P161 THE LIVED EXPERIENCE OF FATIGUE IN MND: A QUALITATIVE STUDY FROM THE PATIENTS' PERSPECTIVE
GIBBONS C1,2, THORNTON E2, YOUNG C1,2
1Walton Centre for Neurology and Neurosurgery, Liverpool, United Kingdom, 2University of Liverpool, Liverpool, United Kingdom
E-mail address for correspondence: [email protected]
Keywords: fatigue, qualitative analysis
Objectives: The evaluation of patients' perceptions of fatigue in MND.
Methods: Fourteen patients with MND under the care of the Walton Centre for Neurology and Neurosurgery (Liverpool, U.K.) took part in semi-structured interviews. Transcripts were triangulated between the interviewer, a consultant neurologist and a health psychologist and analysed using an Interpretive Phenomenological Analysis (IPA) framework.
Results: IPA analysis revealed 7 main themes:- Physical, Mental, Emotional, Social, Progression, Activity Restriction and Rest. The experience of fatigue was conceptualised as physical, affective and to a lesser extent, cognitive. Physically, fatigue was experienced as a combination of motor failure following minimal muscle use and ‘whole-body' tiredness unrelated to exertion or rest. Some patients reported difficulties concentrating, especially whilst doing tasks that involved muscle use (ie holding a book). Initiation of movement, stress, anxiety and breathing difficulties were associated with increased fatigue. Fatigue was perceived to be progressive and unremitting over the course of the illness. Increased fatigue whilst carrying out activities of daily living (ADLs) led to frustration as well as adaptive changes in social motivation, periodic rest and pre-planning of activities.
Discussion: Fatigue appears to be experienced in different ways, both as an inability to sustain motor function and as a pervasive tiredness. Fatigue had an impact on patients' day-to-day lives and the way in which they planned ADLs and social activities.
Conclusion: The results of this study support a multi-dimensional model of fatigue in MND.
P162 PREDICTING QUALITY OF LIFE OF PEOPLE WITH MND: EXPLORING THE ROLE OF PSYCHOLOGICAL DISTRESS AND FUNCTIONAL STATUS
GIBBONS C1,2, THORNTON E2, TENNANT A3, EALING J4, MITCHELL D5, SHAW P6, TALBOT K7, YOUNG C1,2
1Walton Centre for Neurology and Neurosurgery, Liverpool, United Kingdom, 2University of Liverpool, Liverpool, United Kingdom, 3University of Leeds, Leeds, United Kingdom, 4Hope Hospital, Manchester, United Kingdom, 5Royal Preston Hospital, Preston, United Kingdom, 6University of Sheffield, Sheffield, United Kingdom, 7University of Oxford, Oxford, United Kingdom
E-mail address for correspondence: [email protected]
Keywords: quality of life, depression, functional status
Objectives: To evaluate the predictive power of depression, anxiety and functional status for quality of life (QoL) in MND.
Methods: Eighty six patients from five regional MND care centres in the UK completed questionnaires either at home or during a routine clinic appointment. Anxiety and depression were measured by using the Hospital Anxiety and Depression Scale (HADS), functional status was measured using the ALSFRS-R, and patient quality of life was measured using the World Health Organization's WHOQOL-BREF measure.
Results: Hierarchical multiple regression was used to assess the ability of measures of psychological distress (anxiety and depression) and functional status to predict patient quality of life. Predictor variables were entered into the model based on Pearson's correlation values with the dependant variable. The variance explained by the model as a whole was 42.9%, F (3,84) = 22.00 P<0.001. The largest single predictor of quality of life in MND was depression (beta=0.540); neither anxiety (t(3) = 1.67 P=0.10) nor functional status (t(3)=−1.73, P =0.09) made a significant unique contribution to the model. Depression was also the only significant predictor of item 1 of the WHOLQOL instrument “How would you rate your quality of life?”
Discussion: The results show depression to be an important variable for predicting QoL in our model. This is congruent with previous research findings in MND that depict depression as correlated to health-related QoL and suggest that physical function is not directly related to patient QoL in MND.
P163 THE CHALLENGES OF CARE-GIVING: CONTRIBUTIONS OF COGNITIVE FUNCTION, BEHAVIOUR, AND SOCIAL COGNITION DIFFICULTIES IN MND
FISHER F
Calvary Health Care Bethlehem, Melbourne, Australia and Victoria University, Melbourne, Australia
E-mail address for correspondence: [email protected]
Keywords: care-giving, cognition, behaviour
Background: There is a growing conceptualisation of Motor Neurone Disease (MND) as a disorder affecting multiple systems, with dysfunction in anterior cortical regions associated with mild to severe cognitive impairment and behavioural dysfunction in up to 50% of patients. The overlap of MND with Frontal Temporal Dementia (FTD) in a small percentage of cases is also now well established. The psychological impact of providing care for someone with MND who also experience changes in behaviour and cognition is not well documented. While published FTD literature reports increased psychopathology and burden amongst caregivers, it is unclear whether caregivers of persons with MND (PwMND) experience similar levels of psychological distress when cognitive and behavioural changes are present.
Objectives: The current research aimed to examine the relationship between cognitive impairment, behaviour change, social cognition, and carer burden and psychological wellbeing, in a group of PwMND and their caregivers.
Methods: Thirty-nine participants with formally diagnosed MND (46% females and 54% males; mean age 66.03 years) and their nominated caregiver (77% female, 33% male; mean age 57.20 years) were recruited through specialist MND clinics. Demographic information (eg age, months since symptom onset and MND phenotype) was collected for all participants. Level of functioning (ALSFRS-R) was rated by the treating neurologist. The study assessed cognition (Addenbrooke's Cognitive Examination - Revised), behaviour (Cambridge Behavioural Inventory, and Frontal Behavioural Inventory), and social cognition (Reading the Mind in the Eyes Test).
Results: Participants were grouped according to level of cognitive impairment. Participants with bulbar onset MND were not over-represented in the cognitively impaired group (P>0.05).
Those who were cognitively impaired (36%) were also significantly worse in their recognition of emotional expressions (ie reduced social cognition) than those with preserved cognitive functioning (P<0.001). Cognitively impaired participants were also rated as being more adynamic (ie loss of interest and motivation) by their caregivers (P<0.05). Observed group differences in social cognition and adynamia occurred independently of physical disability and functional impairment as measured by the ALSFRS-R.
Cognitive impairment and social cognition were not associated with increased caregiver burden or depressive and anxious symptomatology in caregivers. However, correlational analyses showed a significant positive relationship between caregiver burden and adynamia in PwMND; such that the presence of adynamia in PwMND was associated with higher levels of caregiver burden.
Conclusions: Levels of caregiver burden, as well as symptoms of anxiety and depression were not significantly associated with the presence of cognitive impairment or reduced social cognition in PwMND. Overall poor motivation and loss of interest was what carers identified as most challenging.
P164 DECISIONS ON LIFE SUSTAINING TREATMENT: THE ROLE OF COGNITIVE FACTORS
SORG S, HEIZMANN C, LUDOLPH AC, LULÉ D
Department of Neurology, University of Ulm, Ulm, Germany
E-mail address for correspondence: [email protected]
Keywords: cognitive functioning, LST-decisions
Background: In recent years, ALS has been understood as a multiple system disorder that comprises cognitive impairments in addition to the loss of physical function. Evidence and opinions on the extent of cognitive impairments differ. The most salient cognitive deficits relate to executive functions like attention, memory, and verbal fluency. In most studies, bulbar onset patients seem to suffer from stronger cognitive impairments than spinal onset patients. To our knowledge, there are no data on the relationship between the status quo of cognitive functioning and decisions regarding life sustaining treatment.
Objectives: First, we wanted to investigate cognitive functioning in our patient sample; and secondly, we aimed to present data on the association between cognitive impairment and decisions regarding life sustaining treatment.
Methods: 48 patients and 73 controls were tested by a version of the d2 attention test that relied on eye movements as reaction indicators. The d2 measures vigilance (missings) and behavioural inhibition (false alarms). Speech comprehension and verbal memory/intelligence were assessed by standardised instruments. Verbal fluency was tested in patients and compared with controls (exclusion: score ≤ 3 on ALS-FRS speech item). Depressive symptoms and quality of life were assessed by standardised instruments. Decisional status was measured for NIV, PEG, and IV.
Results: Controls had significantly higher scores in cued verbal recall/verbal intelligence than both spinal and bulbar onset patients (P = 0.000). This held true after we controlled for level of education. Both patient groups scored lower on the d2 vigilance score, but only bulbar patients showed signs of behavioural disinhibition. In our sample, no differences were found in speech comprehension as well as verbal fluency between controls and patients. None of the cognitive variables were related to the decisional status on life sustaining treatment options and psychosocial adaptation.
Conclusion: We found no evidence for an association between cognitive impairment and decisions in favour of life sustaining treatment. This implies that patients with mild cognitive deficits may well be able to make adequate decisions on this matter.
P165 APATHY AND DEPRESSION IN ALS: TWO DIFFERENT CONSTRUCTS
CALVO A, MOGLIA C, CANOSA A, GALLO S, BERSANO E, MELISA A, TAVERNA R, MONTUSCHI A, CHIÒ A
ALS Center, Neuroscience Department, University of Turin, Turin, Italy
E-mail address for correspondence: [email protected]
Keywords: apathy, depression
Background: Apathy is a frequent symptom in ALS patients, possibly reflecting a dysfunction of frontal lobes and of the frontal-subcortical circuits. Apathy is a multidimensional concept referring to a mental state of reduced interest and participation in various activities, characterized by a sense of indifference which is not present in depression, where mood is distinctly negative and causes emotional suffering. In Parkinson's disease the absence of a correlation between apathy and depression has been demonstrated. However, there are no studies on the relationship between apathy and depression in ALS.
Aim: To evaluate the presence and the correlates of apathy and depression in patients with ALS.
Methods: Forty one patients with ALS consecutively seen in our ALS Clinic between 1 January 2010 and 28 February 2010 were administered with the Lille Apathy Rating Scale (LARS), a patient-based assessment of apathy in PD, the Hospital Anxiety and Depression Scale (HADS), as a brief instrument to measure psychological distress, the McGill Quality of Life Questionnaire (MQOL) and the Single Item Scale (SIS), rating overall quality of life. The functional status of patients was evaluated with ALSFRS-R.
Results: The patients (21 men and 20 women; 31 spinal and 10 bulbar onset) had a mean age of 65.5 (SD 11.1) years. Their mean ALSFRS-R score was 36.8 (range 19–47). According to the rating scales cut-off scores, mild to severe depression was present in only 4 (9.8%) patients, anxiety in 14 (34.1%) patients and apathy in 13 (31.7%) of patients. Apathy scores were not related to depression (P =0.07) or anxiety (P =0.85) scores. However, among the four domains of the LARS, action initiation (P= 0.01) and intellectual curiosity (P=0.03) were related to HADS depression scores, while emotion (P= 0.91) and self-awareness (P=0.46) were not related. LARS scores were not correlated with MQOL scores. Conversely, in multivariable analysis, both depression and anxiety scores showed a strong correlation with quality of life scores.
Conclusions: Depression and apathy are different constructs in ALS. Apathy was detected in about one third of ALS cases in our series, in keeping with the description of frontotemporal features in 20 to 50% of ALS patients. Apathy does not seem to worsen patients' QOL, differently from its negative impact on caregivers' QOL.
P166 A STUDY OF ALEXITHYMIA AND DEPENDENCE ON EXERCISE IN ALS
ROY-BELLINA S1, LECORNEY A2, CAMU W1, PAGEOT N1, JUNTAS-MORALES R1, GÉLY-NARGEOT M-C2
1Clinique du Motoneurone, Montpellier, France, 2Université Paul Valéry, Montpellier, France
E-mail address for correspondence: [email protected]
Keywords: alexithymia, exercise
Background: Different authors agree about the ‘kindness’ of ALS patients. Aside from this surprising characteristic, some others write about ‘the apparent indifference’ of the patients when facing this devastating disorder.
Objectives: Taking into account that addictive behaviors are associated with an alexithymic profile on one hand, and on the other hand that ALS patients often practice sport intensively, we searched for a link in ALS patients between ‘addiction’ to exercise, and alexithymia.
Methods: Sixteen ALS patients (8 men, 8 women, aged 63 yrs±8.3), with a mean duration of disease of 37 months (±34) and a mean ALSFRS score of 37 (±4.77), and 16 controls (7 men, 9 women, aged 60 yrs±9.37) were evaluated with the following: MMSE, EDF (frontal scale), BDI-II and MADRS (depression), STAI-Y (anxiety), Ekman test (identification of emotional states from 60 facial expressions), Emotional State Questionnaire (ESQ), Bermond Vorst Alexithymia Questionnaire (BVAQ) and Exercise Dependence Scale-Revised (EDS-R). Patients with dementia were excluded from this study.
Results: ALS patients were significantly more depressed (P= 0.009) and anxious (P=0.03) than controls. There were no differences regarding dependence on exercise and alexithymia. A trend was noted for a difference in the ability to identify emotions in the Ekman test (P = 0.06). We could not find any correlation between our tests (dependence to exercise, depression, anxiety, emotional profile) and alexithymia : EDS-R☆BVAQ (P=0.45), BVAQ☆BDI-II (P=0.3), BVAQ☆MADRS (P =0.21), BVAQ☆STAY-E (P =0.1), BVAQ☆STAY-T (P=0.24), BVAQ☆ESQ (P = 0.29).
Discussion and conclusions: Conversely with several studies we found that ALS patients were more depressed than controls, and also more anxious. No specific anxious profile could be determined from our analysis. Our hypothesis of a link between ‘addiction’ to exercise and alexithymia could not be confirmed.
P167 BEHAVIOURAL CHANGES IN MOTOR NEURON DISEASE
LILLO P, MIOSHI E, HODGES JR
Prince of Wales Medical Research Institute, University of New South Wales, Sydney, NSW, Australia
E-mail address for correspondence: [email protected]
Keywords: behavioural changes, carer burden, apathy
Background: Motor Neuron Disease (MND) has traditionally been considered a pure motor syndrome which spares aspects of cognition and behaviour, although in recent years it has been suggested that up to 50% of patients with MND may develop frontal dysfunction which in some cases is severe enough to reach criteria for Frontotemporal Dementia (FTD). Behavioural changes, emotional lability and impaired social cognition are part of the clinical spectrum but estimates of the frequency of presentation remain unknown.
Objectives: To assess the frequency of behavioural changes in patients with MND, to assess the potential impact of behavioural changes on carer burden and to compare the behavioural changes in MND vs. behavioural variant frontotemporal dementia (bvFTD).
Methods: A postal survey was completed by patients with MND (n =92) and their carers (n =81) from New South Wales. The survey covered motor function and neuropsychiatric symptoms in the patients. Carers received questionnaires regarding their current mood and burden of care. Carer reports were compared with carers of patients with bvFTD (n= 25).
Results and discussion: Carers reported moderate-severe behavioural changes in MND patients involving principally motivation (40%). A substantial proportion of MND patients manifested behavioural changes of the type seen in FTD with 11% fulfilling criteria of frontotemporal dementia. Less than 25% of the carers reported depression but 44% reported severe burden. The carer burden was explained predominantly by the presence of abnormal behaviour. As expected, behavioural changes were significantly more frequent in bvFTD than in MND patients (P< 0.001).
P168 DEFICITS IN CONCEPT FORMATION IN AMYOTROPHIC LATERAL SCLEROSIS
ELMAN L1, LIBON D2, GUNAWARDENA D1, MCMILLAN C1, AVANTS B1, MCCLUSKEY L1, GROSSMAN M1
1University of Pennsylvania Medical Center, Philadelphia, PA, United States, 2Drexel University College of Medicine, Philadelphia, PA, United States
E-mail address for correspondence: [email protected]
Keywords: frontotemporal lobar degeneration, executive function, structural MRI
Background: The type of cognitive impairment that is most commonly seen in patients with amyotrophic lateral sclerosis (ALS) is in the spectrum of frontotemporal lobar degeneration (FTLD), which includes a prominent dysexecutive component. Letter and category fluency tasks are the most commonly used measures to screen for dysexecutive function but are not ideal in this population because they are timed and include a motor component.
Objectives: We chose an untimed measure of concept formation for administration to ALS patients to determine if this measure would discriminate between ALS patients who are cognitively intact (ALS-nl), have mild cognitive impairment (ALS-CI), or have FTLD (ALS-FTLD). We related performance on the concept formation task to MRI assessments of cortical thickness.
Methods: A standard screening procedure divided patients into three groups, ALS-nl, ALS-CI, ALS-FTLD. They performed the Delis-Kaplan Executive Function System Sorting Test (DKST). This involves sorting 16 stimulus words printed in various fonts and styles on differently shaped cards. A subset of 16 patients underwent high resolution structural MRI scans.
Results: ALS-CI and ALS-FTLD groups had significantly lower age-corrected scaled scores compared to the ALS-nl group on measures of free, recognition and composite free/recognition sorting. The ALS-FTLD group scored lower than the ALS-CI group on all sorting measures. Regression analyses related measures of action naming, single word semantic knowledge, and mental search/working memory to sorting performance. MRI showed cortical thinning in bilateral premotor/dorsolateral prefrontal regions and left anterolateral temporal cortex. Reduced performance on the composite measure of free/recognition sorting was related to cortical thinning of left dorsolateral prefrontal cortex.
Discussion and conclusions: ALS is a multi-system neurodegenerative disorder. Cognitively impaired or demented ALS patients performed poorly on untimed concept formation tasks that do not depend on motor functioning. Disease in prefrontal regions interrupts a network of cognitive operations leading to the concept formation deficit in ALS.
P169 IMPAIRED INSIGHT FOR COGNITIVE DECLINE IN ALS WITH COGNITIVE IMPAIRMENT
FLAHERTY-CRAIG C, BROTHERS A, SVOBODA R, SIMMONS Z
Penn State College of Medicine/Hershey Medical Center, Hershey, PA, United States
E-mail address for correspondence: [email protected]
Keywords: cognitive impairment, insight, anosognosia
Background: It is now generally accepted that amyotrophic lateral sclerosis (ALS) is a complex multisystem disorder which is accompanied by symptoms of frontotemporal lobar degeneration (FTLD) in up to half of patients. Loss of insight, a characteristic deficit of FTLD, has been reported in patients with ALS with regard to behavior, but loss of insight for cognitive deficits (cognitive anosognosia) has received less attention.
Objectives: To examine cognitive anosognosia in patients with ALS and cognitive impairment (ALS-Ci).
Methods: Participants included ALS subjects with one or more deficiencies on brief exam measures of letter fluency, COGNISTAT abstractions, or COGNISTAT judgment (ALS-Ci), ALS subjects with intact cognitive brief exams (ALS control), and healthy controls. Using Barrett et al's method, cognitive anosognosia ratios were calculated for four motor-free neuropsychological assessments, including verbal problem-solving (Guilford Alternate Uses), non-verbal problem-solving (Guilford Missing Cartoons and Expression Grouping), and emotional recognition (Ekman Faces). Descriptive statistics are reported for demographic and disease data. One-way Analysis of Variance was used to compare test performance and anosognosia ratings among all three groups. Post-hoc analyses were conducted using the Games-Howell test.
Results: There were 56 subjects: 25 ALS-Ci, 13 ALS controls, and 18 healthy controls. Participant Characteristics: No statistical difference was found between groups based on demographic variables or disease characteristics. On average, participants were 62.4 with 13.7 years of education. Patients had an average forced vital capacity (FVC) of 67.7, with 35.4 months since onset. Test Performance: ALS-Ci participants performed worse on Alternate Uses compared with ALS-control (P = 0.003) and healthy control (P < 0.001) participants. ALS-Ci participants fared worse than healthy controls on Missing Cartoons (P = 0.002) and Expression grouping (P <0.001). On Ekman Faces, the ALS-Ci group performed significantly worse than both the ALS-control group (P = 0.02) and the healthy control group (P = 0.002). Insight: ALS-Ci participants overestimated their performance on the Alternate Uses task compared to healthy controls for both the pre-test (P = 0.03) and post-test (P = 0.001). ALS-Ci participants over-estimated their performance compared to healthy controls on Missing Cartoons post-test (P=0.04). A significant difference was found between ALS controls and healthy controls on the Missing Cartoons pre-test (P=0.04). No differences were found for anosognosia ratios for Expression Grouping or Ekman Faces.
Discussion and conclusions: Not only did ALS-Ci patients perform significantly worse than controls on each task, they also tended to over-rate their performance on measures of verbal and non-verbal problem solving alike. The ALS control group over-rated their performance compared to healthy control subjects. Further studies are needed to address whether minimization of cognitive deficits in ALS represents a psychological response to the cognitive decline by the patient with co-existing motor neuron disease, or a genuine indication of loss of insight. Areas of impact include treatment compliance and safety, affecting longevity and quality of life.
P170 THE CLINICAL UTILITY OF PRIMITIVE REFLEX EXAMINATION IN MOTOR NEURON DISEASE
FISHER F1, REARDON K1, PAVLIS A2
1Calvary Health Care Bethlehem, Melbourne, Australia, 2Victoria University, Melbourne, Australia
E-mail address for correspondence: [email protected]
Keywords: primitive reflexes, cognition
Background: MND is no longer regarded as primarily a motor system disease. Research has consistently identified mild to severe cognitive impairment and behaviour disturbances in as many as 50% of individuals with MND. Accurate detection of cognitive impairment is essential as those with coexisting frontotemporal syndrome have reduced life expectancy and will require additional support with decision-making, particularly in regards to life-prolonging interventions.
The reappearance of primitive reflexes in adulthood has traditionally been taken to be an indicator of pathological brain functioning, and likely indicative of a ‘release phenomenon’ when loss of inhibition occurs in neural networks. The re-emergence of primitive reflexes has also been described as a clinical indicator of prefrontal dysfunction; however evidence for a causal relationship is inconsistent. Primitive reflexes have been suggested to be useful in assessing the severity of Parkinson's disease, and also in distinguishing Parkinson's disease from vascular parkinsonism. While primitive reflexes are commonly assessed during routine neurological examination, the presence of primitive reflexes in individuals with MND, and the potential clinical utility of primitive reflex examination has not been extensively examined.
Objectives: The current research aimed to examine the relationship between the presence of primitive reflexes and the presence of significant cognitive and behavioural impairment in persons with MND.
Methods: The sample comprised 46 participants (42% females and 48% males) with formally diagnosed MND recruited through specialist MND clinics. Demographic information (eg age, months since symptom onset, and MND phenotype) was collected for all participants. Examination of primitive reflexes and current functioning (ALSFRS-R) was conducted by neurologists during routine neurological examinations. Cognition, behaviour, and social cognition were assessed using the Addenbrooke's Cognitive Examination-Revised (ACE-R), the Frontal Behavioural Inventory (FBI), and the Reading the Mind in the Eyes Test (Eyes Test) respectively.
Results: Sixteen ALS patients (34.8%) had at least 1 positive primitive reflex, while 13 participants (28.3%) had 2 or more positive primitive reflexes. Chi-squared analyses revealed that the presence of 1 or more positive primitive reflexes was not significantly associated with age, gender, disease duration, or MND phenotype (P > 0.05). Furthermore, Chi-squared tests found no significant association between the re-emergence of primitive reflexes and the presence of cognitive impairment, behavioural disturbance, and social cognition (P > 0.05).
Conclusions: While positive primitive reflexes were present in 34.8% of the sample, their utility as an additional clinical marker of cognitive dysfunction was not supported. Thus, the examination of primitive reflexes during routine neurological examination does not appear to assist recognition of cognitive impairment in MND.
P171 DEVELOPMENT OF A HANDS-FREE, EYE-TRACKING VERSION OF THE TRAIL MAKING TEST
SHARMA R, HICKS S, KHAN A, BERNA C, KENNARD C, TALBOT K, TURNER M
Oxford University, Oxford, United Kingdom
E-mail address for correspondence: [email protected]
Keywords: eye-tracking, cognitive, frontotemporal
Background: The Trail Making Test (TMT) is a two-part written tool for detecting executive dysfunction. Part A consists of joining a ‘trail’ of ascending numbers, which serves as a control for a second more challenging Part B where the ‘trail’ alternates between ascending numbers and alphabetical letters. Executive dysfunction is common in patients with ALS. Both neuropsychological assessment and oculomotor functions can help inform the wider cerebral disease process in ALS (1). However many neuropsychological tests may be impossible in the presence of upper limb weakness or speech impairment.
Objectives: To design a hands-free version of the TMT performed visually using eye-tracking equipment, and to assess its reliability compared with the standard written version.
Methods: Forty healthy volunteers were recruited. A head-mounted Eyelink® eye-tracking device was used in conjunction with a visual display of the trails. To eliminate any immediate practice effects there was an interval of one week between testing each version of the TMT. The first twenty volunteers performed the visual followed by the written version one week later, and the remaining twenty vice versa. The B:A ratio of time in seconds for each task was used as a standardised reliability outcome measure across the two different paradigms.
Results: Good correlation (r2 = 0.6) was found between written and visual TMT outcome measures.
Discussion: The TMT transferred well to the eye-tracking environment and is independent of limb or speech impairments. The oculomotor TMT provided additional information about the errors made and the search strategies used by subjects, which could not be derived from the traditional written version.
Conclusions: Eye-tracking has significant potential as a tool to explore cognitive impairment as well as specific oculomotor abnormalities. It has subsequently proved easily applicable to ALS patients across a range of disability in an on-going longitudinal study.
Reference:
- Donaghy C, Pinnock R, Abrahams S, et al. J Neurol. 2010;257(7):1134–40.
P172 A NEW METHOD OF CATEGORISING NEUROPSYCHOLOGICAL DEFICITS IN PEOPLE WITH ALS
JORDAN N3, PENDER N1, PHUKAN J2, ABRAHAMS S4, ELAMIN M1, BEDE P1, HARDIMAN O1,2
1Beaumont Hospital, Dublin, Ireland, 2Trinity College Dublin, Dublin, Ireland, 3Royal College of Surgeons in Ireland, Dublin, Ireland, 4Department of Psychology, Institute of Psychiatry, King's College London, London, United Kingdom
E-mail address for correspondence: [email protected]
Keywords: consensus criteria, cognitive/behaviour impairment, methodology
We present the first methodological analysis of the consensus criteria (1) in a population-based study of ALS, and we make our own recommendations for alternative analysis. The current paper is based on the initial dataset gathered by one of the authors as a part of a longitudinal Irish population-based study of ALS; the largest of its kind. Analysis of the results of an extensive neuropsychological battery (including tests of executive function, memory, language and visuospatial ability) from 51 patients with ALS, but with no co-morbid dementia, was carried out.
Results indicate that use of the Strong et al. (1) criteria identified 33% of patients with ALS as ALSci (ALS cognitive impairment; defined as below the fifth percentile on two or more tests of executive function). Our recommended criteria identified 13.7% of patients with ALS as having executive dysfunction and a further 31.4% as having some other cognitive impairment (memory, multidomain, language or visuospatial). Therefore, based on our analysis, 45.1% of people with ALS had some form of cognitive impairment.
Patients were categorized separately for behavioral impairment. Results of an analysis of the Frontal Systems Behaviour Scale (FrSBe) indicated that 23% of the 51 participants with ALS had behavioral impairment. In an attempt to avoid over-diagnosis of behavioural impairments, a classification of behavioural impairment is only given if the person scores more than 65 on the T-scores of 3 or more of the after subscales, unless the person's before score on the same subscale was also over 65, thus demonstrating no change over time.
Based on the results of the analysis, it appears that the Strong criteria may underestimate the number of people with cognitive impairment and the nature of impairment. The results of analysis using the Strong criteria suggests that 33% of bulbar and spinal onset participants combined were classified as cognitively impaired (ALSci), compared with 4% of the control participants. Our analysis demonstrated that in fact up to 45% of ALS participants may be impaired on one or more cognitive domains of interest. However, in our suggested analysis, a smaller percentage of 13.7% of the participants with ALS who have not been classified with FTLD had executive dysfunction (5.9% executive dysfunction, 7.8% multidomain with executive dysfunction).
In addition, a large number had impairment in other areas, such as 7.8% with memory impairment, 11.8% with multidomain impairment without executive dysfunction and 9.8% with language impairment. In addition, based on analysis using our suggested methodology, 2.6% of controls met criteria for executive impairment, in contrast with the Strong criteria, which found that 12% of controls had executive impairment, a number that seems high for a control population.
Reference:
- Strong MJ, Grace GM, Freedman M, et al. Amyotrophic Lateral Scler. Jun;10(3):131–46.
P173 COGNITIVE PROFILE OF ALS PATIENTS WITH COMPARISON OF INCIDENT VERSUS PREVALENT POPULATIONS: A DOMAIN-BASED APPROACH
ELAMIN M1,2, PHUKAN J1,2, BEDE P1,2, JORDAN N1, PENDER N1, HARDIMAN O1 ,2
1Beaumont Hospital, Dublin, Ireland, 2Trinity College Institute of Neuroscience, Dublin, Ireland
E-mail address for correspondence: [email protected]
Keywords: cognitive, classification, incident
Background: Estimates of cognitive impairment in patients with amyotrophic lateral sclerosis (ALS) vary widely. Studies published to date are small, clinic based and are heavily reliant on the prevalent ALS population. The literature is further confounded by the currently used criteria (1) for cognitive impairment in ALS which focuses on executive dysfunction, when there is increasing evidence of involvement of other cognitive domains in ALS.
Objectives: 1) To describe the incidence of cognitive impairment in a population-based ALS cohort using new criteria that focus on single or multiple cognitive domain dysfunction; 2) To compare frequency of cognitive impairment in the incident ALS patients to the prevalent ALS patients.
Methods: All cases of possible, probable or definite ALS in the Republic of Ireland have been invited to participate. Demographic, clinical and neuropsychological data are collected during home visits carried out at baseline and then repeated at six monthly intervals.
Cognitive impairment in ALS patients is defined according to performance on neuropsychological tests covering five cognitive domains: attention/executive function, memory, language, visuospatial skills. Patients were then classified depending on two factors: 1) the number of cognitive domains involved (none, single or multiple); 2) the presence or absence of executive dysfunction. The frequency of each cognitive syndrome in the ALS population was compared to that in age, sex and education-matched healthy controls.
Results: To date, 167 patients have been recruited to the study. Mean age was 63.5 years, 60% were males and 33.6% of patients had bulbar-onset ALS. Incident cases comprised 83.3% of the cohort.
Twenty two patients (13.6%) fulfilled the Neary criteria for frontotemporal dementia (FTD). Compared to healthy controls, non-demented ALS patients had significantly higher frequency of single or multi-domain cognitive impairment with executive dysfunction (29.9% versus 2.6%, P < 0.0001). There was no significant difference between the 2 groups in frequency of single or multi-domain cognitive impairment without executive dysfunction (22.2% versus 16.5%, P = 0.311).
Incident ALS cases had a higher frequency of cognitive impairment compared to prevalent cases. The most striking difference was observed in the frequency of single domain executive dysfunction but it did not reach statistical significance (11.7% versus 3.7%, P = 0.39).
The striking absence of executive dysfunction in the prevalent cases compared to the incident cases led to an investigation into the effect of executive dysfunction on patient survival. The presence of executive dysfunction was found to be significantly associated with shorter survival in ALS patients (31 months versus 38 months, P = 0.005) even after correcting for multiple factors including age, bulbar onset and disease severity.
Discussion and conclusion: The incidence of FTD in a population based predominantly incident ALS cohort was 13.6%. Single and multi-domain executive dysfunction was significantly more frequent in the ALS population compared to healthy controls. The incident population had a higher incidence of impairment in all cognitive domains, in particular executive function. The fact that executive dysfunction was predominantly a feature of incident, rather than prevalent ALS patients, is likely to be secondary to its association with poorer prognosis.
Reference:
- Strong MJ, Grace GM, Freedman M, et al. Amyotrophic Lateral Scler. 2009;10(3):131–46.