C63 NOT ONLY MND: SELF-REPORTED CO-MORBIDITIES AND OTHER DIFFICULTIES THAT IMPACT ON THE NEED FOR HOME CARE SUPPORT AND ALLIED HEALTH SERVICES
GILES M1, LEWIN G2
1Edith Cowan University, Western Australia, Australia, 2Curtin University of Technology, Western Australia, Australia, 3Silver Chain, Western Australia, Australia
Email address for correspondence: [email protected]
Keywords: co-morbidities, home care support, allied health services
Background: In 2006, a survey of the characteristics and support needs of adults living with Motor Neurone Disease (MND) and other neurodegenerative disorders was conducted in Western Australia (Giles and Lewin, 2008). The survey data provided self-reported information on co-morbidities and physical and mental health symptoms as well as respondents’ functioning defined in terms of Lawton and Brody's (1969) Incidental Activities of Daily Living (IADL) and Barthel's Activities of Daily Living (ADL) (Collin, Wade, Davies, & Horne, 1988). Giles and Lewin used the IADL and ADL results to assign a level of dependency to each of the respondents. Home support needs for people with MND, disaggregated by level of dependency and type of support, were reported to the 2008 APF in Birmingham, UK.
Objectives: This paper will report on the development of a composite index to predict the need for home support and allied health services, for people with MND. A comparison will be made of the usefulness of this composite index compared with using broad levels of dependency.
Outcomes: The survey sample of people with MND (n = 56) had up to four co-morbidities. The most common of these were high blood pressure (15%), diabetes (12%), musculoskeletal problems (10%), depression/anxiety (8%) and cancer (7%). Difficulties related to physical and psychosocial symptoms were experienced to some degree by all people with MND in the sample.
The composite index includes number of co-morbidities as well as number of difficulties weighted according to how they are reported in the survey - mild, medium or extreme. These difficulties may be related to co-morbidities or to the underlying MND disorder. Alternatively, the number and severity of the difficulties may also be related to age or length of time since appearance of first symptoms. Hence these two additional variables are also incorporated into the composite index.
The index and suggestions for how it may be used, together with the results of the comparison analysis, will be described in the paper.
Recommendations to the field: Care pathways for people with MND should also consider co-morbidities and physical and psychosocial symptoms that could compound their needs for services, including allied health and home care support services. The composite index described in this paper can provide useful input to the development of these care pathways.
C64 INVESTIGATING ASPECTS OF SOCIAL COMMUNICATION IN AUSTRALIANS WITH MND
FISHER F1, PAVLIS A2, MAULE R1, STAIOS M3, BRINKMANN S1, HOWE J1, REARDON K1, MATHERS S1
1Calvary Health Care Bethlehem, Melbourne, Victoria, Australia, 2Victoria University, Melbourne, Victoria, Australia, 3La Trobe University, Melbourne, Victoria, Australia
Email address for correspondence: [email protected]
Keywords: cognition, emotion, communication
Background: Cognitive and behavioural changes in people with Motor Neurone Disease (PwMND) without FTD are well established. While there has been extensive research into these areas, less research exists examining the impact of social communication changes, particularly the impact on caregivers. High level social communication is dependent upon a combination of factors, including emotion recognition, pragmatic language skills and social cognition. Impaired social communication may be seen as ‘social inappropriateness’ and has been found to be a significant factor in interpersonal relationships and quality of life.
Objectives: The current research aimed to prospectively investigate the nature of social communication deficits in Motor Neurone Disease (MND), by examining affect recognition, and also the recognition of sarcasm and sincerity. It was hypothesised that PwMND without dementia would perform worse than healthy controls, and that these changes would be associated with reduced empathy. The current research also aimed to examine the relationship between changes in social communication, and caregiver burden and psychological ill health.
Methods: 38 PwMND without dementia and their nominated caregiver, and 27 age- and sex-matched healthy controls and informants completed selected TASIT and CATS subtests, the LaTrobe Communication Questionnaire and an empathy questionnaire. Caregivers also completed caregiver burden and mood questionnaires. Data were analysed using non-parametric methods.
Results: In comparison to healthy controls, PwMND showed significant difficulty on a task requiring participants to interpret sincere and complex sarcastic interpersonal exchanges, particularly in the absence of contextual cues. When contextual cues were present, PwMND were able to accurately identify the basic affective states of others as effectively as healthy controls.
For PwMND, difficulties in both discriminating facial affect and recognizing contextual positive emotions were associated with increased anxiety and depression in carers. Behavioural changes such as increased apathy, disinhibition and executive dysfunction were also associated with increased carer burden and depression.
Overall caregivers of PwMND did not identify significant changes in everyday social communication and empathic concern, relative to controls.
Discussion and conclusions: Results demonstrated that PwMND without dementia do have difficulties with affect recognition when contextual cues are absent. While affect recognition was improved when PwMND were provided with context, higher level deficits in the recognition of sarcasm and sincerity in social situations remained. These impairments were present in the absence of noticeable changes in carer-rated everyday social communication and empathy. The current study has captured a cohort of PwMND who may be in the early stages of decline in their overall social communication. Given impaired social communication can negatively impact social relationships and may contribute to relationship stress and social isolation, early screening has the potential to provide more timely support to carers who are dealing with these changes.
C65 JUDGING A BOOK BY ITS COVER? WELL-BEING AND DECISIONS IN ALS
LULÉ D1,2, LANG D1, EHLICH B1, SORG S1, HEIMRATH J1, HEIZMANN C1, KÜBLER A3, BIRBAUMER N2,4, LUDOLPH AC1
1University of Ulm, Ulm, Germany, 2University of Tübingen, Tübingen, Germany, 3University of Würzburg, Würzburg, Germany, 4Ospedale San Camillo, IRCCS, Venice, Italy
Email address for correspondence: [email protected]
Keywords: well-being, peer assessment, advanced directives
Background: Decisions to prolong or shorten life in fatal diseases like ALS are strongly influenced by healthy individuals, like caregivers and physicians. Furthermore, some researchers and clinicians suggest that ALS patients should decide ahead of time on advanced directives to circumvent any confounding effects of cognitive impairments on decision making in the course of ALS. It is not yet clear whether healthy individuals can correctly anticipate a patient's well-being and decisions.
Objectives: It was the aim of the study to determine the ability of healthy individuals (caregivers and aged matched healthy individuals) to anticipate a patient's well-being and his decisions.
Material and methods: Forty ALS patients and their caregivers and 110 age-matched healthy individuals were asked to judge factors of a patient with ALS: emotional well-being (depression and quality of life) and decisions to hasten death. Depression was determined with Allgemeiner Depressions Fragebogen, a German version of the Center for Epidemiological Studies Depression Scale (CES-D-Scale), and the ALS Depression Inventory 12 Items (ADI-12). Quality of life was determined with the Anamnestic comparative self assessment (ACSA). Attitudes toward hastened death were evaluated with the Schedule of Attitudes Toward Hastened Death (SAHD). All scales have been validated in ALS studies.
Patients judged their own well-being and decision status, the caregivers judged the well-being and decision status of the patient he/she is taking care of. Healthy individuals were asked to judge the well-being and decision status of a virtual patient. Additionally, caregivers and healthy individuals were asked to judge their own well-being. Cognition as a confounding factor for decision status was excluded in patient's group.
Results: Patients reported a good well-being and a low wish to hasten death. Caregivers and healthy individuals rated the patient's well-being significantly lower. The wish to hasten death was significantly lower in the patient group compared to what healthy individuals thought the/a patient would think. The rater's own well-being was a significantly better predictor of the peer assessement than the patient's well-being. Cognition was not associated with decision status in patients.
Discussion and conclusions: Patient's well-being was overall good. Healthy individuals were not correct in their assessment of a patient's well being. They rated the patient's well-being significantly lower. They draw their conclusions from their own well-being. Anticipating how a patient actually feels is more than challenging from the perspective of a healthy person. Advanced directives should be dynamically corrected to guarantee that the patient's will is as best met as possible when treatments are to be taken. Healthy individuals influencing decisions concerning the patient's life like caregivers, physicians and politicians should take these findings into consideration.
C66 THE IMPACT OF BEHAVIOURAL, BULBAR, RESPIRATORY AND MOTOR IMPAIRMENT ON ACTIVITIES OF DAILY LIVING IN MOTOR NEURON DISEASE
MIOSHI E1,2, LILLO P1, KIERNAN M1, HODGES J1,2
1Neuroscience Research Australia, Sydney, NSW, Australia, 2School of Medical Sciences, University of New South Wales, Sydney, NSW, Australia
Email address for correspondence: [email protected]
Keywords: activities of daily living, behaviour, ALSFRS-R
Background: The nature of impairment in activities of daily living (ADLs) in motor neuron disease (MND) has been under investigated to date. Although ADL dependency is pervasive and present early in the disease, the main factors behind such disability have not being explored to date.
Objectives: The study aimed to investigate the contributions of behavioural (as measured by the Cambridge Behavioural Inventory Revised, CBI-R) and bulbar, respiratory and motor factors (as measured by the Amyotrophic Lateral Sclerosis Functional Rating Scale, ALSFRS-R) on ADL impairment.
Methods: A postal survey in New South Wales, Australia, included assessments of ADL, behavioural change (CBI-R, carer-based) and MND severity (ALSFRS-R).
Results: 82 patients were subdivided into bulbar (n = 23) and limb (n = 59) onset presentations. There were significant differences on ADL performance between limb and bulbar onset depending on ADL task. ADL disability correlated strongly with ALSFRS-R. More importantly, 57% of the variance on ADL scores was explained by a model combining behavioural and motor factors.
Discussion: Although primarily a condition affecting the motor system, MND also leads to changes in behaviour that, in turn, can also affect ADL performance.
Conclusions: This study confirms the progressive disabling nature of MND, which is strongly associated with disease severity and shows qualitative differences depending on onset presentation. Importantly, ADL disability is not only dependent on motor factors but also behavioural ones. These findings have clear implications for clinical intervention.
C67 PERSONA PROJECT - PERSONALITY CHANGES IN LATE STAGES OF ALS: AGGRESSIVENESS, SEXUALITY AND OBSESSIONS
MARCONI A1, FOSSATI F1, ROSSI G1, LUNETTA C1, BASTIANELLO S1,2, PAGNINI F3, BANFI P1, MELAZZINI M1,2, CORBO M1
1NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Milan, Italy, 2AISLA - Italian Association of Amyotrophic Lateral Sclerosis, Milan, Italy, 3Department of Psychology, Catholic University, Milan, Italy
Email address for correspondence: [email protected]
Keywords: personality assessment, aggressive behaviors, sexuality
Background: Scientific literature describes ALS patients as calm and pleasant, with a polite and accommodating attitude. However, studies about this topic are few and refer to early or middle stages of illness. Clinical evaluations suggest that, in late stages of illness, usually after tracheostomy, some patients may present a change of personality traits, in particular aggressive behaviors, obsessions and attitude toward sexuality. This clinical hypothesis has never been investigated.
Objective: The aim of this pilot study is to evaluate if personality traits of ALS patients during the late stages of illness, after tracheostomy, present significant changes related to aggressiveness, obsessions and attitude toward sexuality.
Method: We recruited 15 ALS patients in the late stages of illness and with invasive mechanical ventilation along with their caregivers. ALS patients were assessed with the Hospital Anxiety and Depression Scale, for the evaluation of anxiety and depression, and with the Big Five Questionnaire, a widely used questionnaire about personality traits. Caregivers underwent a semi-structured interview about the patient's personality changes and behaviors.
Results: From the primary analysis conducted personality trait seem to increase after tracheostomy, in particular patients seem to be more obsessive than before the intervention. Qualitative investigation shows that obsessive attitude of patients might affect caregivers well-being and care-burden.
Discussion and conclusion: The first data confirm our hypotheses, from quantitative analysis we might obtain further confirmation and a complete personality profile of ALS patients in late stages. The personality of ALS patients today is not investigated enough, our results could be the basis for further studies. The results will be entirely presented during the symposium.
C68 THE RANGE AND CLINICAL IMPACT OF COGNITIVE IMPAIRMENT IN FRENCH PATIENTS WITH ALS: A CROSS-SECTIONAL STUDY OF NEUROPSYCHOLOGICAL TEST PERFORMANCE
GORDON P1, DELGADILLO D1, PIQUARD A2, BRUNETEAU G1, PRADAT P-F1, SALACHAS F1, LENGLET T1, PAYAN C3, MEININGER V1,4, LACOMBLEZ L1,3
1Fédération des Maladies du Système Nerveux, Centre Référent Maladies Rares SLA, Hôpital de la Pitié-Salpêtrière, Paris, France, 2Université Versailles Saint Quentin, Laboratoire Santé-Environnement-Vieillissement EA 2506 (AP), AP-HP, Hôpital Sainte Périne, Paris, France, 3Department of Pharmacology AP-HP, Hôpital de la Pitié-Salpêtrière, Paris, France, 4Université Pierre et Marie Curie, Paris VI, Paris, France
Email address for correspondence: [email protected]
Keywords: cognitive impairment, frontotemporal dementia, neuropsychological assessment
Background: Amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD) overlap clinically and pathologically in some patients. There appears to be a range of cognitive changes that occur in ALS, from mild frontal syndromes to overt dementia. The prevalence of impairment in French-speaking patients with ALS has not been examined previously.
Objective: To assess the spectrum and clinical associations of cognitive impairment in French patients with ALS, and determine the effect of cognitive impairment on survival in this population.
Methods: One hundred and thirty-one patients were enrolled in a cross-sectional cohort study of neuropsychological test performance The test battery consisted of measures assessing the following domains: memory (Grober and Buschke verbal episodic memory test; Digit Span and Rey figure recall); language (DO80 naming test); executive function (Stroop test, GoNoGo test; Trail Making Test; verbal fluency; similarities (WAIS III and Wisconsin Card Sorting Test); visual construction (Rey Figure).
ANOVA and chi-square tests assessed differences in clinical characteristics between impaired and unimpaired patients; multiple regression determined which features contributed most strongly to cognitive status; and Cox models compared survival.
Results: Fifty-three patients (40%) were categorized as cognitively impaired based on test performance. Thirteen (10%) patients had frontotemporal dementia (FTD) clinically; all scored in the moderate to severely impaired range on testing. Impaired patients had less education (p = 0.001), and severely impaired patients were more likely to have bulbar-onset than unimpaired patients (p < 0.001). Severe cognitive impairment predicted shorter survival (p = 0.007), even when controlled for motor severity (p = 0.001).
Conclusions: Ten percent of a consecutive series of French ALS patients had overt dementia and 40% were cognitively impaired by neuropsychological testing. Lower education level and possibly bulbar-onset alS were associated with impairment. As in other causes of dementia, higher educational attainment may protect against clinical cognitive deterioration in ALS. French patients with severe cognitive impairment have shorter survival time.