P149 EMOTIONAL CONFLICTS OF PATIENTS WITH AMYTROPHIC LATERAL SCLEROSIS ON WEARING INVASIVE VENTILATORS
Kitamura Y
National Rehabilitation Center, Tokorozawa, Japan
E‐mail address for correspondence: [email protected]
Objectives: This study aimed to reveal emotional conflicts of patients with ALS before and after wearing invasive ventilators.
Methods: Semi‐structural interviews were conducted with 20 subjects (11 patients and 9 family members) in three areas of Japan.
Results: The following results were obtained: 1) most patients felt shocked when they were told about their successive respiratory dysfunction, 2) patients wondered whether they should use ventilators or not, and it took them up to four years to make this decision, 3) five out of six ventilator users felt resistance to wearing ventilators, but four of them accepted use later with the support of their families, peers and health providers.
Conclusion: Emotional conflicts surrounding decision‐making on the use of ventilators is one step on the path of coping with ALS. Adequate support from patients' families, peers, and health providers is important for a smooth decision‐making process.
P150 PSYCHOLOGICAL AND PSYCHOPATHOLOGICAL APPROACH TO PATIENTS AFFECTED BY AMYOTROPHIC LATERAL SCLEROSIS
La Barbera D, Mezzatesta C, Costanzo Pellegrino C & La Cascia C
University of Palermo, Palermo, Italy
E‐mail address for correspondence: [email protected]
Objectives: To examine the relationship between patients' personality traits and the evolution of their disease. Our hypothesis is that family relations form the basis for mood alterations and that these are associated with personality changes in patients suffering from ALS. Secondary aims sought to examine how the patient‐caregiver relationship affects patients' average survival time, to study the prevalence of symptoms such as anxiety and depression in patients with ALS, and the defensive mechanisms commonly employed. Finally, we sought to evaluate how psychological support for both the patient and caregiver can improve their quality of life.
Methods: Twenty patients suffering from ALS and 20 caregivers were administered psychodiagnostic interviews with a focus on psychopathological symptoms, in addition to the Eysenck Personality Inventory (EPI) scale, State Trait Anxiety Inventory (STAI) scale and Hamilton Depression (HDS) scale.
The data were statistically analysed by two‐tailed Student's t‐test (independent samples design). Exclusion criteria for all subjects included dementia and alterations of cognitive function.
Results and conclusions: The psychological evaluation showed that patients suffering from ALS have higher scores on the STAI, as well as higher levels of somatic anxiety and depression. They present a rigid defensive system and their survival time is correlated with quality of life.
P151 HOPE AND ALS
O'Sullivan I, Albert SM, Rabkin JR, Del Bene ML, Tider T, Rowland LP & Mitsumoto H
Eleanor & Lou Gehrig MDA/ALS Center, Columbia University, New York, USA
E‐mail address for correspondence: [email protected]
Background: The assumption that a diagnosis of ALS leads to hopelessness and depression is prevalent in society and among health care professionals. The absence of a cure conjures up images of suffering and death. Recent studies of symptom palliation and effective disease management have begun to challenge this perception. Nevertheless, questions remain about the appropriate point at which to reveal diagnoses and the seriousness of disease progression. Clinicians fear that truthfulness may take away a patient's hope, but we are unaware of studies that have specifically examined hopelessness or optimism in ALS.
Objectives: To examine hopelessness over the course of ALS from diagnosis to death.
Methods: Two cohorts were assessed: 1) patients who were assessed within one year of diagnosis (n = 90), 2) late‐stage patients with high likelihood of dying or need for tracheostomy within six months (n = 80). Both completed 10 items from the Beck Hopelessness Scale (BHS, range 0–10; 10 = maximum hopelessness). Mean hopelessness scores were computed. Late‐stage patients were interviewed monthly as they approached death or tracheostomy. Patients also completed the Beck Depression Inventory (BDI).
Results: Complete BHS data were available for 65 of the recently diagnosed cohort and 60 of the late‐stage cohort. Patients completing and not completing the measure did not differ in sociodemographic or disease characteristics. Mean BHS scores for the two cohorts did not significantly differ (3.54 among recently diagnosed patients versus 3.58 among late‐stage patients). These scores fall in the mild range. By contrast, BDI scores were significantly higher in the late‐stage cohort (13.2 vs. 9.9; p = 0.006), although mean scores in both groups fell below thresholds for clinical significance. In the three months prior to death or tracheostomy, hopelessness scores were mostly stable (3.8, 3.2, 3.8 in people who died; 3.3, 3.5, and 3.0 in people who chose tracheostomy (n = 7)).
Discussion: Empirical findings challenge the assumption that hopelessness increases over the course of ALS. The absence of an increase of hopelessness with disease progression and the approach of death suggests that patients cope with increasing disability and new challenges in disease management. These findings confirm reports of the general resiliency of patients. Additional research is required on patient reactions to physician communication of threatening information and patient coping strategies.
P152 THE PREVALENCE OF DEPRESSION AND ANXIETY IN MND
Wicks P, Abrahams S, Masi D, Hejda‐Forde S, Leigh PN & Goldstein LH
Institute of Psychiatry, London, UK
E‐mail address for correspondence: [email protected]
Background: There is an impression both in clinical practice and the research literature that patients with MND are typically “cheerful, stoical, and positive”. Estimated prevalence rates of clinically significant anxiety and depression in MND are typically low, around 5–10%. This is in marked contrast to higher estimated rates in other neurodegenerative movement disorders such as multiple sclerosis and Parkinson's disease where estimates are around 40–60%. It is not clear whether reliance on specific interview methods may have contributed to differing estimates of affective disorder in people with MND as studies have used varying assessment tools.
Objectives: To estimate prevalence rates of depression and anxiety in MND using different assessment tools.
Method: Three self‐report questionnaires, the Beck Depression Inventory (BDI), Hospital Anxiety and Depression Scale (HADS), and the Spielberger State‐Trait Anxiety Inventory (STAI) were sent to a 12‐ month consecutive sample of non‐demented MND patients attending a tertiary referral clinic in the UK. Standard cut‐off scores were used to identify caseness.
Results: From 190 questionnaire packs sent out, 101 have been returned completed, yielding a 53% response rate. BDI scores reveal that 46% were classified as not depressed, 36% were mild‐moderately depressed, 13% were moderately‐severely depressed, and 5% were severely depressed. The HADS Depression subscale identified that 75% were not depressed, 13% were borderline, and 12% were depressed. STAI State Anxiety identified that 58% had low anxiety levels, 33% had medium anxiety levels, and 8% had high anxiety levels. The HADS Anxiety scale revealed that 65% were not anxious, 17% were borderline, and 17% were anxious. Depression and anxiety scores were both moderately correlated with levels of pain experienced by patients. Despite the presence of psychological distress, 74% of patients were not taking any antidepressant medication, and those patients who were using antidepressant medication had significantly higher levels of both depression and anxiety.
Conclusion: The prevalence of affective disorder amongst MND patients may be higher than previously estimated. However, prevalence estimates for depression vary according to the measure employed, with the BDI producing higher estimates than the HADS. It should also be noted that self‐report questionnaires provide an estimate of symptom frequency, rather than a clinical diagnosis. Nevertheless, their utility in clinical practice should be considered and future research should examine the efficacy of antidepressants and/or psychosocial interventions in MND.
P153 COGNITIVE LINGUISTIC DEFICITS, QUALITY OF LIFE AND DEPRESSION IN ALS PATIENTS: A CROSS‐SECTIONAL STUDY
Pasian V1, Accornero A2, Chio' A3, Terreni AA3, Gauthier A3, Vignola A3, Miletto AM1 & Schindler O1
1Department of Medico‐Surgical Disciplines, University of Torino, Turin, 2Department of Clinical Pathophysiology, University of Torino, Turin, and 3Department of Neuroscience, University of Torino, Turin, Italy
E‐mail address for correspondence: [email protected]
Background: Cognitive symptoms in ALS often go undetected because they are elusive and may be overshadowed by the more apparent physical symptoms. Nevertheless, cognitive deficits are present in ALS although often underestimated. In particular, cognitive linguistic symptoms may be disguised by speech impairment caused by bulbar symptoms. Cognitive linguistic deficits can undermine patients' quality of life, particularly if they go undetected by family members and health professionals. In addition, patients' ability to understand and communicate is essential when crucial decisions about their management are to be taken during the course of the disease.
Objectives: To detect cognitive linguistic deficits in ALS patients and identify any correlations with quality of life and depression.
Methods: Forty‐five patients were administered part of an Italian battery, the BADA (Batteria Analisi Deficit Afasici, ‘Battery for the Analysis of Aphasic Deficits’), comprising four domains: reading comprehension, detection of errors in a text read aloud by the interviewer, number of grammar and spelling mistakes made writing a text, and verbal description of some pictures. Each patient was given the part of the battery they were able to do, depending on whether their writing or speech abilities were preserved. In addition, they were administered the Zung Depression Scale (ZDS) and the McGill Quality of Life Questionnaire (MQoL). Patients' physical status was assessed by the ALS Functional Rating Scale (ALS‐FRS). Pearson bivariate correlations were carried out using Statistical Package for Social Science (SPSS) version 12.0 for Windows. A significance level of p<0.05 was used.
Results: Forty‐five patients were recruited, 31 (69%) of whom were males, and 14 females (31%); 16 (36%) had a bulbar onset, and 29 (65%) a spinal onset. Mean age was 63.0 years (SD 9.8). Mean ALS‐FRS score was 28.8 (SD 7.1). Mean ZDS score was 42.4 (SD 9.5), with 10 subjects over the 50‐point cut‐off score indicating overt depression. Mean MQoL score was 6.9 (SD 1.3). Significant correlations were found between: the detection of grammar mistakes in a text read aloud by the interviewer and the ZDS (p<0.01); the frequency of grammar mistakes and the site of onset (p<0.05); and existential well‐being as measured by MQoL and frequency of spelling mistakes (p<0.05). All cognitive linguistic deficits correlated with age and education, except spelling mistakes, which correlated only with age.
Conclusion: The presence of cognitive symptoms in ALS is somewhat underestimated, partly because they do not stand out as the patient's main problem, and partly because they are difficult to detect, particularly in bulbar patients. Moreover, early symptoms of cognitive impairment might be mistaken for depression, which would not be surprising in someone affected by such a disease. Discriminating between depression and early cognitive impairment may be useful in enhancing patients' quality of life.
P154 FORCED CHOICE COGNITIVE BATTERY IN PATIENTS WITH MOTOR NEURON DISEASE
Rudnicki SA, Drake KW, Fewell DF, Pippenger MA & Hart J
University of Arkansas for Medical Sciences, Little Rock, USA
E‐mail address for correspondence: [email protected]
Background: In recent years, cognitive changes have been reported with increasing frequency in motor neuron disease (MND). Physical changes related to MND may impact test performance and limit the usefulness of timed and written tests. Lengthy neuropsychological testing may prove challenging for a patient who already spends several hours in the multidisciplinary clinic.
Objective: To perform a pilot study of our cognitive battery (CB) in patients with MND in order to determine if it could be administered in less than an hour, and to patients in all stages of disease. We selected brief, untimed, forced choice tests, allowing for eye blink responses if necessary. Some tests had to be modified to fit these criteria. We were also interested in whether performance correlated with disease duration, depression, functional status as measured by ALS‐FRS‐R or forced vital capacity (FVC).
Methods: Patients performed the following tests at baseline, six months and 12 months: Matrix Reasoning (MR), Peabody Picture Vocabulary Test (PPVT), antisaccades (AS), modified CERAD word list recognition (mC), visual analogue scale for depression (VAS‐D), and modified orientation (mO). The neuropsychiatric inventory (NPI) was administered to caregivers. Patients had FVC tested and ALS‐FRS‐R carried out at each visit.
Results: Thirteen patients completed the baseline CB; of these, six have also carried out the six‐month assessment, and one has completed the full 12‐month study. One patient failed to do their six‐month study because of medication‐related sleepiness; all other patients could perform all parts of the CB. Duration of disease at time of the baseline exam was 45.9±44.8 months (range 8–156). Baseline results were as follows: AS (out of a possible 10) 8.6±1.7; scaled MR 11.2±3.2; standardized PPVT 104.1±10.7; mC 9.0±1.7; mO (out of a possible 6) 6±0. The number of patients who scored at least one standard deviation below the normal mean at baseline included two patients on PPVT, four patients on mC, and five patients on AS. On the NPI, caregivers reported abnormalities in four patients, and three of these had at least one other abnormal cognitive test. Three patients had an entirely normal CB. ALS‐FRS‐R scores ranged from 10 to 45, with a mean score of 34.3±9.8. Time to complete the test (exclusive of the NPI) was 35±8 min (range 25–50 min). Using a simple linear regression model, there was a relationship between FVC and MR, FVC and percentile score on PPVT, and ALS‐FRS‐R and MR. There was no relationship between depression or disease duration and CB results.
Conclusion: Our CB could be administered to all patients, regardless of the severity of their disease, in less than an hour. Neither disease duration nor depression played a role in cognitive test results, while respiratory and overall functional status may be a significant influence.
P155 RESEARCH IN AMYOTROPHIC LATERAL SCLEROSIS: INSIGHT OF COGNITIVE FEATURES AND AFFECTIVITY
Palmieri A1, Sorarù G2, D'Ascenzo C1, Baggio L1 & Angelini C1
1Department of Neurosciences, University of Padova, IRCCS S.Camillo, Venezia, and 2Division of Neurology, Piove di Sacco,Padova, Italy
E‐mail address for correspondence: [email protected]
Background: Amyotrophic lateral sclerosis (ALS) has not previously been assessed with a ‘projective’ exam such as the Rorschach Inkblot test.
Method: The Rorschach protocols of 20 patients affected by ALS were examined, using Exner's system of scoring indices. This study evaluated both affective variables of patients and their cognitive information processing, comparing their results to data from the Exner Comprehensive System normative sample; statistical analysis was performed with Student's t‐test.
Results: Patients' responses revealed a significantly abnormal form of perception in the screening of reality, as well as an increased prevalence of disturbed thinking (revealed by high number of ‘special’ scores), inaccurate perceived human responses (M‐) and a higher frequency of reservations. These results suggest impairment of attentional system and cognitive deterioration. Moreover, some of these aspects are linked to psychotic symptoms. Responses also revealed an increased percentage of pure forms (F%) and low affective ratio, which reflects the avoidance of emotional interchange with environment, lack of intense feelings, rigidity on own opinions, ruminative self‐introspections and shame, depressive mood, low self‐esteem, no interest for other people, lack of expectations of close relationship with others, negation of own primary needs, self‐concept thought as partial, and high attention in physical conditions.
Conclusion: Affective features of Rorschach test demonstrate that ALS patients feel a progressive deterioration and are adapting to death, retiring from the outside world. Cognitive aspects are also of interest; Exner Comprehensive System could be used to detect early frontal dementia.
P156 FRONTOTEMPORAL DEMENTIA AND AMYOTROPHIC LATERAL SCLEROSIS: A REVIEW OF CURRENT CONCEPTS
Grossman AB & Bradley WG
Miller School of Medicine, University of Miami, Miami, USA
E‐mail address for correspondence: [email protected]
Background: The presence of dementia in a proportion of ALS patients is increasingly being recognized. Clinical, functional imaging, genetic and pathological studies indicate a continuum of cognitive and behavioural changes among ALS patients that can progress to dementia in some.
Objectives: The present study examined the prevalence of cognitive and behavioural impairment in a sample of ALS patients. Findings will be presented and the current research on FTD and ALS will be reviewed.
Methods: Twenty males and females diagnosed with definite ALS were recruited from the Kessenich Family MDA ALS Center in the Department of Neurology at the Miller School of Medicine, University of Miami. Participants were administered a neuropsychological test battery and a caregiver was asked to rate the patient on a behavioural survey.
Results: Approximately half of the participants were cognitively intact and exhibited no behavioural changes. Two patients were diagnosed with FTD according to the Neary criteria (1). The remaining patients had varying degrees of cognitive and behavioural changes consistent with subclinical levels of FTD. Results of Pearson correlations indicate that degree of cognitive impairment was significantly correlated with severity of bulbar involvement measured by the ALS Functional Rating Scale (p<0.05).
Discussion: The prevalence of cognitive and behavioural changes in this sample of ALS patients is consistent with previous reports. Our hypothesis is that cognitive changes correlate with the severity of bulbar involvement irrespective of whether bulbar involvement started at the outset or only developed later in the course of the disease. First, we will review methodology and results from the current and previous neuropsychological studies on ALS and FTLD, including correlations with pathological data; secondly, longitudinal neuropsychological studies in ALS and the methodological challenges involved in conducting such studies; and finally, the results of our current work on patients' willingness to accept percutaneous endoscopic gastrostomy (PEG), and non‐invasive positive pressure ventilation (NIPPV) (2). The impact of cognitive impairment on patients' underutilization of PEG and NIPPV (3) will also be discussed.
References
- Neary D, Snowden JS, Northen B, Goulding P. Dementia of frontal lobe type. Journal of Neurology, Neurosurgery, & Psychiatry 1988;51:353–61.
- Grossman A, Bradley WG. Factors predicting the acceptance of PEG and BiPap in patients with ALS (manuscript in preparation).
- Bradley WG, Anderson F, Bromberg M, Gutmann L, Harati Y, Ross M, Miller RG, and the ALS CARE Study Group. Current management of ALS: comparison of the ALS CARE database and the AAN Practice Parameter. Neurology 2001;57:500–4.
P157 THE ASSOCIATION OF FRONTOTEMPORAL DYSFUNCTION AND REDUCED VITAL CAPACITY IN ALS
Kim SM, Lee KM, Sung JJ, Min JH, Oh DH & Lee KW
Department of Neurology, Seoul National University Hospital, Seoul, South Korea
E‐mail address for correspondence: [email protected]
Background: ALS is recognized as a multi‐system disorder and impairments of cognitive function have been consistently demonstrated. While the cognitive dysfunction may be related to breathing problems associated with sleep disturbances, the effect of hypoventilation on the cognitive impairments in ALS patients is still unclear.
Objective: To evaluate the association of frontotemporal dysfunction and reduced vital capacity in ALS.
Method: We measured disability with the ALS Functional Rating Scale‐Revised (ALSFRS‐R), Forced Vital Capacity (FVC) and cognitive functions including attention, executive function, and memory in 16 ALS patients (10 males, six females, aged 43–75 years, mean 57.6 ± 10.5 years, ALSFRS‐R 27–44).
Results: The patients were grouped based on FVC. The first group (n = 8, mean ALSFRS‐R = 37) showed reduced FVC (⩽80% of predicted value) and the other group (n = 8, mean ALSFRS‐R = 37) had FVC within normal limits. The groups did not differ significantly in sex, age, education and ALSFRS‐R. The first group performed significantly worse in memory retention (p = 0.028), retrieval efficacy (p = 0.003), spoken verbal fluency (p = 0.03) and spoken verbal fluency index (p = 0.016) than the second.
Conclusion: Reduced vital capacity is correlated with poor performance in tests reflecting fronto‐temporal functions. Therefore, the frontal and/or temporal impairments in ALS might be attributable to potentially reversible secondary effects of hypoventilation, as well as the primary degenerative process observed in the fronto‐temporal dementia‐ALS complex.
P158 PLANNING DYSFUNCTION IN AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Piquard A, Meininger V, Bruneteau G, Le Forestier N, Pradat PF, Quoniam N, Salachas F & Lacomblez L
Hôpital de la Pitié‐Salpêtrière, Fédération des Maladies du Système Nerveux, Paris, France
E‐mail address for correspondence: [email protected]
Background: Executive functions allow coping with new and complex situations. The Tower of London (TOL) was initially developed by Shallice (1) to predict planning problems arising from the dysexecutive syndrome. There is great variability in the extent and degree of impairment in patients with frontal lobe damage. Executive dysfunction has been described in patients with amyotrophic lateral sclerosis (ALS), with frontotemporal dementia (FTD) and in patients with ALS without dementia. However, no study has specifically described the planning function. The study of the link between ALS and FTD appears to be clinically relevant.
Objectives: To characterize the planning function in patients with ALS with and without cognitive deficits (mild cognitive deficit or FTD) and to determine if the TOL (modified version) can discriminate between these two categories of patients.
Methods: We studied the planning performance of 11 ALS patients with FTD (ALS/FTD), 10 patients with cognitive impairment (but not demented) (ALS/CI) and 12 ALS patients without cognitive impairment, compared to 27 age‐matched controls. A modified manual version of the TOL with three different height disks on three rods of different lengths was used in this study. The object is to change the arrangement of the disks to match an arrangement predetermined by the experimenter in the fewest possible moves while respecting certain rules. The subject was given 12 problems with four levels of difficulty (2‐, 3‐, 4‐, and 5‐move levels of difficulty). Five measures were considered: the percentage of perfectly resolved problems (in the maximum allowed moves), planning time, movement (or solution) time, the mean number of moves above the minimum and the number of rule breaks.
Results: Except for the planning time, ALS/FTD and ALS/CI patients generally performed more poorly than both ALS patients and controls as the task difficulty increased. Their respective difficulties were quantitatively and qualitatively distinct. According to the levels of difficulty, ALS/FTD patients could resolve fewer problems, spent more time on problems and made more moves than the ALS/CI patients, ALS patients and controls. Apart from in the easier 2‐move level of difficulty problems, ALS/FTD patients broke the rules more often than ALS/CI, ALS and controls for all the levels of difficulty. ALS/CI patients' difficulties appeared less marked than those of ALS/FTD patients. They performed more poorly than controls but not significantly worse than ALS patients without cognitive impairment. ALS patients without cognitive impairment resolved only the 3‐move level of difficulty problems slower than control participants. Motor deficits could not be considered as biasing performance.
Conclusion: The TOL task was described to be useful for the assessment of problem‐solving and provided a tool for picking up difficulties in planning. Its use appears to be interesting in the ALS population, in which executive dysfunction is increasingly recognized
Reference
- Shallice T. Philosophical Transactions of the Royal Society of London 1982;298:199–209.
P159 PREMORBID PERSONALITY CHARACTERISTICS OF PATIENTS WITH ALS: PRELIMINARY FINDINGS
Grossman A, Levin B & Bradley WG
Miller School of Medicine, University of Miami, Miami, USA
E‐mail address for correspondence: [email protected]
Background: Physicians experienced in treating patients with ALS have often described these individuals as being especially “nice”, noting their remarkable resilience in coping with this relentlessly progressive disease. Previous research studies on the personality characteristics of ALS patients have yielded mixed results.
Objective: The purpose of the present study was to further investigate whether a characteristic premorbid personality profile is associated with ALS.
Methods: Forty‐nine caregivers of newly diagnosed (<six months) ALS patients were recruited for study participation from the Kessenich Family MDA ALS Center in the Department of Neurology at the Miller School of Medicine of the University of Miami. The control group (n = 47) consisted of caregivers of patients newly diagnosed with lung cancer (n = 22), multiple sclerosis [MS] (n = 15), and brain glioma (n = 10). Caregivers completed the NEO‐Personality Inventory and were asked to rate the patient's personality characteristics as they were before receiving their current medical diagnoses.
Results: Results of a multivariate analysis of variance indicated that ALS patients were rated as being significantly lower in the Openness personality trait (F = 3.99, p<0.01) than controls. A univariate analysis of variance was conducted to further examine group differences in Openness. There was a statistically significant difference between the groups (F = 3.37, p<0.05), with the ALS group having the lowest mean.
Conclusion: Preliminary findings indicate that there are differences in premorbid personality characteristics between patients with ALS and those with other chronic diseases. In our study, caregivers rated patients with ALS as being lower in the Openness personality trait than did caregivers of patients with other chronic medical conditions. Individuals who are low in Openness attend to the task at hand, exert control over their emotions, prefer familiarity and routine, and hold traditional values. This personality feature appears to serve an adaptive function in terms of coping with ALS, and to physicians it may cause the patient to appear pleasant, cooperative, and resilient. The exact relationship between the genetic basis of these personality traits and the genes predisposing to or protecting against the development of ALS is uncertain at this time. Future research studies will be needed to examine the association between premorbid personality and the development of aspects of FTD as they impinge on the management and health outcomes in ALS.