20. All patients had bilateral papilledema or poststasis optic atrophy with normal cranial MRI results. In 5 patients an improvement was detected by the medical treatment of the carbonic anhydrase inhibitors. The 8 eyes with a progressive visual field defect, of the 4 patients who showed no response to the medical treatment, underwent optic nerve sheath fenestration (ONSF) surgery by transconjunctival medial orbitotomy. An improvement of visual field was shown in 3 eyes whereas visual field stabilization was detected in 5 eyes. ONSF was not performed in 3 cases because of poststasis optic atrophy. During the 1 year follow-up period, papilledema did not recur in any patient.
Conclusion ONSF is an effective and safe surgical procedure for IIH patients who show no response to medical treatment.
II.5.13 (p160)
Normal baseline retinal nerve fiber layer thickness in patients with idiopathic intracranial hypertension
Ugur E. Altiparmak, Banu S. Satana, Nilay Dundar, Deniz Acar, Ozlem Cozkun, Sunay Duman
S.B. Ankara Training and Research Hospital Departments of Ophthalmology and Neurology
Purpose To determine the retinal nerve fiber layer (RNFL) thickness measurement in patients with idiopathic intracranial hypertension (IIH), at a point where there is no disc edema.
Materials and Methods Chart review of 25 patients with IIH was performed. Only 20 eyes of 13 patients fulfilled the criteria, including: minimal or no disc edema (Frisen classification 0 or 1), normal dynamic visual field, best corrected visual acuity of +0.1 or higher and normal color visual acuity with Ishihara color plates.
Results The mean overall RNFL thickness was 104.2 ± 12.5. The mean thickness of individual quadrants are slightly above age-matched normals, though not significant.
Conclusion The baseline RNFL thickness of IIH patients is slightly higher than normal, though not significant. Further data with higher number of patients seem necessary.
II.5.14 (p161)
The incidence of empty sella and the impact of lumbar puncture opening pressure in patients with idiopathic intracranial hypertension
Ugur E. Altiparmak, Banu S. Satana, Deniz Acar, Nilay Dundar, Serap Ucler, Sunay Duman
S.B. Ankara Training and Research Hospital Dpeartments of Ophthalmology and Neurology
Purpose To determine a possible relationship between the lumbar puncture (LP) opening pressure and the incidence of empty sella in patients with idiopathic intracranial hypertension (IIH).
Materials and Methods Chart review of 25 patients with IIH was performed. The patients were subdivided into three groups according to the LP opening pressure: In group 1, the pressures were between 21 and 30 mmHg, in group 2 the pressures were between 31 and 40 mmHg and in group 4 the pressures were 41 mmHg and higher.
Results The mean LP opening pressure was 36.6 ± 12.9 mmHg. There were 7 patients in group 1, 13 patients in group 2 and 5 patients in group 3. The incidence of empty sella was 3/7 (42.7%) in group 1, 7/13 (53.8%) in group 2 and 0/5 (0%) in group 3.
Conclusion There was no positive correlation between the LP opening pressure and the incidence of empty sella incidence in patients with IIH.
II.6. Chiasmal Diseases—Poster Presentations
II.6.1 (p3)
Bilateral Optic Neuritis Asscociated With Pituitary Abcess
A. Oscar1, S. Cherninkova1, V. Karakostov2, V. Busarski2, L. Kaljonski3
1Clinic of Neurology, University Hospital “Alexandrovska”, Sofia; 2Clinic of Neurosurgery, University Hospital “Saint Ivan Rilski”, Sofia; 3Sector of Radiology, Government Hospital, Sofia, Bulgaria
Aim The pituitary abscess is a serious and life-threatening condition that could lead to a lethal end if not treated properly and on time.
Case Report We present a 50–year-old patient, admitted and treated in the Clinic of Neurosugery, University Hospital “Saint Ivan Rilski”, Sofia, due to complaints of intensive occipital headache, blurred vision and high fever. The MRI disclosed heterointensive intra-suprasellar lesion, resembling pituitary apoplexy or abscess. The surgical intervention confirmed the existence of the pituitary abscess. The normal pituitary gland and optic chiasm were decompressed and the purulent substance was removed. Despite the aggressive antibiotic treatment, the Neuro-ophthalmic examination, accomplished just after the surgery, presented bilateral infectious /neighboring/ optic neuritis. A right-sided central scotoma and a left sided diffusely reduced retinal light-sensitivity in the central area was detected trough the method of computerized perimetry. The visual acuity was considerably reduced in the right eye and slightly reduced in the left eye. No afferent papillary defect was detected. Color vision wasbilaterally impaired, more severe in the left eye. A considerable improvement (almost to normal visual acuity and visual fields) of visual functions was demonstrated after 15–20 of dynamic observation and antibiotic treatment.
Conclusion The proper diagnosis and adequate treatment of thepituitary abscess and consequent complication (bilateral infectious optic neuritis) dramatically improves the chances not only for the life of the patient but also considerably improves the visual functions.
II.6.2 (p5)
Assessment of the efficacy of the radiotherapy for para-and suprasellar meningiomas (opthalmological aspects).
N. Serova, A. Galanov, P. Koudryiavtseva, S. Maryashev, I. Pronin
The Burdenko Neurosurgery Institute, Moscow, Russian Federation
Purpose To analyze the ophthalmological symptoms and the efficacy of the radiotherapy for para- and suprasellar meningiomas.
Material and methods 51 patients (43 female and 8 male) aged 29–70 (median 49) with para- and suprasellar meningiomas were studied. Tumor volume ranged from 0.79 to 70.5 ml (median 17.7). The patients were treated with stereotactic radiotherapy (SRT) on linear accelerator “Novalis” by use of 1.8 Gy daily fractions to cumulative dose of 34.2 to 74.2 Gy. All patients were examined before radiotherapy, 18–-during the treatment, 41–-just after the treatment, 18-in follow up (2–19 months, median 6).
Results Before treatment, deficit of eye movement was observed in 66.7% of the patients' visual disturbances in 58.8%. The improvement of the visual functions after treatment was more often (41.7%), then the improvement of the eye movement (19.2%). We would like to emphasize the dramatic improvement in the visual acuity from practical blindness to normal vision within treatment in two patients. N.oculomotorius was more sensitive to treatment then N abducens. In follow up we observed positive dynamic in two patients only; they had improvement of the eye movement. Visual functions remained stable in all patients. We revealed no impairment of the visual functions or eye movement, no neurological deterioration just after radiotherapy or in follow up.
Conclusion according to our experience the visual functions in patients with para-and suprasellar meningiomas are more sensitive to treatment then the eye movement disorders and can improve during the radiotherapy or in a shortperiod after.
II.6.3 (p32)
A new visual field testing in empty sella syndrome: rarebit perimetry
G.F. Yavas*, P. Aydi˙n**, T. Küsbeci*, O. Eser***, F. Öztürk*
*Kocatepe University, Dep of Ophthalmology, Afyonkarahisar, Turkey; **Mesa Hospital, Ankara, Turkey; ***Kocatepe University, Dep of Neurosurgery, Afyonkarahisar, Turkey
Purpose Empty Sella Syndrome (ESS) is the herniation of the cerebrospinal fluid found in the supracellar arachnoid space and the sella turcica resulting in compression of the pituitary gland. Several ophthalmic manifestations and visual field defects can be seen in ESS. In this study, we compared the visual field defects obtained with Rarebit perimetry and Humphrey field analyzer in patients having ESS.
Materials and Methods Twenty-five eyes of 13 patients having secondary ESS and 30 eyes of 15 control subjects were included in the study. After routine ophthalmic examination visual field testing was performed by Humphrey Visual Field Analyzer II (Fastpack 30-2 strategy) and rarebit perimetry (regular test). Statistical analysis was performed by independent-samples t-test and ROC curves.
Results In the Humphrey visual field, MD was measured to be −3.46 dB in the control group and −5.95 dB in patients having ESS (p =0.024). Pattern standard deviation, CPSD and SF were measured to be higher in patients having ESS (p =0.040, p =0.261 and p =0.040, respectively). Mean hit rate was measured to be 90.24% in control group and 75.96% in cases having ESS (p =0.001). Mean hit rate was found to be significantly decreased in the temporal quadrants and upper nasal quadrant in patients having ESS.
Conclusion Humphrey visual field and rarebit perimetry provide comparable and reliable data in cases with ESS.
II.6.4 (p60)
Short-term Visual Outcomes of Transsphenoidally resected pituitary adenomas
Burcu Kazanci1, Burak Kazanci2, Gölge Acaroğlu1, Murat Bavbek1, Bayazit Ilhan1?
1Ministry of Health Ulucanlar Eye Hospital; 2Ministry of Health Yildirim Beyazit Hospital–-Neurosurgery Department, Ankara, Turkey
We present the visual outcomes of 11 consecutive patients who were operated trans-sphenoidally for pituitary adenoma during the year 2006 in our neurosurgery department and had at least 3 months of follow up. There were 5 males and 6 females, their mean age was 42,2 (+/−-11,1).
Preop symptoms included headache in 6 (54,5%), acromegaly in 4 (36,4%),menstrual dysfunction in 4 (36,4%), hirsutism in 2 (18,2%), and galactore in 1 (11,1%). Headache continued in only 1 acromegalic patient with an adenoma larger than 20 mm who had increased growth hormone and prolactine levels.
Preoperative visual acuity increased from 0,84 (+/−-0,25) to 0,9 (+/−-0,17) post operatively. Two patients remained color vision defective. Five patients had uni or bilateral temporal optic disc pallor, and in 3 patients c/d ratios were greater than 0,3. These findings did not change post operatively. Preoperatively 9 patients had visual field defects, 4 of which were bitemporal hemianopic, and in 2 patients who had only 3 months of follow up and large adenomas, these defects persisted postoperatively.
II.6.5 (p61)
Apoplectic Optochiasmal Syndrome due to Posttraumatic Pituitary Apoplexy
Burcu Kazanci, Gölge Acaroğlu, Burak Kazanci
1Ministry of Health Ulucanlar Eye Hospital and Ministry of Health Yildirim Beyazit Hospital,Neurosurgery Department, Ankara, Turkey
We report a case of bilateral optic neuropathy resulting from hemorrhage into a pituitary adenoma following minor head trauma. Presenting symptoms were reduced visual acuity and headache. Enlargement of optic chiasm and optic nerves in acute stage and normalization after medical therapy is demonstrated by magnetic resonance imaging (MRI).Optic neuropathy and MRI results were not thought to be related by the department of neurosurgery. Therefore, pulse steroid treatment was given for presumed bilateral papillitis. Visual acuity, visual field defects, and optic fundi improved in the following week. In this patient, our therapy for presumed bilateral papillitis replaced acute surrenal insufficiency by chance.
II.6.6 (p92)
An unusual visual field due to demyelinating disease affecting the optic chiasm
Jorge Arruga1, Laura Gubieras2, Carlos Majös3, Silvia Muñoz1, Txomin Arbizu2
1Department of Ophthalmology, Hospital Universitari de Bellvitge. Institut Català de Retina. Barcelona, Spain; 2Multiple Sclerosis Unit. Department of Neurology, Hospital Universitari de Bellvitge. Barcelona, Spain; 3Institut de Diagnòstic per la Imatge. Centre Bellvitge. Hospital Universitari de Bellvitge. Barcelona, Spain
Objective To report an uncommon visual field defect in a patient with demielinating chiasmal neuritis.
Background Chiasmal neuritis is an ocasional clinical manifestation of multiple sclerosis, usually presenting as bitemporal upper quadrantic, hemianopic or scotomatous defects, or juntional scotomas in the visual fields.
Design/Methods Observational case report.
Results A 37-year-old woman experienced acute visual loss in her LE, with pain on ocular movements. Visual acuity was 20/20 RE and 20/60 LE, with a decreased color vision in the LE and a left RAPD. The ocular fundus was normal OU. Perimetry revealed a relative upper temporal vertical step in the RE and a similar upper nasal defect, as well as a dense temporal hemianopic defect, in the LE. Neurological examination was otherwise unremarkable. Brain MRI showed on T2 three hyperintense lesions at the juxtaventricular white matter, as well as a lesion involving the left optic nerve and the left half of the optic chiasm, that enhanced after contrast on T1. The CSF study disclosed oligoclonal bands, not present in serum. All the visual symptoms and signs resolved after the patient was treated with a megadose of methylprednisolone, and the image of the left chiasm decreased on follow up.
Conclusion This is an interesting combination of visual field defects, presumably due to involvement of the hemiretinal nasal fibers of the LE before decusating, and of the fibers arising from the lower periphery of the left hemiretina in both eyes, joining at the central part of the left hemichiasm, in the context of a clinically isolated syndrome, suggestive of MS.
II.7. Iatrogenic Neuro-Ophthalmologic Disorders—Poster Presentations
II.7.1 (p1)
Benztropine-induced Esotropia and Mydriasis
Sun-Young Oh, Byoung-Soo Shin, Ji Soo Kim, Yeon-Hee Lee, Ae Young Lee
Department of Neurology Chonbuk National University, Departments of Neurology and Ophthalmology, Chungnam National University, and Department of Neurology, Seoul National University
Anticholinergic drug may cause dry mouth, bladder dysfunction, constipation, impaired cognition, and visual disturbance. With repeated doses of certain anticholinergics, reduction in the near point of accommodation has been reported. Benztropine mesylate, a tertiary-amine muscarinic receptor antagonist frequently used to antagonize the parkinsonian side effects of antipsychotics, may impair accommodation and near visual acuity, especially when used with neuroleptics.
We recently experienced esotropia and dilated pupils in a patient who had received haloperidol and benztropine mesylate to treat Tourrette syndrome. Immediate resolution of the esotropia and mydriasis by discontinuation of benztropine mesylate indicates a causal relationship between the medication and the ocular signs. To the best of our knowledge, this is the first report on esotropia concurrent with mydriasis induced by anticholinergics.
II.7.2 (p39)
Epiretinal membrane secondary to vigabatrin: A case report
Didar Ucar1, Cengiz Aras1, Ahmet Sarici1, Ebru Gorgun2
1Istanbul University Cerrahpasa School of Medicine Ophthalmology Department, Istanbul; 2Yeditepe University Eye Hospital, Istanbul, Turkey
Purpose Vigabatrin is a selective, enzyme-activated, irreversible GABA aminotransferase inhibitor. Visual field abnormalities have been reported in adults and children treated with this antiepileptic drug. The site of toxicity is proposed to be the retina, where GABA is an important modulatory transmitter .It has been shown to cause accumulation of GABA in the retinal Muller cells. We report on a case of bilateral epiretinal membrane in a patient under medication of vigabatrin for the treatment of epilepsy.
Methods A 42-year-old epileptic male patient, who has been using vigabatrin 1000 mg/day for 3 years was referred to our clinic for ophthalmologic examination. A complete ophthalmologic examination was performed.
Results His visual acuity was 20/20 in both eyes. Fundus examination revealed bilateral asymptomatic epiretinal membrane. OCT confirmed the presence of epiretinal membrane.
Conclusion Although it may be coincidental, this is the first report of epiretinal membrane formation likely due to chronic use of vigabatrin.
II.7.3 (p49)
A case with bilateral optic nerve atrophy associated with isotretinoin treatment for acne vulgaris
Umut Asli˙ Dinc, N. Melda Yenerel, Ebru Görgün, Deniz Oral, Banu Öncel, Murat Öncel
Yeditepe University Eye Hospital, Istanbul, Turkey
Purpose To report a case with bilateral optic nerve atrophy associated with isotretinoin use for the treatment of acne vulgaris.
Methods A complete ophthalmologic evaluation including visual acuity, intraocular pressure measurement, anterior segment and fundus examinations was performed. Central 30 degrees static visual field (VF) and 45 degrees kinetic VF was tested by Humphrey perimetry. Electroretinophysiologic (ERG) testing was performed by Retiport32. Retina nerve fiber layer (RNFL) was evaluated by Stratus-OCT. Cranial magnetic resonance imaging (MRI) and MRI angiography were also performed together with systemic and neurological examination.
Results An 18-years-old girl presented with sudden onset of severe ocular pain and headache lasting for two weeks. She was taking 80 mg/week isotretinoin for the last 6 months. Her medical history revealed a preterm delivery secondary to maternal pre-eclampsia. Visual acuity was 20/20 and anterior segment findings were normal in both eyes. Fundus examination revealed optic disc pallor particularly in temporal area in both eyes. Direct and indirect light reflexes were normal without any afferent pupillary defect. Central 30 degrees static and 45 degrees kinetic VF revealed left inferior quadranopia without macular sparing. Average RNFL thickness was 69.8 μ and 70.2 μ in the right and left eyes respectively. RNFL loss was detected in superior and inferior quadrants in the right eye and in superior and nasal quadrants in the left eye. Neurologic examination and systemic evaluation was normal. A defect in the right temporoparietoccipital periventricular white matter was found in cranial MRI that was attributed to possible hypoxia during pre-term delivery. MRI angiography revealed normal findings. Pattern-ERG, photopic ERG, scotopic ERG and 30 Hz flicker were within normal ranges in both eyes. Pattern visual evoked potential (VEP) testing revealed significant decrease in P100 amplitude without a significant delay in latency. P100 amplitude was 1.5 ìV and 3.3 ìV in the right and left eye respectively.
Conclusion In the present case, left inferior quadranopia seems to be a coincidental finding and independent of isotretinoin use. Although it is not certain, isotretinoin may cause optic atrophy consistent with significant amplitude decrease in pattern-VEP and RNFL loss in Stratus-OCT without decrease in photopic ERG.
II.7.4 (p84)
The effect of contact lens wear on the excitability of blink reflex circuit: a prospective controlled study
Meral E. Kiziltan1, Guzin Iskeleli2, Derya Uluduz1
1Department of Neurology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey; 2Department of Ophthalmology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey
Objective To determine whether the use of contact lenses constitutes a good model for adaptive alterations.
Methods The study included 8 volunteers using contact lenses (6 females, mean age 23.0 ± 5.3) and 11 volunteers (6 females, mean age 26.2 ± 4.8 years) as control group. The eye blinking reflex (EBR) was measured bilaterally using the double stimulation technique at 200, 400, 600, 800, and 1000 ms. The test was performed once for the control, and three-times for contact lens group, before the use of lenses, and at the end of the 1st week and 1st month.
Results EBR latencies were normal in both groups. No differences were observed between groups in initial values of R2 response recovery at 200, 400, 800, and 1000 ms. However, the 600 ms measurements were significantly lower in the contact lens group (p =0.037). The recovery at 600 ms was significantly altered with time. There was a decrease in the 1st week, which reached the value of the control group in the 1st month (p=0.049).
Conclusion It was concluded that the contact lens does not cause an increase in the excitability of the interneurons that affect the EBR cycle.
II.7.5 (p131)
Uremic optic neuropathy–-case report
Beyza Citci, Vildan Ozturk, Geysu Karlikaya, Hasan Huseyin Karadeli, Ulkem Yakupoglu, Ferda Ciftci, Canan Aykut Bingol
Yeditepe University Hospital, Departments of Neurolog, Ophtalmology and Nephrology
Aim Acute visual loss can be caused by a variety of conditions affecting the retina, optic nerve or brain. Optic neuropathy due to uremia is a rare cause of acute visual loss.
Case Report A 78 year old female patient presented to our hospital with sudden loss of vision in both eyes. Five days before admission vision loss started in her right eye and moved to the left eye after a few days. There was no pain with eye movement. She had a history of end stage chronic renal insufficiency related to cisplatin-induced nephrotoxicity. She was treated with periton dialysis, which was interrupted during the last 10 days due to peritonitis. She was not hypertensive or diabetic. Her neuroophtalmologic examination displayed diminished visual acuity in both eyes (20/200 on the left eye, no vision on the right eye). She had no other neurological deficit. Her VEP examination displayed a prolonged P 100 latency of 134 ms on the left eye and no response on the right eye. Her visual field examination demonstrated. Her laboratory data included blood urea nitrogen-101 mg/dl, creatinine 9.47 mg/dl, hemoglobin 11.4 g/dl. Nonreactive VDRL and HIV. She had multiple lacunary infarcts on her cranial MRI examination. The patient was diagnosed with uremic optic neuropathy and was treated with urgent periton dialysis and intravenous methyl prednisolone (375 mg, 5 days). This treatment led to a rapid improvement in the visual acuity in the left eye (20/50). The optic neuropathyin uremia may be classified as non-ischemic neurotoxic uremic optic neuropathy, ischemic optic neuropathy (anterior ischemic optic neuropathy) and optic neuropathy as result of drug side effects, benign intracranial hypertension and optic neuritis.
Conclusion The immediate institution of dialysis and corticosteroid therapy can optimize the chances of visual recovery in these patients. The close cooperation between neurologists, ophtalmologists and nephrologists is important in order to treat this interdisciplinary emergency.
II.8. Genetics Related Neuro-Ophthalmology Diseases—Poster Presentations
II.8.1 (p4)
Ophthalmologic symptomatology in Bulgarian Roma patients with intermediate form of Niemann-Pick disease
S. Cherninkova1, V. Mihaylova1, I. Tournev1, J. Hantke2, I. Sinigerska3, R. Tincheva4, À. Oscar1, L. Kalaydjieva2
1Clinic of Neurology, University Alexandrovska Hospital, Sofia; 2 Centre for Medical Research and Western Australian Institute for Medical Research, The University of Western Australia, Perth, Australia; 3 Laboratory of Molecular pathology, University Hospital of Obstetrics and Gynecology, Sofia; 4 University Pediatric Hospital, Sofia, Bulgaria
Niemann-Pick disease, an autosomal recessive disorder, results from mutations in the SMPD1 gene, leading to deficient activity of the lysosomal acid sphingomyelinaså. Except for two classical forms of Niemann-Pick disease–-type A and type B, atypical intermediate forms are described.
À group of 20 Bulgarian Roma patients from 16 unrelated families with an intermediate form of Niemann-Pick disease was investigated. Macular halo, slight variant of cherry-red spot, is the most consistent finding, present in 16 of the 18 ophthalmologically examined patients. Macular halos in our patients differ in appearance and size, showing an age-related progression in size. The mean ratio of the outer diameter of the halo to the optic disc diameter is 0.75 ± 0.28. Visual functions are spared in our patients.
II.8.2 (p43)
Oct abnormalities in retinal nerve fiber layer in autosomal dominant optic nerve atrophy
Ana Blázquez, Patrizia Amati-Bonneau, Cristina Masuet, Silvia Muñoz, Pascal Reynier, Jorge Arruga
Department of Ophthalmology. Hospital Bellvitge, Barcelona, Spain; Department of Biochimie et Génétique. CHU; INSERM U 694, Angers, France. Department of Ophthalmology. Institut Català de Retina, Barcelona, Spain; Department of Preventive Medicine. Hospital de Bellvitge, Barcelona, Spain
Purpose To compare retinal nerve fiber layer (RNFL) abnormalities in patients with autosomal dominant optic nerve atrophy (ADOA) caused by OPA1 gene mutation, against patients with bilateral optic nerve atrophy of unknown cause using optic coherence tomography (OCT)
Method Twelve patients with longstanding progressive visual loss, normal IOP, bilateral optic disk pallor, including the neuro-retinal rim, and symmetrical visual field defects (centrocaecal, supero-temporal or diffuse) were enrolled. Exposure to toxics and drugs had been ruled out by negative history. Results of laboratory tests (serology for syphilis, B-complex vitamins, Leber DNA mutations), as well as cerebral MRI, had been unremarkable. Retinal peripapillary fiber layer thickness was measured using RNFL thickness average analysis protocol fast RNFL 3.4 with Stratus OCT, version 3. DNA samples from serum of all patients were obtained, amplified and sequenced to detect OPA 1 gene mutation. Finally, OCT results obtained were statistically compared, by means of U Mann-Whitney test, between OPA1 + and OPA1-groups.
Results OPA1 mutations linked to chromosome 3q were identified in 6 out of 12 patients. A statistically significant difference was found (p=0.02) in RNFL thickness, in a comparative study between OPA1 + and OPA1-groups, consisting of a selective preservation of optic nerve fibers at the nasal sector in the OPA1 + group. Differences were not statistically significant in the other three sectors (superior, inferior and temporal).
Conclusions OCT provides a major knowledge in various disorders of the optic nerve. It may be useful in differentiating the pattern of RNFL abnormality in autosomal dominant optic nerve atrophy type OPA1, characterized by a selective preservation of the nasal sector, from optic neuropathies of unknown cause (OPA1 -), in which this selective preservation is not observed.
II.8.3. (p91)
Optic pathway gliomas in children with neurofibromatosis: clinical and genetic evaluation
A. Varan, Y.K. Terzi, S. Oguzkan, S. Ayter, B. Anlar, T. Kansu
Departments of Pediatric Oncology, Medical Biology, Pediatric Neurology, and Neurology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Background Neurofibromatosis type 1 (NF1) can be associated with optic gliomas, more frequently in children under age 10. The Hacettepe Neurofibromatosis Study Group has been working in close interdisciplinary collaboration for the diagnosis and consultation of NF1 patients.
Aim Of children with optic pathway tumors seen in the group, 17 (10 boys, 7 girls, age 1 month–13 years, median 7 years) with adequate follow-up and genetic analysis are presented.
Results The localization of the tumor was unilateral in 12 patients, with right and left sides equally involved, and bilateral in five. One patient had a glioma in the hypothalamus and optic radiation without optic nerve involvement. The chiasmatic and prechiasmatic regions, hypothalamus and optic tract were frequently accompanying the optic nerve (n=10).
Fourteen patients have stable disease in 1–10 years' follow-up. Two (age 2.5 and 9 years) received chemotherapy and radiotherapy with no tumor regression, and are currently under follow-up with stable disease.
Haplotype analysis was done in 15/17 patients. One patient was shown to carry the 499delTGTT mutation described before in the NF1 gene exon 4b. This patient's mother had cafe au lait spots, axillary freckling and a plexiform neurofibroma; two offsprings aged 21 and 23 years had cafe au lait spots and axillary freckling, and the other offspring aged 26 years had optic glioma in addition to the same skin findings. This situation suggests the effect of environmental factors and modifying genes on the severity of the disorder. The phenotypic variation among patients within one family can be explained by differences in genetic status and exposure to environmental effects, and creates difficulty in establishing a genotype-phenotype relationship in NF1.
II.8.4 (p99)
Is the diagnosis acquired cone dystrophy ? Or a new type of central autoimmune retinopathy?
Sevim Kavuncu1, MollyM Gilbert2, Peter J Savino2
1Ankara Ulucanlar Eye Hospital, Ankara, Turkey; 2Wills Eye Hospital Neuro-ophthalmology, Philadelphia, PA, USA
Purpose To make a reasonable explanation for a group of 11 patients presenting with normal fundus and biomicroscopic examination, adult onset subacute progressive visual acuity loss and color vision loss, that we had difficulty in explaining the cause of the symptoms.
Material Eleven patients with subacute progressive visual loss were referred to the neuro-ophthalmology department of Wills Eye Hospital. After routine neuro-ophthalmologic examination, they had Humphrey visual field, MRI, OCT, ERG, VEP,mVEP, mERG test done. They had a report from their family physician completed examination for yearly cancer work-up. They had no toxic medication and alcohol abuse. After evaluation of the each patient in the group, 5 patients were included in the study.
Results All of the patients had visual loss. None of the patients had systemic disease. All patients had an normal fundus examination except the patient with granular appearances in both maculas. All patients had visual field loss in Humphrey visual field. Three of the patients had normal and two had abnormal ERG testings. All patients had abnormal central isoelectricity in mERG testings. All patients in the group had normal MRI, OCT and VEP testings. mVEP testing with Accumap showed different patterns.
Conclusion It is likely that, these patients with such clinical presentations may have an acquired, or possibly an autoimmune cone dysfunction. We collected blood to differentiate autoimmune retinotpathy and cancer associated retinopathy from a new named disease presenting with late onset acquired cone disfunction. We sent the blood samples to the laboratories of a university in order to include our patients in a multicenter study.
II.8.5 (p110)
Lymphotropic therapy in a complex with the electrical stimulation of the optic nerve at children with the optic atrophy
A.G. Aubakirova, G.K. Toksanbaeva, S.S. Chuprina
Kazakh Eye Research Institutå, Almaty, Kazakhstan
Purpose study of efficiency of application lymphotropic therapy in a complex with electrical stimulation of the optic nerve at children with optic atrophy.
Material and Methods Under observation were 39 (70 eyes) with optic atrophy of various genesis. Among principal causes of optic atrophy were marked: a pathology of the central nervous system, craniocerebral traumas, hereditary anomaly, congenital anomaly. At 4 etiological factors to establish it was not possible. Observable children have been parted on 2 groups: patients of the basic group (34 eyes) received the complex therapy with base medicamental lymphotropic treatment in a combination to electrical stimulation of optic nerve. Lymphotropic therapy carried out by introduction of cerebrolysinum in quantity 1.0 ml subcutaneously in the lymphotropic zone of the periorbital zone. The electrical stimulation spent with use of the apparatus the “Serdolic” (MNTC, Russian). Duration of a session from 5 till 15 min. Children of control group (36 eyes) received standard treatment in a complex with the electrical stimulation. Ophthalmologic research included investigation of visual acuity, perimetry, ophthalmoscopy, electrophysiological investigation (the visual caused potentials).
Results and discussions The comparative analysis of results of treatment has shown, that in the basic group visual acuity was enlarged on 0.14: with 0.2 ± 0.05 up to 0.26 ± 0.08 (p < 0.05), in control group on 0,08: with 0.16 ± 0.04 up to 0.24 ± 0.02 (p < 0.05). In the basic group the peripheric field of vision has extended on the sum of 8 meridians on 36.2 ± 14.2, in control on 22.8 ± 14.2. Improvement of functional results of treatment was expressed in authentic augmentation of amplitude of the basic positive peak P2 from 7.5 ± 1.8 mkV and decrease of a latence of the visual caused potentials from 120.4 ± 2.2 ms to 113.4 ± 3.8 ms in the basis group. Change of amplitude P2 was from 7.2 ± 2.3 to 8.9 ± 1.6, latence of the visual caused potentials was in control group insignificant and had no authentic character.
Conclusions The offered way is the effective method as a result of which authentic improvement of visual functions and a functional condition of a retina is established.
II.8.6 (p124)
Is the clinical picture of leber hereditary optic neuropathy changing?
Wefers Bettink1, M. Remeijer2, L. Spruijt2,3
1Neuro-ophthalomology Department Het Oogziekenhuis Rotterdam, Rotterdam, the Netherlands; 2Department of Genetics and Cell Biology University of Maastricht, Maastricht,the Netherlands; 3Department of Human Genetics, University Medical Centre Nijmegen, the Netherlands
Purpose To report a series of patients with a late onset of Leber Hereditary Optic Neuropathy
Methods A series of nine patients with a (sub) acute bilateral loss of vision, caused by late onset of Leber Hereditary Optic Neuropathy (LHON)
Results Age of onset in these patients varied from 48–73. (mean 61.4) 7 male and 2 female patients. Visual Acuity varied from HM to 0.1 Four had a positive family history for LHON. None had a history of alcohol abuse, but most of them were “social” drinkers. Four of the patients were heavy smokers (1 female). None of the patients had a Vitamin B12 deficiency. There were no clues for toxic optic neuropathy or other deficiencies. Mutations found were mainly two of the three primary mitochondrial DNA mutations: 11778G>A, 3460G>A
Conclusion Nine patients with a (sub) acute bilateral loss of vision had a late onset Leber Hereditary Optic Neuropathy. Mean age 61.4. Usually LHON develops earlier in life with a peak in the age of 15–35.
II.8.7 (p128)
Electrophysiological proof of midline axonal crossing abnormalities in congenital horizontal gaze palsy with progressive scoliosis
Çağri˙ Mesut Temuçin1, Mehmet Demirci2, Tülay Kansu2
1Hacettepe University Institute of. Neurological Sciences and Psychiatry Ankara, Turkey; 2Hacettepe University, Faculty of Medicine, Department of Neurology, Ankara, Turkey
Purpose To study electrophysiologically motor and sensory pathways in patients with horizontal gaze palsy with progressive scoliosis (CHGPPS)
Background During the development of central nervous system, ipsilaterally projected axons must resist against crossing the midline where contralaterally projecting axons must cross but only once. It has been shown that, a mutation in Robo 3 protein, one of the axonal guidance molecules that act in this process, can cause autosomal horizontal gaze palsy in patients with CHGPPS.
Methods and Patients Electrophysiological studies were done in 3 female and 1 male patients from 3 unrelated consanguineous families at the age of 5, 6, 11, and 19 years. They were referred to our neuro-ophthalmology Unit for evaluation of horizontal gaze palsy and three of them had progressive scoliosis. After stimulation of median nerve, somatosensory evoked potentials were obtained from ipsilateral sensory cortex, instead of contralateral side in all 4 patients. In two patients, transcranial magnetic stimulation induced motor evoked potentials were obtained from ipsilateral muscles. Ipsilateral silent period was observed from contralateral muscles and contralateral silent period from ipsilateral side. These findings indicate a midline crossing abnormality both in motor and sensory pathways. Robo 3 gene mutation was studied and detected only in one of our patients.
Conclusion Our findings provide an electrophysiologic evidence for axonal crossing abnormality in patients with CHGPPS.
II.8.8 (p150)
Papilledema in central neurofibromatosis
Huban Atilla, Teksin eryilmaz, Hakan Ulubay
Ankara University, Faculty of Medicine, Department of Ophthalmology, Visart Imaging Center, Ankara, Turkey
Purpose To report a case of neurofibromatosis type 2 with papilledema
Method Ophthalmological and radiological findings of the case were described.
Results Patient with diagnosis of neurofibromatosis type 2 admitted with the complaints of headache and blurry vision. Ophthalmological examination revealed bilateral papilledema with hyperemic optic nerve head. Cervical and cranial MRI showed multiple gliomas, meningiomas and bilateral acoustic schwannomas. MR venography showed no venous sinus thrombosis. Her CSF pressure was found to be high so she was treated with diuretic therapy and her complaints and papilledema resolved.
Conclusion Papilledema in neurofibromatosis type 2 is a rare finding and the underlying mechanism as well as the prognosis may vary. Frequent follow-ups and early intervention can prevent permanent visual loss and ophthalmologist must be aware of visual loss due to papilledema as well as classically defined optic nerve meningioma, optic nerve head glioma, retinal hamartoma and presenile posterior subcapsular cataract.
II.9. Optic Neuropathies—Poster Presentations
II.9.1 (p7)
Bicycles and traumatic optic neuropathy: Report of two childhood cases
İlkay Çaki˙r Mermut, Şeyda Uğurlu, Deniz Eğrilmez
Atatürk Research and Training Hospital, İzmir, Turkey
Purpose To present two cases of childhood traumatic optic neuropathy following cycling accidents.
Methods Case presentation.
Results Two adolescent boys aged 14 and 15 suffered collisions while riding bicycles with an additional passenger and no protective gear. They presented with unilateral no light perception and Marcus Gunn pupillary reaction. Both patients had periorbital ecchymosis along with hematoma over the involved eyebrows. Anterior segment examinations and ocular motility were within normal limits. Optic discs of the involved eyes had mild pallor and the margins were slightly obscured. Radiologic imaging with high resolution computed tomography and MRI revealed no optic canal fractures or intraoptic nerve sheath hemorrhage, however, multiple fractures involving the orbital walls were observed. Treatment was attempted with mega dose corticosteroids with no apparent improvement in both patients.
Discussion Optic neuropathy caused by high velocity injuries sustained with motor vehicles is not uncommon. Although cycling injuries are lower velocity accidents, adolescents and young adults can reach high speeds. The addition of a passenger might increase the weight and thereby the momentum of the vehicle especially during down hill riding. These two cases of consecutive optic neuropathies caused by very similar circumstances emphasize the importance of protective head gear during cycling. Children and adolescents should be counseled rigorously to use cycling safety practices and the need to avoid riding with a passenger.
II.9.2 (p27)
Contrast sensitivity alterations related with coronary artery bypass graft surgery
T. Küsbeci1, G.F. Yavas1, M. Emmiler2, A. Cekirdekci2, F. Öztürk1
1Kocatepe University, Dep of Ophthalmology, Afyonkarahisar, Turkey; 2Kocatepe University, Dep of Cardiovascular Surgery, Afyonkarahisar, Turkey
Purpose Ocular complications related with emboli, haemodilution, or platelet related dysfunction have been shown after coronary artery bypass graft surgery (CABG). Visual loss can occur from ischemic perioperative optic neuropathy. In this study, we aimed to investigate the changes in contrast sensitivity in patients undergoing CABG who have normal visual acuity and to compare the effect of off-pump and on-pump CABG on contrast sensitivity.
Materials and Methods Sixteen patients undergoing CABG were enrolled in the study. All patients underwent routine ophthalmic examination before surgery. Contrast sensitivity at five spatial frequencies [1.5, 3, 6, 12, and 18 cycles per degree (cpd)] was measured using Functional acuity contrast test before surgery and at the 5th day after surgery. Scores were obtained for each frequency and statistical analysis was performed using paired samples t-test.
Results Mean preoperative contrast sensitivity scores at 1.5, 3, 6, 12, and 18 cpd spatial frequencies were 27.6 ± 8.8, 50.4 ± 11.7, 57.0 ± 19.3, 35.6 ± 12.4, and 22.4 ± 9.8, respectively. Mean postoperative contrast sensitivity scores at 1.5, 3, 6, 12, and 18 cpd spatial frequencies were 33.8 ± 4.9, 48.0 ± 12.9, 54.0 ± 14.3, 30.8 ± 14.3, and 22.0 ± 12.2, respectively. The change was not significant in all frequencies (p =0.61 for 1.5 cpd, p=0.49 for 3 cpd, p=0.98 for 6 cpd, p=0.7 for 12 cpd and p=0.36 for 18 cpd). The difference in contrast sensitivity scores among off-pump and on-pump CPBG was not significant.
Conclusion Our results indicate that contrast sensitivity does not change at the 5th day after off-pump and on-pump CABG. Evaluating contrast sensitivity alterations in patients who had undergone CABG in larger series may give valuable information.
II.9.3 (p29)
A comparison among Humphrey Field Analyzer, Microperimetry and Rarebit Test in the evaluation of the macula in Primary Open Angle Glaucoma
F. Öztürk1, G.F. Yavas1, T. Küsbeci1, S.S. Ermis1, P. Aydi˙n2
1Kocatepe University, Dep of Ophthalmology, Afyonkarahisar, Turkey; 2Mesa Hospital, Ankara, Turkey
Purpose Glaucoma is an optic neuropathy characterized by retinal ganglion cell loss. Retinal nerve fiber layer is primarily composed of ganglion cell axons, it is 4–6 layers and forms 35% of the retinal thickness at the macular area. Macular thickness can be affected in glaucoma. The aim of this study was to evaluate functional changes of macula in glaucoma patients using Humphrey field analyzer (HFA), Microperimeter-1 (MP-1), and rarebit perimetry, also to report the correlations among these diagnostic tests.
Materials and MethodsTwenty patients with early primary open angle glaucoma and 15 normal control subjects were enrolled into the study. Macular threshold was evaluated by HFA II 10–2 threshold test, MP-1 Humphrey 10–2 pattern test for central 10° and by rarebit fovea test for the central 3°. Results were evaluated by independent samples t-test and ROC-curves.
Results Macular sensitivity was found to be significantly decreased in glaucoma patients (for HFA p =0.002, for MP-1 p=0.004 and for rarebit perimetry p=0.010). For HFA area under curve (AUC) was 0.763, for MP-1 AUC was 0.653 and for rarebit perimetry AUC was 0.703. The difference between HFA, MP-1 and rarebit perimetry was not significant (between HFA and MP-1 p=0.27, between HFA and rarebit perimetry p=0.58, between MP-1 and rarebit perimetry p=0.11).
Conclusion Our study suggests that MP-1 and rarebit perimetry are as reasonable as HFA in the evaluation of functional macular defects in primary open angle glaucoma.
II.9.4 (p41)
The efficacy of brimonidine tartrate 0.15% in patients with optic atrophy
Ahmet Ozer, Afsun Sahin, Nazmiye Erol, Hikmet Basmak
Eskisehir Osmangazi University, Medical Faculty, Department of Ophthalmology, Eskisehir Turkey
Aim To evaluate the efficacy of topical brimonidine on visual function in patients with optic atrophy due to non-arteritic anterior ischaemic optic neuropathy (NAION) and methanol intoxication.
Material and methods Fifteen patients with optic atrophy due to NAION and 8 patients with optic atrophy due to methanol intoxication were included in this study. Informed consent form was obtained from all the study participants. Optic atrophy was unilateral in the NAION group, and bilateral in the methanol intoxication group. In all patients' affected eyes, perception and projection were negative. Eye vision losses were for less than 6 months. Optic atrophic eyes were started on brimonidine tartrate 0.15% eye drops two times daily for up to 6 months. Visual function was evaluated by performing visual evoked responses every month during the study period. The amplitudes and latancies of P100, N70, and N145 for each eye were recorded.
Results There were 10 male and 5 female patients in the NAION group with a mean age of 51.2 ± 3.4 years (range: 43 to 56 years), and 7 male and 1 female patients in the methanol intoxication group with a mean age of 54.7 ± 8.9 years (range: 33 to 67 years). The amplitudes and latancies of P100, N70, and N145 did not change significantly in any eyes in both groups during follow-up.
Conclusion Brimonidine is a highly selective and potent alpha-2 adrenergic receptor agonist, which lowers intra-ocular pressure and is believed to be neuroprotective. The optic atrophy is characterized by ganglion cell loss due to certain etiologies. A drug with neuroprotective activity might increase the vitality of degenerative retinal ganglion cells. In the current study, topical brimonidine tartrate 0.15% showed no significant improvement on visual function loss of retinal ganglion cells. In future, a drug with neuroprotective activity might increase the resistance of retinal ganglion cells to degenerative etiologic factors and therefore enhance survival before development of optic atrophy findings.
II.9.5 (p46)
Nonarteritic anterior ischemic optic neuropathy following refractive surgery
Nazife Sefi Yurdakul, Safiye Yi˙lmaz, Ahmet Maden
İzmir Atatürk Education and Research Hospital, İzmir, Turkey
Purpose To present a case of unilateral nonarteritic anterior ischemic optic neuropathy (NAION) following laser in situ keratomileusis (LASIK) surgery.
Methods: A 53-year-old man with moderate hypermetropia underwent bilateral LASIK. Complete eye examination including pupillary reactions, color vision, intraocular pressure measurement, anterior and posterior segment examination was performed. Preoperative clinical records, detailed medical history, central 24-2 SITA standard achromatic perimetry (Humphrey Field Analyzer II), serologic evaluation, cranial and orbital magnetic resonance imaging were obtained.
Results While best corrected visual acuity was 10/10 in both eyes preoperatively, in the left eye decreased suddenly to 1/10 after uncomplicated LASIK. After the surgery the patient had optic disc edema initially, developed optic atrophy later and visual recovery isn't obtained in the affected eye. There was a relative afferent pupillary defect and color vision defect in the left eye. Visual field examination showed a dense field defect in the diseased eye and normal in the unaffected eye. Preoperatively, intraocular pressures were normal and family history was negative for glaucoma. Searching for different etiologies of the optic neuropathy was resulted negatively. Therefore he was presumed to have an NAION following LASIK.
Conclusions NAION is the most extraordinary complication of refractive surgery. The etiology may be related to the increase in intraocular pressure during the surgery by the suction ring. The patient who has hypermetropia may be particularly susceptible for ischemic optic neuropathy because of small disc and cup/disc ratio. Risks of the procedure should be told to every LASIK patient before the surgery.
II.9.6 (p48)
Linezolid optic neuropathy
B F. Sánchez Dalmau1, L. Pascual2, A. Aranda 1, M.D. Vela3, A. Burés 1, A. Adán1
1Institut Clínic d'Oftalmologia (ICOF). Hospital Clínic. Barcelona; 2Servei d'Oftalmologia. Hospital Mútua de Terrassa; 3Servei d'Hematologia. Corporació de Salut del Maresme I la Selva, Spain
Introduction Linezolid is an oxazolidinone antibiotic that is increasingly used to treat drug-resistant, gram-positive pathogens. The mechanism of action is inhibition of bacterial protein synthesis. Methicillin-resistant Staphylococcus aureus infections are a special indication. Although maximal recommended therapy is 28 days, treatment durations greater than this are common. Optic and/or peripheral neuropathy and lactic acidosis are reported side effects in treatments over 4 weeks.
Methods Observational case report.
A 70-year-old male, with gastroesophagic reflux, hypercholesterolemia, received a hip replacement surgery. Because high suspicions of prosthesis infection, a surgical reevaluation was done. Exudate culture was positive to methicillin-resistant Staphylococcus aureus infection (MRSA). He started therapy with linezolid 600 mg bid, initially intravenous during inpatient and after discharge oral at the same dose during 4 months. At this point, the patient reported bilateral visual loss. Ophthalmological examination disclosed decreased visual acuity, dyschromatopsia, and cecocentral scotomas, characteristic of bilateral toxic optic neuropathy. The treatment was discontinued. Several months later his visual function had partially improved.
ConclusionLinezolid inhibits mitochondrial protein synthesis with potentially severe clinical consequences. With prolonged linezolid therapy, ophthalmologists should be aware that bilateral optic neuropathy is possible. Early withdrawal of the antibiotic may be associated with visual recovery. Prolonged courses of linezolid should be avoided if alternative treatment options are available.
II.9.7 (p50)
Bilateral anterior ischemic optic neuropathy in patients under dialysis for end-stage renal disase. A two-case report
José Nieto Enríquez, Jesus Torres Blanch, Miguel Angel Zapata, Alicia Galán Terraza
Hospital Vall d'Hebrón, Barcelona. Spain
Purpose To describe two cases of non-arteritic AION in two patients with end-stage renal disease (ESRD).
Case 1 56-year-old male under ambulatory peritoneal dialysis every night for ESRD of unknown cause. He presented to the ER complaining of bilateral painless visual loss on awakening. His visual acuity was R.E : 20/400 L.E: Hand movement. His pupils were poorly reactive, without RAPD. Fundoscopy revealed bilateral pale swelling of the optic nerves, with two splinter hemorraghes on the left. Arteritic, uremic, toxic and Leber's optic neuropathies were ruled out. He referred morning arterial blood pressures of 90/60 mmHg. In spite of switching the patient to hemodialysis, improved blood pressure control, and steroids, vision declined to counting fingers in R.E and remained stable in L.E.
Case 2 29-year-old male under hemodialysis for bilateral congenital hypoplasia. He complained of painless visual loss in his left eye after hemodialysis, where he had presented an hypotensive event, with transient,bilateral visual loss. His visual acuity was R.E 20/30 L.E 20/200, with left RAPD. His left optic nerve was pale and swollen. The following days, his left eye evolved to no light perception. He recieved no treatement. One week later he presented with reduced visual acuity in his right eye, again after hemodialysis. His right optic nerve was then pale and swollen, and evolved to no light perception within a few days in spite of steroids and blood pressure control.
Comment Patients under dialysis for ESRD are at increased risk of cardiovascular disease, and generalized atherosclerotic disease is the major cause of death in the long-term dialysis population. Ischemic optic neuropathy (ION) is a recognized complication of both hemodialysis and peritoneal dialysis, and may present as bilateral and simultaneous, which is rare otherwise. Hypotension seems to be the most important precipitating factor behind this ophthalmologic emergency. This, together with atherosclerosis and anemia, put these patients at increased risk for ION. Differential diagnosis includes mainly uremic and toxic optic neuropathy. Prevention of this devastating complication, giving special care to blood pressure control, is crucial, because once installed, treatment will probably be ineffective.
II.9.8 (p52)
Analysis of nerve fiber layer thickness after supposedly partial non-arteritic anterior ischemic optic neuropathy
Michel Van Lint, Marijke Wefers Bettink-Remeijer
The Rotterdam Eye Hospital, Rotterdam, Netherlands
Purpose To see if in patients with a seemingly partial non-arteritic anterior ischemic optic neuropathy (NAION), the corresponding retinal nerve fiber layer (RNFL) of the unaffected visual field is affected.
Design Retrospective, observational case series.
Methods 16 patients with partial NAION were selected with an accompanying altitudinal visual field defect at presentation, of whom 11 were unilateral and 5 bilateral. We define a NAION as being partial, when the visual fields demonstrate an altitudinal defect at presentation and an ophthalmoscopically altitudinal pallid disc edema. Only two patients presented with an inferior unilateral NAION, whereas the remaining 14 patients had a superior NAION. The superior NAION group can be further subdivided in 9 unilateral cases and 5 bilateral. Four patients of the five bilateral NAIONs, met the inclusion criteria with only one eye, due to unreliable GDX measurements (see methods). This yielded a superior NAION group of 15 eyes.
GDX scanning laser polarimetry has been performed to assess the RNFL thickness. An age matched control group of 13 patients was created for comparison. RNFL thickness was compared between the eyes with a NAION and the normal fellow eye and the eyes from the control group. Both absolute values and relative superior:inferior RNFL thickness were compared.
Results In our 14 selected patients with superior NAION, we observed a significant reduction in RNFL thickness for both the superior and the inferior half, compared to the normal fellow eyes and the control group.
Conclusions In apparently partial superior NAION both the superior and inferior RNFL thickness are diminished.
II.9.9 (p53)
Unilateral exophthalmos and optic neuropathy in a patient with graves' disease
Özlem Barut Selver, Zeynep Ozbek, Emel Ada, Ismet Durak, Meltem Soylev Bajin
Dokuz Eylul University School of Medicine, İzmir, Turkey
Purpose To report a patient with Graves' disease who had an asymmetrical exophthalmos and unilateral optic neuropathy.
Case Report A 45-year-old woman with a two-year history of Graves' disease presented with right exophthalmos and progressive visual loss. The duration of exophthalmos was 2 years and that of visual loss was 1 year. She was treated with intravenous pulse corticosteroids, followed by oral steroids with the diagnosis of optic neuropathy due to thyroid ophthalmopathy with no responce during the last 2 months. She had undergone magnetic resonance imaging two times and computed tomography scans one time for the diagnosis of her orbital disease since the beginning of her symptoms all of which were reported as active thyroid ophthalmopathy. Neuro-ophthalmic examination revealed right exophthalmos and right optic nerve dysfunction. Ocular motility was full with no lid retraction. Consultation of her images with neuroradiologists revealed typical radiological characteristics of sphenoid ridge meningioma. Her optic nerve functions improved after surgical decompression.
Conclusion Although thyroid ophthalmopathy is the most common cause of either unilateral or bilateral exophthalmos, detailed clinical examination and neuroimaging are necessary especially in unilateral and/or asymmetrical cases.
II.9.10 (p54)
Serum uric acid levels are decreased in patients with optic neuritis and multiple sclerosis
A. Tsakiri, Jette Frederiksen
Department of Neurology, Glostrup Hospital University of Copenhagen, Denmark
Background A few small series have reported low serum levels of the endogenous antioxidant uric acid (UA) in patients with multiple sclerosis (MS) and patients with optic neuritis (ON). We aimed to confirm this in a population-based greater material and to correlate the values to results of CSF examination and MRI of the brain.
Design/Methods The mean age of patients was 38 years. The first group comprised 76 consecutive untreated patients with acute ON: fifty-three patients (70%) had clinically isolated ON. The remaining 23 patients (30%) had ON as part of MS. The second group comprised 33 consecutively examined patients with relapsing remitting (RR) MS (mean disease duration 1.6 years). The reference interval of UA was 0.20–0.45 mM.
Results Twelve patients (15%) with ON had decreased UA, of whom 9 had isolated ON. In RRMS 5 patients (15%) had decreased UA. In ON the frequency of decreased UA was significantly higher in women than in men (p=0.03).
Conclusions: Decreased serum values of UA was present in 15% of patients with ON or RRMS, equally distributed between these two groups. Only in ON was the frequency of decreased UA significantly higher in women than in men. The relationship of UA levels and the results of CSF and MRI findings will be presented at the congress.
II.9.11 (p55)
Prognosis in ischemic optic neuropathy
Bilge Çetin, Fatma Polat, Önder Akyürekli
Ege University Medical Faculty, Neurology Department, İzmir, Turkey
IntroductionIschemic optic neuropathy (ION) can be divided into 2 groups: anterior and posterior. Nonartheritic ION which is characterized by sudden, painless, unilaterally visual loss has an ischemic pathogenesis. Risk factors determined in ethiology are found to be small optic disc, atherosclerosis, diabetes mellitus, hyperlipidemia, hypertension, hypotension, hemoconcentration, hemodilution and hypercoagulations. Various medical and surgical treatments including antiagregan, decompression of optic nerve, high dose corticostreoids, levodopa, carbidopa, hyperbaric oxygen and neuroprotective agents have been tried.Yet any useful treatment is absent.
We aimed to evaluate risk factors, efficiency of the treatment and the prognosis in whom has the diagnosis of ION.
Method The patients who have ION diagnosis in 2002–2006 in neuroopthalmology department are icluded in our study. Visual acuity, fundus examination and visual field of the patients are noted. Total of 54 patients, 42.6% (23) were male, 57.4% (31) were female.
ResultsTreatment and prognosis are compared and found to be the only 25% of the ASA group has partial remission in visual acuity, 25% complete remission and 50% no benefit.ASA and Levodopa group 12.5% has complete remission, 25% partial remission and 62.5% no benefit. Improvement in vision compared in two groups (ASA and ASA + levodopa) and no difference is found statistically between them. (p=0.675)
II.9.12 (p59)
Optical Coherence Tomography in Optic Neuritis and Multiple Sclerosis: A Review
K. Kallenbach, J.L. Frederiksen
Department of Neurology, Glostrup Hospital, Denmark
Aims Optical coherence tomography (OCT) is a new non-invasive high-resolution method that measures the retinal nerve fiber layer thickness (RNFL). An overview of the use in optic neuritis (ON) and multiple sclerosis (MS) is presented.
Method Literature survey on PubMed.
Results The RNFL thickness in eyes of healthy control subjects was 102.9–111.11μ, in eyes affected by ON 59.79–85μ, and in fellow eyes 82.73–99.8μ. All studies found a significant reduction of the RNFL in eyes affected by ON compared with fellow eyes and with eyes of healthy controls. Two out of three studies found a significant reduction of the RNFL in fellow eyes compared with control eyes. One study showed that atrophy after ON typically developed between 3 and 6 months after onset of visual loss. The RNFL thickness was correlated with visual acuity, visual field, low-contrast letter acuity, contrast sensitivity, and color vision. Correlations were also found with the optic nerve area evaluated by MRI, neurological impairment score, and increasing disease duration. One of two studies found a significant correlation with amplitudes of visual evoked potentials, neither correlated with latencies.
Conclusion Further investigation of OCT measurements in patients with ON and MS is needed, but OCT is a promising new tool to evaluate RNFL atrophy in ON patients and MS patients.
II.9.13 (p64)
Sjogren's Syndrome Presenting With Neurological Symptoms
A. Kurne1, A.I. Isikay1, U. Kalyoncu2, K.K. Oguz3, B. Anlar4, R. Karabudak1
1Department of Neurology, Faculty of Medicine; University of Hacettepe, Ankara, Turkey; 2Department of Rheumatology, Faculty of Medicine; University of Hacettepe, Ankara, Turkey; 3Department of Radiology, Faculty of Medicine; University of Hacettepe, Ankara, Turkey; 4Department of Pediatric Neurology, University of Hacettepe, Ankara, Turkey
Aim The neurologic manifestations of primary Sjogren syndrome can mimic multiple sclerosis. Especially the differential diagnosis of some particular cases beginning with “clinical isolated syndrome” such as optic neuritis and transverse myelitis may be difficult in clinical and radiological aspects.
Case Report We present two interesting cases whose neurological symptoms begin before the systemic signs of Sjogren syndrome. They both have recurrent optic neuritis and transverse myelitis having different prognostic properties when compared with multiple sclerosis.
Both patients were referred to our hospital as multiple sclerosis resistant to immunomodulatory treatment. They had serious sequela from optic neuritis and transverse myelitis. Lesions on brain MRI were not suggestive of MS and they had acute transverse myelitis longer than four vertebral segments which is atypical for multiple sclerosis. After insistent examination, laboratory data and lip biopsy, we have confirmed the diagnosis of Sjogren syndrome
Conclusion Although the relapsing remitting course, female dominance and neurological signs resemble multiple sclerosis, the systemic symptoms, cerebrospinal fluid properties and vasculitic markers can be informative for Sjogren syndrome. As the treatment procedure is different we suggest that Sjogren syndrome should always be thought in the differential diagnosis of the patients resembling multiple sclerosis.
II.9.14 (p65)
Bilateral anterior ischemic optic neuropathy associated with the combined therapy of chronic hepatitis C with PEG-Interferon alpha 2-a and Ribavirin
Ligia Onisim, Raluca Ungureanu, Victoria Arama
Clinical Emergency Hospital “Bagdasar-Arseni”, Bucharest; Clinical Medical Hospital “Victor Babes”, Bucharest; Infectious Diseases Institute “Prof. Dr. Matei Bals”, Romania
Background The chronic hepatitis C treatment is based on the association of two drugs peginterferon alpha 2-a/2-b and ribavirin. Following this therapy different neuro-ophthalmologist reported the appearance of unilateral AION or retinal changes. Only few cases of bilateral AION associated with this therapy are communicated in the literature till now.
Case A 55-year-old woman, treated for 6 months with peginterferon alpha 2-a and ribavirin for chronic hepatitis C, experienced a sudden painless impair of vision in her right eye, associated with inferior visual field defect, no colour perception and mild edema of the optic disc-clinical suspicion of right AION. The blood tests and head CT ruled out other disorders which could explain the visual disturbance.After few days without any positive visual changes, patient received intravenous methylprednisolone 500 mg for 3 days followed by oral prednisolone, keeping the therapy with peginterferon. She noticed a slowly recovery in her right eye vision, but after 6 weeks she came with blurring of vision in her left eye, visual field disturbances and papilloedema. The right optic disc became pale meantime. In this moment the ophthalmologist suggested interruption of peginterferon-ribavirin association and concluded a bilateral AION induced by this therapy.
Comments The literature describes a small number of cases with unilateral AION and only few cases with bilateral AION induced by interferon therapy for chronic hepatitis C. Despite of right AION we tried to keep the treatment with interferon in association with steroids and we were very hopeful when the right visual function improved slowly. Because after 6 weeks the left eye revealed signs of AION, the therapy with interferon was discontinued. Hepatitis C treatment should be stopped promptly when important ophthalmologic complications occur.
II.9.15 (p69)
Anterior ischemic optic neuropathy and Turner's syndrome
Ana Fonseca, Manuela Novais, Ivone Cravo, Fátima Campos, M. Monteiro Grillo
Hospital de Santa Maria, Lisbon, Portugal
Purpose To report one case of an optic ischemic anterior neuropathy in a adult female with Turner's syndrome.
Design Case report.
Methods Thirty-six-year-old Caucasian female, with Turner's syndrome, under hormonal therapy (oestrogen and progesterone) that presented with sudden and painless decrease in visual acuity OD. The following examinations were performed: visual acuity, pupillary light reflexes, slit-lamp examination, fundoscopy, Farnsworth-Munsell 15 chromatic test, visual field testing, fluorescein angiography. Laboratory evaluation of vasculopathic risk factors and neuroimaging were requested.
Results The clinical and functional evaluation revealed an optic ischemic anterior neuropathy OD. The neuroimaging studies were normal. The laboratorial evaluation revealed no other identifiable vasculopathic risk factors, besides Turner's syndrome.
Conclusions Turner's syndrome is one of the most common chromosomal abnormalities in females, with a wide array of phenotypical manifestations, being diagnosed by karyotipe. It is associated with a shorter life expectancy, primarily due to cardiovascular complications, including atherosclerotic events. Ophthalmic problems are seen in 63% of women with Turner's syndrome, but no ischemic optic nerve events are reported, previously. Taking that into account, it should be considered in the differential diagnosis of an optic ischemic anterior neuropathy, particularly in young females, without other recognizable risk factors.
II.9.16 (p72)
Bilateral optic neuritis after influenza vaccination
Funda Uysal Tan1, Cengiz Akarsu2, Reyhan Gullu2, Tulay Kansu1
1Department of Neurology, Faculty of Medicine, Ki˙ri˙kkale University, Turkey; 2Department of Ophthalmology, Faculty of Medicine, Ki˙ri˙kkale University, Turkey
Introduction Optic neuritis is a rare complication of vaccination such as smallpox, hepatitis B, polio, tetanus toxoid, measles-rubella and influenza. The mechanism of post-vaccination optic neuritis is immune mediated demyelinization of the optic nerve. We report a patient with bilateral optic neuritis following influenza vaccination.
Case Report A 55-year-old woman was admitted to neurology outpatient clinic with pain on eye movements and bilateral gradual loss of vision for 10 days. She had influenza vaccination 4 weeks before and 3 weeks after that she noted blurry vision and pain on the orbits. Ophtalmological examination revealed best corrected visual acuity of counting fingers at 1 m on the left and 3/10 on the right. Extraocular motility was intact. Fundoscopy demonstrated mild optic disc swollen and hyperaemia on the right and bilateral engorged veins. There was no relative afferent pupillary defect. Ishihara plates for color vision were 1/17 on the right and 0/17 on the left. Visual fields on the Humphrey perimeter demonstrated absolute scotoma on the right and infero-nasal defects on the left. Fundus fluorescein angiography showed no vascular or macular pathology. Visual evoked response was normal on the right but there was no potential on the left. Cerebrospinal fluid analysis was normal except mild protein elevation (63 mg/dl). Cranial magnetic resonans imaging (MRI) and orbital MRI were also normal. A diagnosis of bilateral optic neuritis was made. The patient was treated with intravenous methyl-prednisolone (1 g/day) for five days followed by slow tapering of oral prednisolone (1 mg/kg/day). After 6 months the disk edema resolved on both eyes and the visual acuity also recovered both on the right (9/10) and on the left (8/10). Mild optic disc pallor was noted on the left.
Conclusion This case implicates influenza vaccination as a cause of optic neuritis. The patient had good recovery of vision following steroid treatment.
II.9.17 (p73)
Bilateral anterior ischemic optic neuropathy following second-trimester abortion related hemorrhage
Funda Uysal Tan1, Zafer Onaran2, Pelin Yilmazbaş2, Yüksel Onaran3
1Department of Neurology, Faculty of Medicine, Ki˙ri˙kkale University, Turkey; 2Department of Ophthalmology, Faculty of Medicine, Ki˙ri˙kkale University, Turkey; 3Gynecology and Obstetric Specialist, Middle East Clinic Ankara, Turkey
Introduction Bilateral acute ischemic optic neuropathy is a rare complication of massive hemorrhage and related hypotension and anemia in young individuals. We report a patient with bilateral non-arteritic ischemic optic neuropathy (NAION) after an abortion related massive hemorrhage.
Case Report A 34-year-old woman presented with sudden painless visual loss in her left eye after an abortion complicated with massive hemorrhage. Visual acuity was 20/20 in the right eye and hand motion in the left. There was also a relative afferent pupillary defect (RAPD) in the left eye. Fundus examination revealed bilateral swollen, hyperemic optic discs and neurofiber layer hemorrhages. Brain MRI and magnetic resonance venography were normal. Lumbar puncture revealed clear and colorless cerebrospinal fluid with an opening pressure of 16 cmH2O. Thus, the diagnosis was bilateral NAION and intravenous pulse corticosteroid therapy (1000 mg/day) was administered for three days. On the 6th day, optic disc edema regressed bilaterally and visual acuity improved to finger counting at 2 m in the left eye. On the 3rd week, in the left eye, the visual acuity was 20/80, the visual field showed only a small spared area in the nasal region and persistent RAPD was present. Ocular findings persisted on the 2nd month.
Conclusion Severe acute hemorrhage is an important risk factor in healthy young individuals for NAION. Despite the absence of proven effective therapy besides the correction of hypotension and anemia, intravenous high dose corticosteroid may be a treatment option.
II.9.18 (p76)
Unilateral papilledema
J. Costa
Egas Moniz Hospital Lisbon
Aim Asymmetric papilledema is not rare but there is no consensus about its fisio-pathogenesis.
Case Report Our patient with unilateral papilledema and a very fast growing tumor adjacent to the origin of the left intra cranial optic nerve, allowed us to think that it probably blocks CSF in the sub arachnoidal space of the left optic canal and so protects the nerve from edema and it is the main cause of such an asymmetric/unilateral papilledema. The more dilated right optic nerve head also supports this hypothesis.
Conclusion Bilateral visual obscurations in this patient also suggests that the mechanism may not be due only to ischemia of the optic nerve head related with the severity of the papilledema but instead a more mechanical / pressure effect on the intracranial optic nerve, blocking intra axonal fast flow of both optic nerves.
II.9.19 (p79)
Short-term outcome of high dose intravenous methylprednisolone for optic neuritis in Turkey
Yasemin Ozdamar, Golge Acaroglu, Kuddusi Teberik, Bayazit Ilhan, Seyhan S Ozkan
Ulucanlar Eye Research Hospital, Ankara, TURKEY: Departments of neuro-ophthalmology and retina
Purpose To describe the clinical characteristics and outcome of optic neuropathy (ON) cases treated with high dose intravenous methylprednisolone (IVMP) in a tertiary referral clinic in Ankara, Turkey.
Methods Consecutive patients in the past 5 years with acute monosymptomatic ON, who were treated with IVMP and had a follow-up of at least 3 months were reviewed. Symptoms and findings, results of brain magnetic resonance imaging (MRI), etiologies, visual outcomes and side effects of the treatment were evaluated.
Results Sixty-seven patients with a mean age of 29.0 ± 10.81 and a mean follow-up of 7.86 ± 8.29 months met the inclusion criteria. The most common presenting complaint was blurred vision, followed by pain with ocular movements. Etiological causes were elicited in 57 (85.0%) patients, the most common of which was Multiple Sclerosis (MS). At least 1 MS–-compatible white matter lesion was present on brain MRI in 31 (46.2%) patients. Twenty-seven (40.3%) patients had clinically definite MS; in 17 of whom (25.4%), ON was the initial presentation. Sixty-one eyes out of 76 eyes (80.3%) had a final vision of at least 0.5. Only 2 eyes (2.6%) had a final vision of less than 0.1. Fourteen patients (20.8%) experienced mild and reversible side effects.
Conclusions Majority of our ON cases were associated with MS. Although our population did not exactly meet the Optic Neuritis Treatment Trial (ONTT) criteria, evidence from our study confirmed the short term benefit of the standard therapy recommended by the ONTT in our population.
II.9.20 (p81)
Clinical features of patients with nonarteritic anterior ischemic optic neuropathy
Bayazit Ilhan, Gokce Tasdemir, Golge Acaroglu, Cigdem Can, Sibel Polat, Orhan Zilelioglu
Ankara Ulucanlar Eye Education and Research Hospital Ankara, Turkey
Clinical features of our patients diagnosed as nonarteritic anterior ischemic optic neuropathy (NAION) between 2002–2006 and followed up for at least 3 months were studied retrospectively from their records. Seventy-five patients, aged 35–84 years (mean 59.9 ± 10.4) were included in the study. Of these, 8 patients were below 50. Forty-seven (62.7%) were male and 28 (37.3) were female. Follow up time was 3–24 months (mean 3.64 ± 2.78). Thirty patients (40%) had systemic hypertension and 22 (29.3%) had diabetes mellitus. NAION was found in the right eye in 32 (42%) and left eye in 43 (58%) patients. Decreased visual acuity and some degree of disk pallor in the other eye that might be interpreted as previous NAION were observed in 12 (16%) patients. Simultaneous bilateral involvement was not seen. Visual acuity was less than 20/200 in 41 (55%) and more than 20/60 in 22 (29%) patients. Most frequently encountered visual field defect was altitudinal defect, found in 53% of the 39 patients in whom visual field tests were done successfully. Cranial MRI was ordered in 40 patients. White matter hyperintensities were seen in 17 (42.5%) and normal cranial MRI was found in 16 (40%) of them. 34patients were observed without medication and aspirin was used in the rest. Increase in visual acuity defined as gaining 20/200 or more for those below that level and increase of two or more lines for those equal to or above 20/200, was seen in 34 (45%) patients at the last visit. There was no statistically significant difference between aspirin and no medication groups (p=0.455).
Although NAION is a disease of elderly, younger patients may also be affected. White matter hyperintensities in MRI and altitudinal defects in visual field tests were frequent findings. Increase in visual acuity could be seen and aspirin was not seen to be effective in recovery of visual acuity in this study. As the mean follow up time was rather short, effect of aspirin on prevention of the other eye could not be determined.
II.9.21 (p85)
Lyme disease, monosimptomatic type: Case report
Bianca Szabo, I. Szabo, D. Cirstina
Ophthalmology Department, Medical University “Iuliu Hatieganu”, Cluj Napoca, Romania
We present the case of a patient (a 23-year-old woman) with acute and complete loss vision of the left eye, and severe periodic left ocular and left orbital pain. Visual acuity right eye=20/20. Under steroid and nonsteroid general and local treatment, the visual acuity was improved (VA left eye=0.1) and a central scotoma was developed. Ophthalmoscopic analysis of the left eye showed elevated and blurred optic disc margins, retinal hemorrhages, venous congestion.
The diagnosis of borreliosis was based on clinical ocular findings and determinations of antibodies to Borrelia bugdorferi by enzyme-linked immunosorbent assay and immunoblot analysis, the detection of DNA of B. bugdorferi by polymerase chain reaction, and exclusion of other infectious and inflammatory causes (tuberculosis, toxoplasmosis, syphilis, and sarcoidosis were excluded). The commonly used blood, urine, and spinal fluid tests, cerebral MRI and angiofluorography were also done.
The patient received oral ceftriaxone 2 g daily for 4 weeks–-2 cures. Under the treatment, the clinical signs had significally improved (VA left eye=20/20), but the central scotoma remained.
The case is unusual because only one eye was affected (typically decreased visual acuity occurs on both eyes), other signs of the disease were absent and the recovery, under the antibiotic treatment, was excellent.
II.9.22 (p88)
Anterior ischemic optic neuropathy (aion) accompanied by herpes virus
M.S. Kasimova
Tashkent Institute of Postgraduate Medical Education, Republic of Uzbekistan
The Purpose of the research is to reveal the frequency of AION caused by herpes virus and specifics of this diseases. Ninety-seven patients with AION were examined within 2002–2006. All these patients went through clinical, ophthalmologic and laboratory assessments, plus special methods such as: MRT, KT of cerebrum, supersonic dopplerography of transcranial, ophthalmologic and neck mayor vessels. Laboratory assessments included: general blood and urine tests, biochemical blood examination, blood coagulation test and general immunogramm. Conjunctivae smear for herpes virus, CMV, toxoplasmosis and chlamydia has been carried out. The same infections were analyzed in the blood. All patients were examined by therapeutics, neurologists, neurosurgeons, ottolaringologist, urologist, immunologist and other specialists. In addition to all above mentioned methods of general study we added our own computer based program of the analysis of the scene fundus ocular.
As a result of our research we came to the conclusion that the specific features of AION accompanied by Herpes virus refer to the following: high subtitles of HV in conjunctivae and blood; a sharp reduction of the quip of the visual acuity; specific visual fields defects; comparative raising of intraocular pressure accompanied by decreasing the speed of eye liquid formation; pale choked optic disk; narrow arteries and sometimes wide veins; small hemorrhages on the disc; decreasing state of immunities; increasing of blood urea and creatinine shelters, increasing, the tolerance to glucose; KT or MRT signs to some atrophies of the temporal cortex; disorder of transcranial and major neck vessels blood circulation; increasing blood circulation in temporal artery, normal blood circulation in central retinal artery and reduction of blood circulation in ophthalmic artery and the short posterior ciliary's arteries supplying the anterior optic nerve.
II.9.23 (p89)
Bilateral papilledema in sleep apnea-Pickwickian syndrome
Özlem Barut Selver, Meltem Söylev Bajin, A. Osman Saatci
Department of Ophthalmology, Dokuz Eylul, University School of Medicine, Izmir, Turkey
Purpose To report a patient with Pickwickian syndrome and obstructive sleep apnea syndrome who presented with severe bilateral visual loss due to pseudotumor cerebri.
Method Case report of a 54-year-old patient who presented with a three-month history of visual loss in both eyes. He had been a heavy smoker for 20 years and had arterial hypertension for 5 years. He was referred for papilledema. His cranial CT scan was within normal limits. His pupils were equal in size and briskly reactive to light. No afferent defect was noted. Visual acuity was 20/200 OD and counting fingers at 3–4 meters OS. Color vision was 19/21 OD and 2/21 OS by Ishihara's pseudoisochromatic plates. Slit-lamp examination was normal. Fundus examination showed bilateral optic disc edema with peripapillar hemorrhage and macular edema. Mild blind spot enlargement and generalized depression were demonstrated in both eyes by automated static perimetry. A lumbar puncture showed normal cell count and chemistry, with an opening pressure of 350 mmH2O. Complete blood count revealed polycythemia that was considered secondary by the internists, depending on the long history of loud snoring and excessive daytime somnolence. Polysomnography was planned with a diagnosis of obstructive sleep apnea syndrome (OSAS) and Pickwickian syndrome. Phlebotomy was performed, hypertension was regulated and the patient was put on a diet program. Optic disk swelling resolved and visual acuity was 20/20 in both eyes at 2 months. A nocturnal polysomnography confirmed OSAS and Pickwickian syndrome. The patient was started on bilevel positive airway pressure (Bi-PAP) therapy.
Conclusion Chronic respiratory insufficiency (i.e., Pickwickian syndrome and OSAS) is one of the systemic diseases which cause pseudotumor cerebri. In these patients, the prognosis depends on the underlying respiratory problem. Careful sleep history and work-up should be obtained because treating the underlying cause may be more effective than traditional therapies for pseudotumor cerebri. Correction of hypercarbia leads to resolution of papilledema.
II.9.24 (p96)
Structural and functional evaluation of retina and optic nerve in patients with multiple sclerosis without optic neuritis attack
F.C. Gundogan1, S. Demirkaya2, G. Sobaci1
1Gulhane Military Medical Academy, Department of Ophthalmology; 2 Gulhane Military Medical Academy, Department of Neurology
Aim To determine: (1) the association of retinal neurodegeneration in MS without optic neuritis attack, (2) the value of structural and functional tests in evaluation of retinal neurodegeneration in multiple sclerosis (MS) patients without clinically-proven optic neuritis.
Methods Nineteen patients with a diagnosis of definite MS who had not been affected by optic neuritis attack, and 37 age-and sex-matched control subjects were enrolled. Retinal nerve fiber layer (RNFL) thickness was determined by optical coherence tomography (OCT, Zeiss). The measurements in each quadrant (superior, nasal, inferior, and temporal) were averaged. The data in all quadrants were identified as RNFLoverall, whereas the data obtained in the temporal quadrant-only were identified as RNFLtemporal .Pattern visual evoked potentials (PVEP) was recorded by using high contrast (99%) checkerboard stimuli subtending 15' and 60' of the visual arc. Multifocal electroretinogram (mfERG) response to a scaled 61 hexagonal pattern was averaged over five concentric rings (R1–R5). PVEP, ERG, and mfERGs were recorded using Roland Consult RetiSCAN system. Only the right eyes were included in the groups.
Results There was a significant (p =0.007) reduction in RNFLtemporal thickness in MS patients (study group: 70.2 ± 2.8, control group: 84.6 ± 17.1 micrometer). P100 latency was significantly prolonged, and P100 amplitude was significantly reduced in both 60' and 15' checks (p < 0.05). Rod response b wave implicit time, maximum combined response, and a and b wave implicit times were significantly prolonged (p =0.03, 0.009, <0.001, respectively). There was no significant difference in mfERG responses in all concentric ring analysis (p > 0.05). RNFLtemporal was correlated to both P1 peak implicit time in the central ring (R1) in mfERG (p < 0.05, r =−0.598), P100 latency for 60' (p < 0.05, r =–0.387),15' checks (p < 0.05, r=−0.441), and P100 amplitude for 15' check (p < 0.05, r =0.431). The most sensitive parameter-P100 latency to 60' check size-detected 12 over 19 patients (63%) outside limits of the 95th percent confidence interval for normative data.
Conclusion Retinal neurodegeneration exists in MS without optic neuritis attack, and PVEP and OCT values obtained from RNFL seems to be more sensitive and may be used as a biomarker in MS.
II.9.25 (p103)
The value of the tests used in the clinical evaluation of non-arteritic anterior ischemic optic neuropathy
Bilge Yamen, Arif Taylan Ozturk, Aylin Yamen, Meltem Soylev Bajin
Department of Ophthalmology, Dokuz Eylul University School of Medicine
Purpose To assess the sensitivity of the tests used for the diagnosis and evaluation of non-arteritic anterior ischemic optic neuropathy (NAION) and the correlations between them.
Methods The clinical records of 23 patients with unilateral NAION were reviewed. Patients who were 50 years of age or older with sudden and painless loss of vision, without any other underlying ocular disease which could cause a decrease in visual acuity or loss in visual field and a normal fellow eye were included in the study. Detailed neuro-ophthalmologic examination including relative afferent pupillary defect (RAPD), brightness sense, automated visual fields and color vision examinations with Ishihara's pseudoisochromatic plates were performed in all cases. RAPD and brightness sense were evaluated quantitatively by Cokin's neutral density filters.
Results A total of 23 subjects (14 women, 9 men) participated in this study; the mean age was 66.3 ± 7.6 years (range 51–80). The results of RAPD and brightness sense examinations were 0.3 log unit or more in 22 patients (95.6%). Likewise, decreases over 5 dB in mean deviation values were also found in 22 patients (95.6%) in visual field examinations. The most common visual field defect was lower altitudinal scotoma and was seen in 11 patients (47.8%). Visual acuity was decreased in 21 patients (91.3%). 15 patients could not identify 3 or more plates in color vision examination. Spearman correlation analysis indicated that there was a significant correlation among RAPD, brightness sense and visual field measurements. While visual acuity compared to RAPD, brightness sense and visual field measurements, a mild correlation was noticed. Statistical analysis showed that visual acuity measurement significantly correlated with color vision. Color vision measurement was found significantly different from RAPD, brightness sense and visual field analysis.
Conclusion: Clinically practical, reliable and cost-effective methods such as quantitative measurements of RAPD and brightness sense by neutral density filters help in the diagnosis and evaluation of the severity of NAION.
II.9.26 (p106)
Optic disc cupping in dominant optic atrophy
Marianne Wegener1, Birgit Sander1, John Thygesen2, Morten la Cour1, Pascal Hasler1, Miriam Kolko1, Michael Larsen1, Dan Milea1, Henrik Lund-Andersen1
1Glostrup Hospital; 2Rigshsopitalet, Copenhagen University
Introduction Dominant optic atrophy (DOA) is a classical cause of non glaucomatous optic disc cupping. Optic disc palor is a constant finding in this setting, associated with a variable degree of cupping, which can be found in 50 to 80% of cases. It has been stated that the optic disc cupping can make the distinction between glaucomatous and non-glaucomatous excavation difficult. The aim of our study was to assess optic discs features in three different groups: DOA patients, open angle glaucoma patients and normal controls.
Patients and Methods Six independent observers randomly assessed in a blinded fashion 35 fundoscopic images including 19 eyes of 10 genetically homogenous DOA patients, 6 eyes of 6 glaucoma patients and 10 eyes of 10 normal controls. Quantification of neuroretinal rim, optic disc excavation, peripapillary atrophy, and presence of grey crescent was recorded in each case.
Results Four observers out of six correctly classified 11 of the DOA eyes and all 16 non-DOA eyes (p < 0.0001). Mean cup-to-disc ratio was 0.28 in the normal eyes, 0.30 in DOA and 0.82 in the glaucoma patients. Only one DOA eye had an estimated cup-to-disc ratio >0.5. Five out of 6 observers found variable temporal pallor in 12 DOA eyes. Absence of temporal pallor was found only in 1 DOA eye. Five out of 6 observers found some degree of peripapillary atrophy in the three groups, and none had a grey crescent.
Conclusion Temporal pallor of the optic disc was the most characteristic parameter in our DOA population. Pathological optic disc cupping and neural rim crescent may not be such common findings as previously described, at least in our limited Danish population.
II.9.27 (p112)
A case with tuberculosis suffering from bilateral sudden loss of vision in the postpartum period
Ayşe Ki˙yak,Suzan Güven,Süheyla Köse,Önder Üretmen
Ophthalmology Department, Ege University School of Medicine
Purpose To present a case with tuberculosis suffering from bilateral sudden loss of vision during postpartum period.
Materials And Methods A 32-year-old woman was referred to Ege University School of Medicine Ophthalmology Department with the complaint of bilateral sudden loss of vision. After a detailed medical history was taken, she had undergone complete ophthalmological examination including electroretinography (ERG) and visual evoked potentials (VEP).
Findings She had delivered a normal spontaneous partus a month ago. She had diagnosed with tuberculosis a week ago and her visual acuity impaired gradually within three days after diagnostic bronchoscopy.
On the initial examination, her visual acuity was P(–)P(–) bilaterally, Any P-100 potential was not detected on pattern and flash VEP. ERG findings and fundus examination was normal. Since ocular findings were accompanied by numbness on the left upper and lower extremities, she was referred to the neurology clinic. Cranial MRG examination revealed an bilateral and symmetric increase in signals obtained from optic chiasm and optic tract. Also, there was edematous expansion in optic chiasm. Antituberculosis treatment together with pulse steroid therapy was initiated for the patient. Pulse steroid treatment consists of IV methyl prednisolone (1 g daily) for 3 days followed by oral prednisone (1 mg/kg/daily) for 11 days. The visual acuity improved on the 5th day of therapy. On the tenth day, visual acuities were 20/20 bilaterally. The patient developed a second attack of sudden significant visual loss 20 days after cessation of the steroid therapy. Systemic steroid therapy was reinitiated and the visual acuity improved to 20/ 20 within 15 days.
Conclusion Considering that the visual loss is recurrent and responsive to steroid and antituberculosis therapy, we could suggest that infectious and demyelinizating pathologies might be the cause of visual loss in this patient.
II.9.28 (p114)
Vi˙sual evoked potential(VEP) responses in the pati˙ents with glaucoma and ocular hypertension
Sevda Aydin Kurna1, Tomris Şengör1, Yasemin Ağirman1, Eren Gözke2, Semih Açikgöz1, Yelda Buyru Özkurt1, Müge Ünal2
1Fatih Sultan Mehmet Education And Research Hospital Ophthalmology Clinic; 2Fatih Sultan Mehmet Education and Research Hospital Neurology Clinic
Purpose To compare visual evoked potential(VEP) responses in the patients with glaucoma and ocular hypertension and to evaluate the effect of glaucomatous optic nerve damage on the latency of VEP results.
Methods A total of 43 patients with glaucoma (83 eyes, group 1) and 8 patients with ocular hypertension (15 eyes, group 2) are included in to the study. During the ophthalmic examination; visual acuity, intraocular pressure, corneal pachmetry, cup to disc ratio. Humphrey field analysis 30/2 and pattern VEP results are recorded.
Results VEP P100 latency was 116.0 ± 12.8 msec in group 1, 106.5 ± 2.7 msec in group 2 (p =0, Mann Whitney U test) and VEP N 75 to P100 amplitude was 8.76 ± 6.5 to 10.04 ± 3.8 μmV (p =0.08), respectively. Significant correlations were observed in patients between VEP P 100 latency and Humphrey visual field scores MD(r =0.64, p =0) PSD (r =0.31, p =0.005),cup to disc ratio(r =0.56, p =0) and N 75 to P100 amplitude with Humphrey visual field scores MD (r =-0.30, p =0,005), PSD (r =-0.24, p =0,03), cup to disc ratio (r =-0.21, p =0.04-Pearson)
Conclusion VEP P 100 latency increase and N 75 to p100 amplitude decrease correlating with other tests is observed in the glaucoma patients. VEP as a non-invasive, easy and objective method may have a role in the diagnosis and follow up of glaucoma for the evaluation of glaucomatous optic nerve damage.
II.9.29 (p118)
A comparative study of hardy-Rand-Rittler 4th edition and ishihara color plates for detection of color-vision defects in optic neuropathy
Ruth Huna-Baron, MD, Zohar Habot-Wilner, MD, Yoseph Glovinsky, MD
Goldschleger Eye Institute, Sheba Medical Center, Tel-Hashomer, Israel
Objective To compare the sensitivity of the updated Hardy-Rand-Rittler 4th edition (HRR) and Ishihara color plates to color-vision defects in patients with optic neuropathy.
Background Color vision testing is one of the most useful components of the sensory neuro-ophthalmologic examination. Although the American Optical HRR 1966 edition has been traditionally considered a better test for acquired dyschromatopsia the Ishihara has been more commonly used due to its availability. In 2002 the updated 4th edition of HRR was published. There is insufficient information about its sensitivity to dyschromatopsia in cases with optic neuropathy.
Methods Study group included consecutive patients with newly diagnosed optic neuropathy seen in the Neuro-ophthalmology clinic from February 2005 to September 2005. Control group included consecutive patients referred to eye clinic for causes other than optic nerve or retinal macular disorders. Only patients with visual acuity of 20/70 or better were included in both groups. Patients with congenital color blindness were excluded.
Patients underwent comprehensive eye examination and color-vision test in both methods at a random order under standardized lighting conditions.
Ishihara and HRR test scores were set as the number of identified plates out of 12 and 6, respectively.
Results Study group consisted of 44 patients (49 eyes). Control group consisted of 43 subjects (66 eyes). Mean Ishihara score in the study group was 9.75 ± 2.16 and 11.75 ± 0.33 in the control group, while mean HRR score was 2.39 ± 1.37 in the study group and 5.29 ± 0.47 in the control group. With a cut-off score of 11/12 and 10/12 the Ishihara test achieved specificity-sensitivity levels of 91–69% and 100–51%, respectively. The HRR test achieved a better specificity-sensitivity balance of 92–86% and 97–84% for cut-off scores of 4.5/6.0 and 4.0/6.0, respectively.
Conclusion HRR 4th edition is more sensitive in detecting acquired dyschromatopsia due to optic neuropathy than the Ishihara plates.
II.9.30 (p120)
Optic nerve hypoplasia: clinical features
Eyyüp Karahan, A. Tülin Berk, Aylin Yaman
Pediatric Ophthalmology Department, Faculty of Medicine, Dokuz Eylül University
Purpose The purpose of our study is to determine the ocular and systemic anomalies associated with optic nerve hypoplasia (ONH) in a pediatric population.
Material and Method The records of patients with a diagnosis of ONH were retropectively reviewed. Clinical assessment included visual acuity, strabismus, nistagmus, refractive status were documented and the results were compared with those of 32 age and sex matched normal subjects. Associated systemic and neurologic abnormalities, the presence of prematurity, consanguinity had been detected. Nineteen of the children (59.3%) underwent neuroimaging
Results Thirty-two children (58 eyes) were included in the study. Twenty-six of the patients had bilateral, 6 had unilateral involvement. Eleven out of bilateral cases (42.3%) had nystagmus, none of the patients with unilateral involvement demonstrated nystagmus. Twenty-four children (75%) had strabismus. Seven of those (29.2%) with strabismus had esotropia, fifteen (62.5%) had exotropia .Thirty-two eyes (55%) eyes had astigmatism equal or greater than 1.0 diopters (D). We observed that the hypoplastic children had significantly more astigmatism than the control group. The mean astigmatism in the right eye of ONH and control group was 1.1 D and 0.2 D respectively (p =0.001), The mean astigmatism in the left eye of ONH and control group was 0.86 D and 0.18 D, respectively (p =0.001). Fifteen children (84%) that underwent neuroimaging had positive imaging finding. Thirteen children (40.6%) had motor mental retardation, seven had cerebral palsy (21.8%). One child had hypoplasia in one eye and morning glory appearance in the other eye.
Conclusion Most of the patients with unilateral and bilateral ONH was found to have strabismus. Children that present with strabismus should have careful ophthalmologic examination for associated organic pathologies. We found the association of astigmatism and ONH in our group and this association highlights the importance of careful retinoscopy. Correction of refractive errors can diminish the development of amblyopia secondary to ONH.
II.9.31 (p126)
Bilateral optic neuropathy associated to Interferon-alfa treatment of chronic hepatitis C
Hans C. Fledelius, Anne Wiencke
University Eye Dept, Rigshospitalet, Copenhagen University, Denmark
Purpose To discuss the possibility of drug-related optic nerve toxicity as related to treatment of hepatitis C by Interferon-alfa and Ribavarin.
Case story: After a quiet clinical course over 10 years, eventually a liver biopsy showed chronic hepatitis and advanced fibrosis. In June 2005 systemic therapy was instituted (Pegintron and Ribavarin). Three months later she experienced decreased vision. We found a slow 0.3 acuity of both eyes and marked dyschromatopsia and constriction of visual fields. A few retinal exudates were observed, but no optic disc changes. By and large, the visual complaints persisted despite a 20% reduction in the dose of Pegintron. With prime regard to preserving liver function, this was what the hepatologists would allow. No special findings by serological work-up or MRI scan, and VEP was normal. The treatment series was withdrawn after one year. Subsequently her visual acuity increased to 0.6-0.7, though with only minor improvement regarding the deficits in colour perception and visual fields.
Discussion Mild and transient interferon related retinopathy is well known, and NAION cases were also described in literature. In our case there were no disc changes, and we held drug-related optic nerve toxicity for more likely than ophthalmic manifestations due to the basic hepatitis C infection. So far, we found no similar reports in literature.
II.9.32 (p130)
Neurosarcoidosis–-a case report
Beyza Citci, Geysu Karlikaya, Vildan Ozturk, Hasan Huseyin Karadeli, Ferda Ciftci, Canan Aykut Bingol
Yeditepe University Hospital, Departments of Neurology and Ophtalmology
Aim Neurological abnormalities can be seen in sarcoidosis and they may be the presenting symptom. Cranial nerve symptoms are a dominant clinical feature in neurosarcoidosis with the optic nerve being affected most commonly.
Case Report We present a 66 year old male patient who was admitted to our hospital because of sudden onset ptosis and diplopia. His systemic examination was normal. On neurological examination he had a partial 3rd cranial nerve palsy on the left with ptosis, diminished light reflex and extraocular muscle weakness and bilateral optic disc pallor. There was no other neurological deficit. He was diagnosed with multiple cranial neuropathy. His electromyograpic examination was normal. His diagnostic evaluation included laboratory analysis for tuberculosis, lyme disease, brucellosis, syphilis and the results were normal. His serum ACE level was normal but he was diagnosed with neurosarcoidosis on the basis of the elevated protein and high ACE levels in the cerebrospinal fluid (CSF). He was treated with prednisolone and he had prompt improvement in his ocular symptoms.
Conclusion The diagnosis of neurosarcoidosis is straightforward when the ophtalmological symptoms arise in patients with an established diagnosis of sarcoidosis. However it may be a diagnostic challenge when patients present with neuro-ophtalmic symptoms before systemic involvement. Not infrequently it may present with a multiple cranial neuropathy and even when the serum ACE is normal, determination of ACE activity in the CSF can help the diagnosis. And rapid intervention with intravenous corticosteroids are the mainstay of treatment.
II.9.33 (p132)
Papilledema as the presenting manifestation of neurobrucellosis
Ümit H. Ulaş2, Ozan Dağli˙1
1GATA Göz Kliniği-Ankara; 2GATA Nöroloji Kliniği-Ankara
Aim To report a case of neurobrucellosis with papilledema in a young male.
Methods A 20-year-old young male was admitted with a history of transient headache for 1 year and permanent headache for 1 month. He also had a transient diplopia for 1 month. He reported that he intook unpasteurized goat's milk in the past.
Results Neurologic examination was normal. In ocular examination, papilledema was observed in both eyes. No apparent gaze palsy was noted. Lombar puncture was done. Pressure was 285 mmH2o, Protein was 114 mg/dl, number of cell were 200 (all PMNL). Agglutination titer of antibodies to Brucella was 1:100. The other infection agents were not detected. The Diagnosis was Brucella infection. Patient was treated with rifampin 600 mg/day for 30 days, trimethoprim-sulfamethoxazole for 2 weeks, doxycycline 200 mg/day for 6 months, and ceftriaxone for 30 days. Patient's complaint decreased gradually and 2 months later, papilledema disappeared. Six months later, lombar puncture findings and agglutination titer of antibodies to Brucella was normal. Ten months later, the patient had no symptom.
Conclusion Papilledema can be the first sign of neurobrucellosis. History of ingestion of unpasteurized goat's milk should remind the neurobrucellosis in patients with bilateral papilledema.
II.9.34 (p135)
Antibodies to aquaporin-4 in a child with recurrent bilateral optic neuritis
J. Leu, F. Paul, P. Alles, S. Jarius, O. Aktas, F. Zipp, M. Bluthner, N. Schrage
Hospitals of the City of Cologne, Department of ophthalmology, Charitè, Berlin, Germany
Case Report We describe a 13 year old boy with recurrent bilateral optic neuritis, 6 years since the last bilateral neuritis episode, currently presenting with visual impairment RA 5/20 and LA 6/20, colour deficiency (Ishihara) and no specific findings in ophthalmoscopy. Visual evoked potentials showed reduced amplitudes and prolonged latency in both eyes. MRI-findings suggest mild Gd-enhancement in both retrobulbar optic nerves with no other MS-specific lesions. MRI of the spinal cord was normal. CSF diagnosis showed no elevated levels of glucose, cells or protein, nor any other signs of infection, and no oligoclonal bands. The patient's symptoms responded well to intravenous steroids.
IgG-antibodies to the brain water channel aquaporin-4 (AQP4) were detected in the patient's serum by a newly developed radioimmunoprecipitation aasay (ratio 14.16; cut-off 11), which tended to show a normal value under steroid therapy (ab-ratio <10 at follow-up). AQP4 has recently been described in patients with neuromyelitis optica (NMO), a rare inflammatory disorder of the CNS that preferentially attacks the spinal cord and optic nerves. There is increasing evidence that auto-antibodies against Aqp4 represent a highly specific marker of the disease.
Conclusion Whether these antibodies are of pathogenic relevance in NMO, remains to be shown. In this case, antibodies against AQP4 may indicate a risk for the conversion to NMO which will be evaluated in follow-up examinations.
II.9.35 (p136)
Persistent visual loss in pseudotumur cerebri with neurobrucellosis
Tanri˙verdi Çiğdem1, Tuğcu Betül1, Önder Feyza2, Sencan Sadi˙k1
1Baki˙rköy Dr. Sadi Konuk Research and Education Hospital, Ophthalmology Clinic; 2 Haseki Research and Education Hospital, Ophthalmology Clinic, Istanbul, Turkey
Aim We aimed to review clinical signs and treatment regimens in pseudotumor cerebri.
Case Report We report a case with persistent visual loss due to pseudotumor cerebri because of neurobrucellosis. A 25 aged female patient consulted to our clinic because of bilateral abdusens palsy and pseudotumor cerebri was examined. With the symptoms of headache, vomiting and diplopia, patient was diagnosed as neurobrucellosis by the infection clinic. A treatment of specific antibiotic and lumbar puncture for increased BOS pressure were done. The cranial MR and cranial MR venography surveys were normal. In the first examination in our clinic the visions were at counting finger level in both eyes. Light reflexes were bilateral reduced. She had left esotropia with limited adduction. Fundus examination revealed bilateral optic disc pallor secondary to poststasis optic atrophy. In endemic areas, in the presence of bilateral papilla stasis, one must consider and rule out neurobrucellosis.
Conclusion Neurobrucellosis may cause permanent blindness with the impacts of increase in the intracranial pressure and poststasis optic atrophy. Ophthalmologists should be guides about diagnosis and surgical treatment by optic nerve sheath fenestration.
II.9.36 (p143)
Bilateral papillitis due to terbinafine?
Fatma Yülek1, Nurullah Çaği˙l1, Hasan B Çakmak1, Emine K. Akçay1, Şaban Şimşek1
1First Ophthalmology Department of SB Ankara Atatürk Training And Research Hospital
Purpose Evaluating a patient with bilateral papillitis and normal intracranial pressure.
Methods Neuroophthalmological and biomicroscopic examination of the 43 year-old male patient complaining of slightly decreased vision for about one month in both eyes, were performed. Ancillary tests involved lumbar puncture, magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), magnetic resonance venography (MRV)and some serological markers together with serum biochemistry, sedimentation rate and serum CRP.
Results The patient had noticed slightly decreased vision on the right eye one month ago. Two weeks later the vision of left eye was also affected. He had no significant headache or other neurological symptoms. The patient was using only terbinafine HCl for onychomychosis as systemic medication. The visual acuities (VA) of the right and the left eye were 0.8 and 1, respectively on the Snellen chart. There was slightly decreased color vision of the right eye. Fundus examination revealed bilateral low grade papilledema. Lumbar puncture (LP) was planned, but it was postponed due to patient's problems. One month later the patient presented with VA of 0.3 in both eyes. The pupillary light reflexes were weak in both eyes, but color vision was normal. The right optic disc was slightly pale and the left one was edematous with a splinter hemorrhage on the superior pole. His blood pressure and intracranial pressure (measured during the LP) were normal. The MRI, MRA, and MRV were all within normal limits. The results of the serological markers and serum biochemistry were not significant. The patient was followed for 8 days. Meanwhile he stopped taking terbinafine and was discharged with VA of 0.5 in both eyes. Five days later the vision of both eyes was 0.8. Right optic disc edema had resolved and the temporal side of left optic disc was slightly edematous.
Conclusion Bilateral papillitis due to terbinafine HCl has not been reported before. However, the intracranial pressure, imaging and the other tests were within normal limits. These findings together with the coincidence of improvement of vision and removal of the medication suggest that terbinafine HCl may cause papillitis.
II.9.37 (p144)
A case report of bilateral Morning Glory Disc Anomaly (MGDA)
Birsen Gokyigit, Mehmet Cakir, Serpil Akar, Vedat Kaya, Seren Mert
İstanbul Beyoglu Education and Research Eye Hospital, İstanbul, Turkey
Aim Morning glory disc anomaly (MGDA) is usually known to be unilateral.
Case Report We report an unusual case of a bilateral form of the disorder. The patient was 8-month-old boy. He had no systematic anomaly. He had nystagmus on both eyes. In eye examination anterior segment was normal. Posterior segment examination showed large MGDA on both eyes.
II.9.38 (p151)
Bilateral occipital lobe stroke with inferior altitudinal defects.
Atilla Bayer, Gökhan Özge, Fatih M. Mutlu
GATA Department of Ophthalmology, Ankara, Turkey
Aim Patients who survive cerebrovascular accidents exclusive of the occipital lobe often have no significant neurological deficits other than visual-field loss.
Case Report We report a patient who had visual symptoms and no neurologic complaint. Visual-field defects from occipital lobe stroke typically include congruous homonymous hemianopsias or quadranopsias, with or without macular sparing. A 75-year-old man came to us with symptoms of sudden loss of vision. Visual-field testing revealed a bilateral inferior altitudinal defect with normal optic nerve and fundus appearance in both eyes. Visual acuity was 6/10 in the right eye and 8/10 in the left eye. Slit-lamp examination showed that grade 2 nuclear cataract in both eyes. There was no pupillary defect. He was using drugs for diabetes mellitus, hypertension, and coronary artery disease. Two years ago, he had a coronary bypass surgery. On radiological examination, he was found to have had a bioccipital lobe cerebrovascular accident secondary to an embolic event causing the artery occlusion. After neurological consultation, patient referred to department of neurology for advanced examination and treatment. Inferior altitudinal defect is usually seen in patients with ischemic optic neuropathy. In our case, fundus examination was normal.
Conclusion Patients with infarction exclusive of the occipital lobe typically have no other neurological deficits except visual-field loss but these patients must be evaluated for the source of embolic event to avoid recurrence.
II.9.39 (p153)
Bilateral optic neuropathy after influenza vaccination
Didem E. Erdemir, Feyza Önder
Haseki Education and Research Hospital Eye Clinic İstanbul, Turkey
Purpose To evaluate the optic neuropathy which occurred after the influenza vaccination.
Method The visual acuity, visual field, fundus examination and MRI results of the patient with optic neuropathy after the influenza vaccination were evaluated.
Results A 63-year-old male patient with bilateral, sudden visual loss which occurred 2 days after the influenza vaccination. The visual acuity of the right eye was at the level of hand movement, and the left eye was at the counting finger level. Both optic discs were normal. Bilateral pupillary reactions were poor.
Conclusion Optic neuropathy is a rare complication following influenza vaccination. Visual loss may be unilateral or bilateral. As for the etiology, cases of demyelinization induced by immune system or ischemia have been mentioned in the literature with the case reports.
II.9.40 (p154)
Optic disc cupping in various optic neuropathies
Feyza Önder, Didem E. Erdemir
Haseki Education and Research Hospital Eye Clinic İstanbul, Turkey
Purpose To report the cases with nonglaucomatous cupping.
Method and Materials A case report, etiologic and clinical properties of 4 patients with nonglaucomatous cupping were evaluated.
Results The physiological cup of the optic disc is usually enlarged in glaucoma patients and is defined as the optic disc cupping. The manifestation of optic disc cupping caused by reasons other than glaucoma is very rare. In this study 4 patients with nonglaucomatous optic disc cupping were evaluated. In 1 case, arterial anterior ischemic optic neuropathy, in 1 case methylalcohol toxic optic neuropathy, in 1 case traumatic optic neuropathy, and in 1 case compressive optic neuropathy (anterior clinoid meningioma) were detected. The patients' intraocular pressure (IOP), investigated by applanation tonometry and evaluated according to the results of corneal thickness measurements by pachymetry, was normal. Optic disc cupping was shown in all cases by Heidelberg retina tomography (HRT). The differences between the glaucomatous cupping and nonglaucomatous cupping were evaluated.
Conclusion When the IOP measurements of the cases with optic disc cupping are normal, the ischemic, toxic, compressive and traumatic optic neuropathies must be remembered as the differential diagnosis of normal tension glaucoma, and must be excluded by neuro-ophtalmologic investigation and, if necessary, neuroradiologic imagining must be performed.
II.9.41 (p163)
Leber's hereditary optic neuropathy diagnosed in a 6-year-old-female with no family history
Haneen Jabaly-Habib, MD1, Anat Kesler, MD2
1Ha-emek Medical Center, Afula 18101, Israel; 2Ichilov Medical Center, Tel-Aviv, Israel
Aim Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease. It is the second most common hereditary optic neuropathy that presents more in males, mainly between the second and fourth decade of life.
Case Report A 6-year-old-female was referred due to left eye low vision following a school visual acuity screening program. Eye examination, Best corrected visual acuity (BCVA): right eye (RE) 6/6, left eye (LE) 6/60. Color plates RE 10/11, LE 1/11. Pupils had slow reaction to light and RAPD could not be evaluated appropriately due to photophobia. Confrontation visual field test: RE normal, LE large central scotoma. Fundus examination was normal in both eyes. Brain and orbital MRI was normal. Two months later on follow-up: BCVA: RE 6/18, LE 2/60. Fundus examination showed RE paripapillary tiny telangiectatic vessels and LE temporal optic disc pallor. Patient underwent a comprehensive investigation that was normal, but genetic test for LHON came back positive to 3460 mutation. Last follow-up examination: BCVA in both eyes: 2/60, temporal pallor of both optic discs.
ConclusionLHON should be considered in cases with progressive visual loss especially when other abnormalities are ruled out.
II.10. Teaching Neuro-Ophthalmology—Poster Presentations
II.10.1 (p11)
Training in Neuro-Ophthalmology: A personal experience
S.J. Hickman, G.T. Plant
Department of Neurology, Royal Hallamshire Hospital, Sheffield (SJH); Department of Neuro-Ophthalmology, Moorfields Eye Hospital, London (GTP)
In the UK, no Neuro-Ophthalmology fellowships exist for Neurology trainees. The first author (SJH) underwent informal training in Neuro-Ophthalmology during the course of his PhD and Neurology residency (1999–2006) under the supervision of the second author (GTP). SJH kept a log of the diagnoses of patients seen in the general Neuro-Ophthalmology and optic neuritis clinics at Moorfields Eye Hospital, London. The number of consultations for each condition was added up. Out of a total of 989 consultations: 495 were for optic nerve disorders; 118 were for other ocular causes of visual problems; 46 were for visual field abnormalities; 168 were for ocular motility and eyelid disorders; 14 were for pupillary abnormalities; 111 were for headaches, migraines and other causes of facial/ocular pain; 9 were for vascular abnormalities; and 10 were for intracranial tumours with 18 others/unclassified. The range of diagnoses covered all the diagnoses listed in the American Academy of Neurology (AAN) Neuro-Ophthalmology Fellowship Core Curriculum apart from orbital masses, orbital fractures, acute inflammatory demyelinating polyneuropathy, and botulism! In the absence of a specific fellowship the authors believe, that with appropriate clinical exposure, it is possible to train Neurologists to a sufficient standard to have a sub-speciality interest in Neuro-Ophthalmology. As there is a current drive to shorten the training period in Neurology in the UK there will be increasing time pressures, especially given the need to comply with the European Working Time Directive. The new Neurology training programmes will need to be devised to allow for the development of sub-speciality interests, either through attachments spread during training, or out-of-programme fellowships.
II.10.2 (p119)
A decade of litigation in neuro-ophthalmology in England
M.N. Ali
Royal Victoria Infirmary, Newcastle-upon-Tyne, UK
Purpose To determine the place of neuro-ophthalmology within ophthalmic litigation as a whole. To find out the commonest causes of malpractice claims in neuro-ophthalmology.
Methods All malpractice claims arising from ophthalmology, from 1995–2006, in the National Health Service (NHS) in England were analyzed. Data was obtained from the NHS Litigation Authority database. Claims were classified according to ophthalmic subspecialty, and grouped according to causes.
Results From 1995–2006, there were 848 claims in Ophthalmology. Neuro-ophthalmology accounted for 40 cases (5% of the total) and total payments of £1.1 m (10% of the total). The average payment for neuro-ophthalmology was £63,000 (compared to an average for ophthalmology of £37,100). The likelihood of a claim leading to payment was 58% for neuro-ophthalmology (compared to an average of 46% for ophthalmology as a whole). The commonest causes of claims were: failure to diagnose intracranial tumor (40%), of which half were pituitary tumors; and failure to diagnose giant cell arteritis (20%). The remaining causes included failure to diagnose intracranial aneurysm (8%), failure to diagnose nondemyelinating optic neuropathy (8%), failure to monitor idiopathic intracranial hypertension (8%), and complications of Botox (8%).
Conclusion Malpractice claims in neuro-ophthalmology in England are infrequent. They make up a small proportion of ophthalmic litigation, but claims are more likely to lead to payment, and with higher levels of payment, compared to the rest of the specialty. Failure to diagnose intracranial tumors and giant cell arteritis accounts for 60% of malpractice claims in neuro-ophthalmology.
II.10.3 (p152)
The Collaborative Curriculum: Using a digital library to create an illustrated curriculum for all facets of Neuro-Ophthalmology
Kathleen Digre, Nancy Lombardo, Larry Frohman
New Jersey Medical School on behalf of the North American Neuro-Ophthalmology Society (NANOS)
Case Report Today's neuro-ophthalmology practitioner is often both a practitioner and an educator. Like most practitioners of academic medicine, Neuro-Ophthalmology faculty must teach, conduct research, and tend to patients in the clinic. A workload of this size leaves little time for an individual to acquire educational materials or organize a course, let alone design a curriculum that will allow this knowledge to be disseminated in a standardized, meaningful fashion.
Materials and MethodsThe Neuro-ophthalmology Virtual Education Library (NOVEL) is a collaborative effort between the Eccles Health Sciences Library at the University of Utah and the North American Neuro-Ophthalmology Society (NANOS). This collaboration has produced a resource that allows busy medical practitioners and educators to access high quality, peer reviewed, neuro-ophthalmology learning assets anytime, anywhere, free of charge. These materials are the aggregate submissions of the best teaching materials from the NANOS membership. The NANOS curriculum has been developed and published by the NANOS Curriculum Committee (The Neuro-Ophthalmology Curriculum. Journal of Neuro-ophthalmology. 2006; 26: 303–315). The digital assets in NOVEL are now being organized according to the curriculum, allowing neuro-ophthalmologists around the world to find high quality teaching and learning objects to assist them in teaching medical students, residents, fellows and even patients. We will show the progress of this project in terms of the illustrated curriculum for Neuro-Ophthalmology.
II.11. Miscellaneous—Poster Presentations
II.11.1 (p2)
Successful treatment of otogenic Cavernous Sinus thrombosis with reversable blindness and hearing impairment
S. Adzura1, M.K. Khairidzan1, R. Ahmad2, V.K. Thangasamy3
1Department of Ophthalmology, Kulliyyah of Medicine International Islamic University of Malaysia, Malaysia; 2Department of Otorhinolaryngology, International Islamic University of Malaysia, Malaysia; 3Ophthalmology Department, Hospital Tengku Ampuan Afzan, Kuantan, Pahang, Malaysia
Purpose To report an interesting case of otogenic cavernous sinus thrombosis associated with reversible blindness and hearing loss secondary to masked mastoiditis
Methods Case Report
Results A twenty year old female presented with history of recurrent left sided headache for six months duration associated with progressive reduced vision of the left eye. Prior to presentation, she complained of tinnitus with gradual hearing impairment of the left ear and left sided facial numbness.
Examination revealed visual acuity of counting finger on the left eye and positive relative afferent pupillary defect. The posterior segment examination showed hyperemic and swollen of the left optic disc. Bilateral esotropia and limited abduction of both eyes noted. Otological finding showed evidence of secretory otitis media and moderate conductive deafness. The high-resolution computer tomography (HRCT) scan and magnetic resonance imaging (MRI) showed left temporal meningoencephalitis, left cavernous sinus thrombosis and left mastoidits.
Systemic antibiotic was administered to the patient and mastoid exploration and drainage was immediately performed. The patient showed tremendous recovery two weeks after the intervention. On the last follow up, the visual acuity of left eye improved to 6/6 with normal hearing on the left ear.
Conclusion This case highlighted an interesting case of otogenic cavernous sinus thrombosis associated with reversible ocular and ear complications. It illustrates that appropriate and methodological approach in dealing with cavernous sinus thrombosis is necessary in arriving to proper management and good patient recovery.
II.11.2 (p17)
Primary central nervous system lymphoma presenting as uveitis
Aylin Koc, Sumru Onal, Haluk Kazokoglu
Marmara University, School of Medicine, Department of Ophthalmology
Purpose Primary intraocular-central nervous system lymphoma (PIOL-PCNSL) is a non-Hodgkin lymphoma of B-cell type. Although it is a rare neoplasm its incidence has increased during the past two decades. The aim of this report is to investigate the clinical and histopathological features of this disease.
Methods Interventional case report.
Results A 38-year-old female presented with a history of uveitis. She started to have bilateral floaters one year ago. Initial manifestations included vitritis and subretinal infiltrations bilaterally and serous retinal detachment in her left eye. She was initially diagnosed with Vogt-Koyanagi-Harada disease due to ocular findings and a good response to intravenous methyl-prednisolone treatment. The patient had undergone diagnostic vitrectomy in her left eye, since uveitis progressed despite immunosuppressive treatment. Vitrectomy did not reveal any finding of PIOL. She was given intravitreal methotrexate (400 μg/0.1 ml) bilaterally. After documentation of lesions on cranial magnetic resonance imaging (MRI) the patient was admitted to our hospital for stereotactic brain biopsy which revealed no finding of PCNSL. Visual acuities at presentation were no light perception OD and counting finger OS. Indirect ophthalmoscopy revealed neovascularization of the disc, intravitreal hemorrhage and massive subretinal infiltration OD and a pale optic disc, empty retinal vessels and secondary pigment epithelial changes OS. Diagnostic enucleation of the right eye was recommended. However, the patient refused to undergo enucleation. Cranial MRI performed 6 months later showed a large supratentorial lesion with contrast enhancement. The patient also developed right facial nerve paralysis and right hemiplegia. The second stereotactic brain biopsy showed neoplastic cells with diffuse cytoplasmic immunopositivity for CD20. Neoplastic B-cells revealed positive immunostaining for Ki-67 with a high proliferative index of 80%. According to histomorphological and immunohistochemical findings the lesion was diagnosed as PCNSL. The patient was given whole-brain irradiation (60 Gy). The patient is still alive with regression of the cranial tumor.
Conclusions Although it is difficult to diagnose PIOL in the absence of central nervous system findings, PIOL should be suspected in any patient with uveitis unresponsive to systemic corticosteroids and/or immunosuppressive agents. Diagnostic vitrectomy, neuroradiological imaging and brain biopsy should be considered to establish the diagnosis.
II.11.3 (p51)
Virchow's triad in cerebral venous thrombosis
I. Tatiana Rosca1, N. Valentina Uscatescu2, I. Marian, Ghervan-Gogoase3, N. Carmela Negulescu Biochem4
1Neuro-Surgery Department, “Saint Pantelimon” Emergency Hospital, Bucharest, Romania (E-mail: [email protected]); 2Hematology Department, “Fundeni” Clinical Hospital, Bucharest, Romania; 3Parasitology Department, “Fundeni” Clinical Hospital, Bucharest, Romania; 4Bucharest University, Faculty of Chemistry, Romania
Aim The presence of Virchow's triad (1854) in case of infections is a sure sign for a future thrombosis, similar to that of the antiphospholipid antibodies (aPL). This has led to the hypothesis that infections themselves may be a ”trigger” for aPL in thrombus pathogenesis.
MethodsCase report. A case with headache, nausea, vomiting, papilledema and thrombosis on the 1/3 anterior superior sagittal sinus, right sigmoid sinus and right transverse sinus is presented. It is a complication of an infection with Toxoplasma gondii.
Results Toxoplasmosis, one of the most common human parasites in the world, affects predisposed people, becoming manifest at the brain, lung, heart, eyes, or liver level. Toxoplasma antigens are responsible for a hypersensitivity reaction that may result in:
retinal shining aspect and brain vasculitis
no fundoscopic changes observable in a subclinical infection,
perivascular demyelinization in the white matter of the cerebral hemispheres.
aPL formation which finally results in clotting even if not immediately
Conclusion Clinical signs and retinal and brain vessels remitted after treating the infection (Virchow's Endothelial injury) and after using low weight heparine, initially injected formula, then oral formula (for Virchow's Hypercoagulability) which finally solved stasis or turbulence (Virchow's venous flow disturbance)– the triad.
II.11.4 (p145)
Menstrual ocular migraine associated with monocular transient visual impairment and optic disc changes
Vuslat Pelitli Gürlü, Ömer Benian, Sait Erda
Trakya University Medical Faculty Department of Ophthalmology
Background To report a case with ocular migraine associated with menstrual cycle in a patiet with prolonged transient monocular visual impairment and accompanying optic disc changes.
Methods A 20-year-old female was examined in the ophthalmology department of Trakya University Medical Faculty with severe pain and monocular visual impairment in the right eye for 3 days. She was hospitalized for evaluation of optic neuritis.
Results In ophthalmic examination visual acuity was measured counting fingers in the right eye and 10/10 in the left eye. Biomicroscopic evaluation was normal. Ophthalmoscopically right optic disc was hyperemic and the disc margin was blurred. MRI, MR angiography of brain and orbit, VEP (P100 latans and amplitudes with in normal limits) and neurologic examination were normal. Her complaints improved following pulse steroid administration and right visual acuity increased to 10/10, optic disc changes regressed. But, she returned with similar complaints recurring at monthly intervals 4 times in the next four months. Laboratory findings were all normal in this period. A thorough history revealed that her eye pain appeared during her premenstrual period and losted for 3–4 days. With the beginning of her menstruation, the pain diminished but monocular visual impairment occurred. She was referred to neurology for the second time and in view of her history she was diagnosed as ocular migraine. She was treated with amitriptyline and zolmitriptan with complete cessation of her monocular visual impairment attacks as well as a reduction of eye pain in last 6 months.
Conclusions In this case report, it was seen that ocular migraine could be associated with menstrual cycle. In addition, although the optic disc changes that accompanied transient monocular visual impairment resembled anterior optic neuritis, they were not verified by VEP.
Section III: Key Notes Lectures
III.1
Brain Injury and the Eye in Children
Creig Simmons Hoyt
Professor Emeritus, Department of Ophthalmology, University of California Medical Center, San Francisco, CA
Case Report Brain injury is the leading cause of blindness in children in Europe and North America. In many countries it accounts for more cases of blindness than all ocular causes combined. Several different sites of injury can account for the visual loss. Understanding these can lead to better habilitation and rehabilitation strategies. Prevention of these injuries with improved critical care of “at-risk” infants seems promising but for the near future brain damage will continue to be the major cause of significant childhood visual loss-often in multi-handicapped children.
III.2
Restitution of visual percepts in blind patients by subretinal microelectrode arrays
Zrenner E.1, Besch D.1, Bartz-Schmidt, K.1, Gekeler, F.1, V.-P. Gabel4, Kuttenkeuler C.1, Sachs H.4, Sailer, H.3, Wilhelm B.2, Wilke R.1, Subretinal Implant Study Group
1University Eye Hospital Tuebingen, Germany; 2STZ Biomedical Optics; 3Retina Implant AG, Reutlingen; 4University Eye Hospital, Regensburg, Germany
Objective Assessment of function of subretinal implants, consisting of a chip (3 × 3 × 0.1 mm, 1540 microphotodiodes, amplifiers and electrodes of 50 × 50 micrometer, spaced 70 micrometer) and a 4 × 4 array of identical electrodes, spaced 200 micrometers, for direct stimulation (DS), chronically implanted next to the foveal rim of 2 blind RP patients.
Method Chip and DS array are positioned on a small subretinal polyimid foil powered via a subretinal transchoroidal, retroauricular transdermal line ending in a radio-controlled, battery-driven receiver-box (for surgical details see Sachs et al. ARVO 2006).
Results: The implants were well tolerated without adverse events. OCTs showed stable attachment of the retina (see Voelker et al ARVO 2006). According to study plan, the implant was removed in one patient. The other decided to keep the implant. Here, the results of chronic subretinal direct stimulation are reported.
The charge injection delivered by 16 DS electrodes was simultaneous or successive in order to present temporal or spatial patterns. Patients reported homogeneously appearing, small, yellowish or greyish phosphenes (e.g., “peanut at arm's length”) for individual electrode stimulation. They were able to differentiate spatial patterns, such as lines, angles or a bright square with round corners if all electrodes were switched on. Simultaneous stimulation of four electrodes in a line or a row was reported by patient one as “bright yellowish rod with round corners in the size of a match with three slight indentations on both sides.” The patient was able to clearly distinguish horizontal from vertical lines and to correctly describe the alignment and direction of dot movement, if three or four neighbouring electrodes were switched on sequentially at one second intervals. Electrical thresholds assessed by chronaxy measurements of each individual electrode and perceptual correlates remained relatively stable (see Wilke et al. ARVO 2006)
Conclusions Chronic subretinal stimulation via small electrodes with a distance less than 1° allows the discrimination of patterns consisting of small, bright, steadily appearing dots that can be individually discriminated as well as combined into lines and figures, reliably repeated over weeks via a well-tolerated, still intact subretinal micro-electrode array in blind RP-patients.
III.3
Visual and neurological aspects of Behçet's disease
Tülay Kansu
Hacettepe Medical School, Ankara, Turkey
Behçet's disease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers, and ocular inflammation. Ocular manifestations include anterior uveitis, posterior uveitis, macular edema, retinal vasculitis, conjunctival ulcers, optic neuropathy, papilledema, secondary glaucoma, cataract and neovascularization of iris or retina. Neurological involvement in BD may be seen as a vascular-inflammatory CNS disease with parenchymal involvement or isolated venous sinus thrombosis and intracranial hypertension.
Although the pathogenesis is largely unknown, the evidence indicates that the underlying immune events in BD are triggered by a microbial antigen and subsequently driven by genetic influences which control leucocyte behavior and the coagulation pathways. Genetic susceptibility to BD is well documented for HLA-B51 and its association with chromosome 6. The functional relevance of the vast majority of other genetic polymorphism studies are yet to be elucidated. Considerable discrepancies exist in the literature with respect to the etiology of thrombosis and the vascular pathology of BD. This review will focus on current data of visual and neurological aspects, pathogenesis and the treatment of BD.
III.4
How to Write a Medical Journal Article That is Going to Be Rejected
Jonathan D. Trobe
Authors who submit scientific papers for publication in American medical journals make the same mistakes again and again. These mistakes often lead to rejection of their work, even then it has scientific merit. What are these mistakes? (1) The title does not properly convey the main points of the article; (2) The introduction does not concisely explain why the work was undertaken; (3) The methods are not explained clearly; (4) The results are not presented in order of importance; the tables and figures are poorly executed; (5) The discussion does not highlight the important results, explain their significance, describe the limitations of the study, or present suggestions for future directions of study; (6) The references are poorly chosen and improperly formatted. In this seminar, the leaders will present examples of mistakes made by authors. Members of the audience will have the opportunity to discover the mistakes and discover how they can be corrected.
Section IV: Symposium Presentations
IV.1. Pediatric Neuro-Ophthalmology Potpourri—Symposium Presentations
IV.1.1
The “overlooking” phenomenon of children with neuronal ceroid lipofuscinosis
J.R. M. Cruysberg1, M.A.A.P. Willemsen2, N.G. van Moll-Ramirez3, M.M. van Genderen4
From the Institutes of 1Ophthalmology and 2Child Neurology, Radboud University Nijmegen Medical Centre; 3Institute Sensis, Grave; 4Institute Bartimeus, Zeist, The Netherlands
Purpose To report permanent upgaze as a manifestation of neuronal ceroid lipofuscinosis.
Design Observational case series.
Methods A total of 25 Patients with neuronal ceroid lipofuscinosis had ocular and neurological examinations.
Results Clinical and biochemical studies confirmed the diagnosis of neuronal ceroid lipofuscinosis in all cases. With clinical and non-clinical observations nearly half of the patients showed permanent upgaze. The “overlooking” phenomenon was not due to a relative preservation of the upper retina.
Conclusion It is concluded that permanent upgaze is clinical sign of neuronal ceroid lipofuscinosis. However, the phenomenon is not explainable by the ocular abnormalities but probably has its origin in brain stem pathology.
IV.1.2
Clinical Features and treatment of pediatric Graves' ophthalmopathy
J.A. Garrity, V.D. Durairaj, G.B. Bartley
Graves' ophthalmopathy (GO) is a presumed autoimmune condition with the pre-adipocyte fibroblast as a putative target cell. Site specific differences in fibroblastic activity have been shown although there is no satisfactory explanation. Similarly, there are differences in clinical expression in the pediatric (∼18 yrs) and adult populations, generally being less severe in the former.
From 1985–1999 we identified 1719 patients with pediatric Graves' disease. Of these, 1662 patients gave consent for their records to be reviewed and we found 57 patients with formal eye examinations. Firm diagnostic criteria for GO were met in 35 (27 females (77%), 8 males (23%)). Hyperthyroidism was present in 31 patients (89%), hypothyroidism in 1 (3%), and euthyroidsim in 3 (8%).∼ Hyperthyroidism was treated with radioactive iodine in 6, surgery in 9, antithyroid medication in 10 or combinations in 6.∼ Mean age at diagnosis of thyroid dysfunction was 13.1 years (range 3–18) while the mean age at diagnosis of ophthalmopathy was 13.9 years (range birth–18 years).∼ The most frequent sign was eyelid retraction (29 patients, 83%).∼ There was no optic neuropathy.∼ Thirty-one patients (87%) required no therapy.∼ No patient received steroid or radiation therapy.∼ One patent (3%) received eyelid surgery and∼ three patients (9%) received transantral orbital decompression for excess proptosis. Two patients (10%) had family members with GO while 12 (60%) had a family history of thyroid dysfunction.
The clinical manifestations of GO are less in the pediatric population and surgery is infrequently required.
IV.1.3
Visual field testing in pediatric neuroophthalmology
Lene Martin
Purpose To describe computerized perimetric methods suitable for examining children and present results from normal subjects and pathological cases, compared to other examination techniques.
Methods Several new perimetric techniques have been developed during the last decades. The examination procedure has been shortened and a variety of different stimuli and setup configurations are now available. Yet manual perimetry using the Goldmann perimeter is still the most common method when examining children. The experience from using four computerized perimetric techniques, the Humphrey field analyzer, high-pass resolution perimetry, frequency doubling technology, Rarebit perimetry, and the Rarebit fovea test, in children will be discussed, and compared to results obtained with other examination techniques.
Results The optimum method for a particular child is dependent of age, maturity, type of disorder and the examiner's experience. Findings from healthy children and children with neurological disorders, examined with the different techniques, will be presented. Conclusions: In adults, computerized static perimetry has become “gold standard” in most diagnosis. Children can perform computerized perimetry from the age of 7 to 10, depending on method used.
IV.1.4
Initial presentation of Humphrey field defect in Leber hereditary optic neuropathy
Masato Wakakura
Inouye Eye Hospital, Tokyo, Japan
Purpose To determine initial presentation of visual field defect in Leber's hereditary optic neuropathy (LHON).
Design Prospective, observational case series.
Participants Four eyes of four LHON patients with mitochondrial (mt) DNA 11778 mutation which has not yet visual loss (two eyes) or has only minimal visual loss (two eyes).The patients were all males and ranged in age from 27 to 56.
Methods To detect early visual loss of LHON, we took attention to interocular interval of the onset. Humphrey field analysis (program 30-2) was regularly performed for the eye with no or less visual loss immediately following unilateral visual loss was evident in the patients carrying the mtDNA mutation.
Results The central-most upper temporal test point was initial site of perimetric sensitivity loss in all four eyes examined. Manifestation of the visual field defect was followed by visual loss.
Conclusions The central-most upper temporal visual field appears to be the earliest impaired site of LHON. This suggests that specific location of retinal ganglion cells is susceptible in early stage of the disease.
IV.2. Approach to genetic-related diseases in Neuro-Ophthalmology—Symposium Presentations
IV.2.1
Practical issues on doing genetic studies in neuro-ophthalmology
Nurten Akarsu
Departments of Pediatrics and Medical Genetics, Hacettepe University, Sihhiye, 06100, Ankara, Turkey
To initiate a genetic study for a clinician in a daily routine is a confusing process although rapid advances in genetic technologies provide opportunities for differential diagnosis of disorders, prenatal and/or presymptomatic diagnosis of patients. Methodology decision, laboratory selection, interpretation of complicated genetic terminology are some reasons behind the confusion. This symposium aims to discuss frequently asked questions with regard to how a genetic study can be design in neuro-ophthalmology. The initiative step to design a genetic study in neuro-ophthalmology is not different from other clinical disciplines. Obtaining available information about the genetic status of the disorder is the first and necessery step. Online version of Mendelian Inheritance in Man developed by McKusick,VA (http://www.ncbi.nlm.nih.gov/ select OMIM) is a friendly, comprehensive and integrative database providing up-to-date clinical and genetic information not only to help researchers to plan their own studies but also lead them to select proper diagnostic strategies. Using this database, effectively addressing six consecutive questions about the genetic status of the disorder might be helpful to plan a study. These questions are: (1) Is the contributing factor purely genetics or not? Genetic epidemiology methodologies are frequently used in this aim; (2) Is it herritable and, if it is, what is the most likely mode of inheritance? Estimation of a likely mode of inheritance of a given disorder is called genetic modeling of the disease. Proper modeling of the disease is critical for further localization identification studies; (3) Is a chromosomal localization of the disorder known? Genetic mapping techniques are used to identify likely localizations of the disorders. Mapping process is an integrated approach combining medicine, molecular biology, statistics and computer sciences. After this step, genetic diagnosis and risk estimation would be available solely for the families proven to be linked to those regions; (4) Is a gene causing the disorder known? Physical mapping and mutation identification studies aim to find the causative gene in the critical interval obtained from the previous step. Genetic diagnosis, prenatal and/or presymptomatic diagnosis, as well as population screen studies would be conducted after this step; (5) What is known about the function of the causative gene? Functional studies including animal models give important information about behavior of the genes. This is the leading step to bring innovative ideas about gene therapy. (6) Could it be possible to repair misfunction of the gene? Gene therapy titles are the final and dreaming step of genetic studies.
Most of these steps have been completed successfully in neuro-ophthalmology Substantial genetic contribution is responsible from many neuro-ophthalmic disorders. Heritable component is evident and likely mode of inheritances are mostly compatible with either a single gene or a mitochondrial inheritance. Current genetic researches highly focus on mapping and gene identification studies. Large families are extremely valuable for mapping aim. Evaluating of functional importance of identified genes are attractive research subjects in neuro-ophthalmology. In addition to sophisticated technologies, establishing genotype-phenotype correlations are also a good model to understand various functions of the genes. Limited research based gene therapy studies to develop, characterize and improve viral vectors in neuro-ophthalmic disorders are also promising research areas in this discipline. Genetic diagnosis can be requested from diagnostic laboratories for the disorders if their genes are known. A detailed genotype-phenotype correlation results are important for prenatal diagnosis and risk estimation.
IV.2.2
Genetics of Nystagmus
Irene Gottlob
University of Leicester, Leicester, UK
Background While cases of nystagmus alone are frequently sporadic, kindreds in which nystagmus segregated as an autosomal dominant, autosomal recessive, or X-linked trait have been reported. Of these, X-linked pedigrees are the most frequent. By linkage analysis, the major X-linked locus for nystagmus (NYS1) was localised to chromosome Xq26-q27.
Methods Clinical examination, visual evoked potential and electroretinograms were obtained from 26 families with idiopathic congenital nystagmus. Linkage analysis and DNA sequence analysis was performed.
Results A novel gene named FRMD7 (FERM domain containing 7) at Xq26.2 has been found. We have identified 22 mutations in FRMD7 in 26 families with X-linked idiopathic nystagmus suggesting that mutations in FRMD7 are a common cause of familial nystagmus. Screening of 42 singletons cases of idiopathic congenital nystagmus (28 males and 14 females) yielded 3 mutations (7%). Mutations in the FRMD7 gene encode a previously unidentified member of the protein 4.1 superfamily. We have also shown using in situ hybridization that FRMD7 is expressed in early human embryos at ∼56 d post-ovulation where there is expression in the ventricular layer of the forebrain, midbrain, cerebellar primordium, spinal cord and the developing neural retina.
Conclusion We found the first gene for idiopathic nystagmus. The function of this novel gene (FRMD7) in brain is unknown. However it has a close amino acid sequence homology with FARP1 and FARP2 which suggest a possible function in membrane signaling, cytoskeletal organization and modulation in the length and degree of branching of neurites.
IV.2.3
Genetics of the congenital cranial dysinnervation disorders
Elizabeth C Engle
Children's Hospital Boston, MA, USA
Many common and complex forms of strabismus are heritable and, hence, genetic studies can provide insight into their molecular etiologies. In the last decade, we have focused our research studies on understanding the genetic basis of a series of complex strabismus syndromes, including various forms of Duane syndrome and congenital fibrosis of the extraocular muscles (CFEOM). The genetic analysis of these disorders has led to the identification of a series of disparate strabismus genes, including PHOX2A, SALL4, KIF21A, ROBO3, and HOXA1. Each gene plays an essential role in the normal development and/or connectivity of cranial motoneurons. Mutations in PHOX2A and KIF21A primarily effect development of the midbrain ocular motor axis, resulting in various forms of CFEOM. Mutations in HOXA1, ROBO3, and likely SALL4 primarily effect development of the pontine abducens motor axis, resulting in various forms of Duane syndrome or horizontal gaze palsy. These gene defects lead to errors at different locations along thedeveloping neuroaxis, including errors in axonal targeting onto the motoneurons, in motoneuron development, and in axonal targeting onto the extraocular muscles. The combination of these genetic findings with accumulating data from additional clinical, neuropathologic, neuroimaging, and molecular studies has led us to rename these syndromes the “congenital cranial dysinnervation disorders” or CCDDs. We anticipate that continued studies of the role of each CCDD gene in neurodevelopment will provide additional knowledge about the pathogenesis of oculomotor disease and the development of the ocular motor axis. In addition, we are now able to apply similar genetic approaches to define the genetic influences that contribute to the common forms of strabismus.
IV.3. Iatrogenic Neuro-Ophthalmologic Disorders—Symposium Presentations
IV.3.1
Toxic optic neuropathies: methanol, ethambutol and isoniazid
Shlomo Dotan
Hadassah∼University Medical Center Jerusalem, Israel
The combination of acute onset, life-threatening systemic symptoms which develop after a 12–24 h latency period and severe visual loss, are typical for methanol poisoning. Patients may regain vision, usually within a week but occasionally later. Formic acid is considered to be the main metabolite in methanol intoxication, which by inhibiting the cytochrome oxidase complex of the mitochondrial respiratory chain may cause cell death. Medical treatment must be instituted promptly. The potential for visual impairment of ethambutol was recognized soon after these antituberculoses agent was introduced in the 1961. The occurrence of ocular toxicity is dose-and-duration related. Loss of vision begins insidiously, is bilaterally asymmetric and not necessarily reversible when ethambutol is discontinued. Animal models demonstrate that toxicity begins in the optic chiasm, while cell culture suggests that the damage is mediated via increased calcium influx and decreased levels of copper in the optic nerve fiber mitochondria.
Patients are often on combined antimycobacterial therapy with isoniazid, which may have additive adverse effect. Toxic reactions with the use of this drug occur frequently with larger doses than 3 mg/kg/day. Isoniazid associated optic neuropathy is less frequent and less severe than that related to ethambutol and is usually reversible.
IV.3.2
Drug-induced intracranial hypertension and drug-related myasthenia gravis
Dan Milea
Glostrup University Hospital, Copenhagen, Denmark
Several medications have been historically associated with intracranial hypertension, although not confirmed by controlled studies. These include Vitamin A and other retinoids, Tetracyclines, Fluoroquinolones, Indomethacin, Amiodarone, Lithium, Steroids withdrawal, etc. Only few of these cases are well documented, or fit into the WHO criteria of drug responsibility, i.e., the challenge-rechallenge concept. In individual cases, such treatments should be discontinued if possible, although their relationship with intracranial hypertension might not be clear.
A myasthenic syndrome can be the result of drug interference with the function of the neuromuscular junction (d-Penicillamine, aminoglycosides, anesthetics, statins, and so on), at the preor postsynaptic level. This can occur in patients with previously unknown myasthenia gravis, or can represent aggravation by concurrent drug therapy. The presence of myasthenia gravis does not appear to be an absolute contraindication for the use of these agents, if a careful monitoring of the dose, renal function and neuromuscular status is performed.
IV.3.3
Function testing in toxic neuro-ophthalmologic disorders
E. Zrenner
University Eye Hospital Tuebingen, Germany
Retinal degeneration is marked by progressive loss of visual functions. In some cases the loss of function can be subjectively noticed by the patient as loss of visual acuity, color vision disturbances or night blindness. In other cases, however, it could pass unnoticed, for narrowing of visual field or minor losses of light sensitivity are sometimes recognized by the patient only in advanced stages. Many functional deficiencies are detectable only by objective examinations of fundus morphology (pigments) or electrophysiological function testing (electroretinogram, electro-oculogram, visually evoked cortical potentials).
Additionally, retinal degeneration may be limited to certain sets of retinal neurons such as cones and/or rods in the outer retinal layer, bipolar cell or Müller-cell function (middle retina) or ganglion cells in the inner retina. Moreover, drugs may primarily affect the retinal periphery (dominated by rods with cones still being present) or the retinal center (e.g., in the region of the macula), where cones dominate, with rods having their maximum density at 20° eccentricity. Moreover, besides direct degeneration of retinal neurons, indirect trigger mechanisms do exist such as loss of pigment epithelial function, loss of Müller cell function or problems with vascular perfusion.
Therefore the design of an appropriate test battery for discovery of retinal degeneration in toxicological studies needs to be based on pre-clinical and clinical studies. For translation into human studies the following instruments are recommended: Electroretinography depending on the state of light adaptation can discern between the rod and cone system on the one hand and the outer and middle retina on the other. Oscillatory potentials (OPs) can provide evidence of inner retina disturbances. While electroretinography discerns on the basis of stratification of the retina rather than localization, perimetry allows us to assess peripheral as well as central retinal locations. Kinetic perimetry is best suited for the peripheral retina, while static perimetry is more suited for central retina.
The function of the fovea, which is very important for high spatial resolution and chromatic information of stimuli due to the different kinds of cones, shall be assessed by color vision tests and visual acuity tests. This functional assessment is complemented by the investigation of retinal morphology with special attention to pigment alterations, deposits, and changes in the vascular system, separately for the central and peripheral retina. Additionally in several toxic disorders, especially those that affect cones, pale optic nerve heads are observed, which can be assessed by ophthalmoscopy as well. Using such a battery of morphological and functional tests would certainly allow us to monitor the development of retinal degeneration at a relatively early stage. Using this target-oriented approach it is highly unlikely that the examiner would overlook a drug induced retinal degeneration within a 3-year time frame.
IV.3.4
Risks versus benefits: putting sildenafil, amiodarone and vigabatrine in the dock.
Fion Bremner
National Hospital for Neurology & Neurosurgery, London UK
The visual side-effects from three drugs in current use are described.
Although initially met with skepticism, it now seems that up to 40% of patients taking vigabatrin develop concentric bilateral irreversible visual field loss. ERG evidence indicates toxicity at the level of the inner retina where the drug preferentially accumulates. Patients are often unaware of the field loss and reluctant to stop the medication, but continued exposure does not seem to cause progression of the visual field defects. Other irreversible inhibitors of GABA-T do not share this side-effect and may eventually replace this drug.
The question of ocular toxicity from sildenafil remains controversial. As a weak inhibitor of PDE6 it interferes with retinal biochemistry and can affect the ERG and psychophysical test performance in healthy subjects; a small number of patients do complain of transient dyschromatopsia after exposure to sildenafil, probably as a result of this mechanism. More controversial is the suggestion that permanent visual loss may be caused by sildenafil-triggered NAION, either from local changes in disc perfusion or systemic hypotension. Because of overlap in the risk factors for NAION and impotence it remains unclear whether this association is cause-and-effect or coincidence.
Amiodarone undoubtedly causes visually insignificant corneal verticillata. However it has also been implicated as a cause of optic neuropathy. The clinical picture varies from acute-onset unilateral irreversible damage indistinguishable from NAION, with which these cardiac dysrhythmia patients share many risk factors, and insidious progressive bilateral disc edema with reversible visual loss, possibly a result of primary lipidosis caused by this cationic amphiphilic drug.
With all three drugs the same questions emerge: do they damage vision, how should we screen for this and what do we do if toxicity is found or deemed likely?
IV.3.5
Neuro-ophthalmic complications of cyclosporine and methotrexate-craniospinal irradiation
Jonathan D. Trobe
Professor of Ophthalmology and Neurology, University of Michigan, Ann Arbor, Michigan
Cyclosporine is a cyclophilin in wide use for the prevention of graft rejection and for the treatment of autoimmune disorders. It is implicated in cerebral blindness by causing a leukoencephalopathy affecting posterior hemispheric white matter. Whether cyclosporine-induced leukoencephalopathy is caused by marked blood pressure elevation or direct toxicity is unclear. Prompt removal of the agent is critical in preventing permanent damage. Cyclosporine is also associated with optic neuropathy manifested by optic disc edema. Whether this is secondary to increased intracranial pressure or vasculopathy of the optic nerves is unclear. Methotrexate combined with craniospinal irradiation, particularly for childhood leukemia, has a high propensity to cause a severe leukoencephalopathy manifesting as cognitive decline and cerebral blindness. The timing and dose of radiation appear to be critical in causing this irreversible and devastating but preventable complication.
IV.4. Diagnostic and Therapeutic Challenges in and Around the Sella Turcica—Symposium Presentations
IV.4.1
Incongruity: When the scan does not match the visual field in Pituitary disorders
Misha Pless
Neurology Department Massachusetts, General Hospital Associate Professor, Harvard Medical School, Neuro-ophthalmology, Multiple Sclerosis, General Neurology, MA, USA
When evaluating pathology resulting from intrinsic or extrinsic compression of the chiasm the clinician has a tendency to look for certain patterns of visual field loss or field defects which respect the vertical meridian or have classic bitemporal loss pattern. However, in clinical practice a clinician will occasionally encounter lesions which by virtue of their chronicity or histological type are associated with patterns of visual field loss whose characteristics do not match that which is anticipated. This ostensible incongruity is typically explained away in terms of the acknowledged relationship between the tumor and the chiasm or the histological type in cases of intrinsic mass lesions. For instance very chronic, slow growing tumors can reach large size resulting in visual field defect which are barely apparent. However a massive tumor with adjacent brain tissue shift will characteristically be expected to result in some chiasmal damage and thus visual field defects–-the problem is when it does not. The paper will consist of a series of case studies retrospectively culled from a busy academic neuro-ophthalmology practice which demonstrate the point of incongruity. By exploring patterns of clinical expectation pertaining to lesion type/size vis-à-vis visual field defect, the cases studies will illustrate that given certain conditions “everything is possible” in chiasmal pathology and incongruity should not be deemed with incredulity. More importantly the cases will serve as a cautionary tale to the practicing ophthalmologist who fails to consider chiasmal compression/pathology when classic field loss patterns are not present.
IV.4.2
Pituitary apoplexy
Robert McFadzean
Institute of Neurological Sciences, Southern General Hospital, Govan Road, Glasgow G51 4TF, United Kingdom
Particular attention will be focussed on the historical background, terminology, epidemiology, pathophysiology, and predisposing factors. Clinical features will be considered in terms of general, visual, neurological and endocrine presentations and the differential diagnosis discussed. Imaging investigation will be illustrated, while the management dilemma will be discussed against the background of the known natural history. Finally, outcomes and the ultimate prognosis in pituitary apoplexy will be addressed.
IV.4.3
Traumatism and other rare lesions of the optic chiasm
François-Xavier Borruat
Hôpital Ophtalmique Jules Gonin, Luasanne, Switzerland
Chiasmal syndromes result most frequently from comprehensive lesions. In this presentation, the clinical profile and diagnostic features of rarer etiologies, such as traumatism, inflammation, and ischemia, will be discussed.
IV.4.4
Surgical approach to sellar region tumors
Murad Bavbek M.D., Neurosurgeon
Diskapi Hospital, Ankara, Turkey
The choice of the surgical approach to the sellar region depends on the type and the extent of the tumor and the surgical anatomy of the sellar region, which is preoperatively investigated by the neuroradiological studies. In addition to the sellar radiography, CT (computed tomography), MR (magnetic resonance) image, cerebral angiography and paranasal sinus tomography (PST) are helpful. PST gives information about the septum position, symmetry and the irregularities of the sphenoid sinus. Conchal abnormalities especially concha bullosa may also be seen preoperatively with the PST. In these patients we first prefer to make endoscopic resection of the concha and then endoscopic resection of the tumor. The Willis aneurysms should also be kept in mind preoperatively which may resemble the appearance of a pituitary neoplasm destructing the sella.
In transcranial sella surgery; after opening the sylvian, chiasmatic and the carotid cisterns, anterior and the middle fossa exposure is carried out in a standard fashion. Lesser sphenoid ring is an anatomical landmark for the anterior fossa exposure with frontal lobe retraction. After visualizing bilateral optic nerves, the chiasm and bilateral carotid arteries, we debulk the tumor, after coagulating and opening its capsule. The pituitary stalk should be visualized while dissecting the tumor which avoids injury of the hypothalamus. Individual vessels of the hypothalamic region should be cautiously coagulated if needed. We believe that the tumor portion which strongly adharent to the hypothalamus should not be extracted and should be left.
If the tumor has parasellar extension, sphenoid wing can be exposed through the clinoid process. Tentorial notch is an another landmark of the tentorial incisura posterior to the internal carotid artery. If the tumor is extending the cavernous sinus, we believe that after subtotal excision of the tumor and decompression of the surrounding neurovascular structures gamma-knife may be used.
Operative exposure of the sellar region is mostly used for the treatment of pituitary tumors, craniopharyngiomas, tuberculum sella meningiomas, optic nerve gliomas.
IV.5. Functional Brain Imaging and Neuro-Ophthalmology—Symposium Presentations
IV.5.1
Functional imaging of cognitive processes in eye movement control
Christopher Kennard
Imperial College London, UK
Considerable understanding of the cognitive processing and neural networks controlling the generation of saccadic eye movements has been gained in recent years. Several cortical areas in the frontal lobes have been implicated in the control of saccades, including the frontal eye field (FEF) and the dorsolateral prefrontal cortex (DLPFC), the supplementary eye field (SEF), pre-supplementary motor area (pre-SMA) and the anterior cingulate cortex (ACC). This network of saccade-related dorsolateral and medial frontal cortical areas show a complex pattern of connections and undertake a number of different cognitive processes responsible for a variety of complex behavioral tasks. These include self-initiated (free-choice) versus instructed saccade generation, inhibition of saccades, conflict resolution and error monitoring, evaluating self actions and implementing control, working memory and reward.
A number of different approaches have been used to determine the specific cognitive functions of these saccadic areas, the main one in recent years having been functional magnetic resonance imaging (fMRI). An overview of the results of these studies will be given, particularly relating to the volitional control of saccades. The importance of combining data from fMRI with behavioural studies in patients with focal lesions will be emphasised, particulalrly in relation to the analysis of medial frontal lobe involvement in the generation of volitional saccades.
IV.5.2
Is fMRI a useful investigative tools for clinical vision scientists?
Anthony Morland
UK
For over a decade investigators have been employing fMRI to study the properties and organization of the human visual cortex. Although this research has revealed much concerning the properties of the normal human visual system, there are very few studies that use fMRI to investigate visual function from a clinical standpoint. In this paper the potential for fMRI to provide a useful source of information about visual deficits will be explored. It will be argued that, in principle, fMRI can provide a means of characterizing some visual deficits and in some cases this can be done with greater sensitivity than with conventional objective measures of vision.
IV.6. Current Concepts in Idiopathic Intracranial Hypertension–Symposium Presentations
IV.6.1
Intracranial Hypertension in Children
Deborah I. Friedman, MD, FAAN
University of Rochester School of Medicine, Rochester, New York
The pseudotumor cerebri syndrome varies somewhat between young children and adolescents. Intracranial hypertension (IH) in pre-pubertal children often arises from a secondary cause such as infection, otitis media or an exogenous agent (e.g., tetracyclines, human growth hormone). IH in young children may present with atypical manifestations of somnolence, a stiff neck, apathy, ataxia, irritability or torticollis. Asymptomatic papilledema may be discovered on a routine eye exam. Boys and girls are affected equally and obesity is not a significant risk factor. After puberty, the symptoms and demographics are similar to adults, primarily affecting overweight girls. Secondary causes, such as implantable contraception, retinoids, tetracyclines, and venous sinus thrombosis must be sought. The diagnosis and treatment of IH in children is similar to that in adults, although teenage girls may be at a higher risk for permanent visual loss.
IV.6.2
Idiopathic intracranial hypertension: difficulties in diagnosis
Gordon T. Plant
The National Hospital for Neurology and Neurosurgery
The origins of the terms “Idiopathic Intracranial Hypertension” (IIH)and “pseudotumor cerebri” (PTC) will be reviewed. In the author's view IIH should be reserved for the specific phenotype of intracranial hypertension associated with an elevated Body Mass Index (BMI). Even in such cases an alternative etiology must be excluded as far as possible, in particular the obstructive sleep apnoea syndrome, which is also associated with obesity. However, in non-obese patients it is better to use the term PTC which does not commit to an etiology and it should be assumed that a cause can be found. Case histories will be shown which illustrate the difficulties which can be experienced in the diagnosis of the many pathological processes which can lead to intracranial venous hypertension and the PTC syndrome.
IV.6.3
Interventions and treatments for IIH
Kathleen Digre
Moran Eye Center, University of Utah, Salt Lake City, UT, USA
Idiopathic intracranial hypertension is a condition in young women of child bearing years. It is characterized by symptoms of headaches and transient visual obscuration and signs of papilledema and rarely a sixth nerve palsy. The major threat of the condition is loss of vision and visual loss occurs in about one-third of patients. Quality of Life and has also been found to be reduced and depression is more frequent in the condition. Interventions and treatments of IIH target (1) resolving the condition (2) preventing visual loss, (3) treating headaches (4) improving quality of life. The only intervention that has been shown to cause resolution of the condition has been weight loss in those with obesity. Medical therapies to prevent visual loss have mainly been diuretics aimed at lowering CSF pressure. Other medical therapies have been proposed and there are not controlled trials in IIH showing the superiority of any one medical or surgical therapy. Surgical therapies have traditionally been used when medical therapy fails. Frequent lumbar punctures are helpful at temporizing pressures while more definitive therapies such as optic nerve sheath fenestration and ventricular-peritoneal shunting is being planned. Each of the therapies has advantages and disadvantages. Placing a stent in the venous sinuses has been proposed, but caution has been recommended in using such a procedure since long-term consequences are not known. Treatment of the headache associated with IIH vexes many clinicians. Studies have shown that treatment with a diuretic in conjunction with a headache preventative (e.g., anti-convulsant, tricyclic, calcium channel blocker) seems to provide the best relief. The ultimate goal in all of our interventions and treatments is improving quality of life for individuals with the condition.
IV.7. Ocular Motor Disorders—Current Issues—Symposium Presentations
IV.7.1
Drugs and eye muscles: The statins
Aki Kawasaki
Switzerland
The statins are a family of HMG-CoA reductase inhibitors which are widely popular in the treatment of hyperlipidemia. Recently, a myasthenic syndrome has been described in patients taking a statin medication. The clinical features of this unusual side effect to statin medication are discussed.
IV.7.2
Myasthenia Gravis and anti MuSK antibodies.
Desmond Kidd
Consultant Neurologist, the Royal Free Hospital and University College, London, UK
Around 80% of people with generalised Myasthenia Gravis and only 50% of those with ocular myasthenia have detectable antibodies against acetyl choline receptors. Six years ago it was found that 20-70% of patients with seronegative myasthenia have antibodies against the tyrosine receptor kinase MuSK. These patients have a different clinical phenotype to those with acetyl choline receptor antibodies in that there is a more severe disease with bulbar and diaphragmatic involvement early on. Muscle wasting is seen in long standing cases. The ocular motor muscles are rarely affected and an ocular pattern is rarely seen. Electrophysiological investigations reveal a low prevalence of abnormalities of neuromuscular transmission, a myopathic EMG being more common. There are no thymic germinal centres on histology. There is often a poor response to acetyl cholinesterase inhibitors, but most patients respond well to steroids and immunosuppression. Resistant cases have been treated with cyclophosphamide and rituximab.
IV.7.3
New developments in pharmacological therapy of nystagmus
Irene Gottlob
University of Leicester, Leicester, UK
Background: Several drugs have been used to treat acquired nystagmus, however pharmacological treatment has so far not been used in congenital nystagmus.
Method: Patients with congenital nystagmus (idiopathic (IIN) or associated with ocular diseases (SN) such as albinism, achromatopsia, congenital cataracts or optic nerve hypoplasia) were treated clinically and in a randomized study with gabapentin (up to 2400 mg), memantine (up to 40 mg) and placebo.
Results: Gabapentin and memantine improved LogMar visual acuity significantly in IIN. Both drugs reduced nystagmus intensity and foveation (calculated with the expanded acuity function, NAFX) in IIN and SN.
Conclusion: Pharmacological agents such as gabapentin and memantine can improve visual acuity and reduce nystagmus intensity and foveation in congenital nystagmus
IV.7.4
Older patients with isolated cranial nerve palsies–-Should they be imaged straightaway?
Paul Riordan-Eva
London, UK
A very large proportion of acute acquired spontaneous isolated third, fourth or sixth nerve palsies in older patients are due to microvascular disease or other causes not requiring neuro-imaging. However, the possibility of intracranial aneurysm or tumour may necessitate emergency or early investigation. Guidelines on which patients should undergo neuro-imaging will be reviewed.