Isabella Morrone

FACTORS RELATING TO CARER BURDEN FOR FAMILIES OF PERSONS WITH MUSCULAR DYSTROPHY

OBJECTIVE To assess the burden on family carers of persons with muscular dystrophy living in their homes and to determine factors contributing to carer burden. METHODS The study included 56 dyads of people with muscular dystrophy and their family carers. The variables for carer burden were compared by logistic regression in 2 carer groups (burden + /burden-). RESULTS The mean age of the patients with muscular dystrophy was 32.7 years (median 26.7, range 15-65 years) and that of the carers 51 years (median 48, range 30-80 years). The carers reported the care burden using the Zarit Burden Inventory (median score 23, range 0-57/88). Multivariate analysis produced 3 adjusted explicative factors: carer characteristics related to risk of perceived burden are self-report of poor social functioning on the SF-36 (OR = 26.6 (2.6-278); p=0.006), self report of anxiety on the Hospital Anxiety Scale (OR = 7.1 (1.4-36); p=0.02) and being a carer under 48 years of age (OR = 7.8 (1.7-34.5); p=0.007). However, it was difficult to dissociate the different health variables of the carers from each other. CONCLUSION This approach should lead to better decision-making by medical teams, patients and their carers.

Transcultural adaptation and psychometric validation of a French-language version of the QoL-AD

Objectives: The aim of this study was to assess the psychometric properties and the transcultural adaptation into French of the quality-of-life in Alzheimers disease (QoL-AD). Methods: A total of 120 patient-proxy pairs were recruited in six French hospitals and one French-speaking Swiss hospital to take part in the study. The patients presented mild to moderate AD (MMSE ≥ 10). The QoL-AD was administered by an interviewer for patients and was self-administered for caregiver proxies. Thus, the psychometric validation enabled the study of feasibility, acceptability, reliability and convergent and discriminant validity of the instrument. Results: The French version of the QoL-AD showed good internal consistency (Cronbachs α coefficient ≥ 0.70) and good reliability (Intra-class correlation > 0.80) at a 2-week interval, for patient and caregiver questionnaires. Convergent validity, as indicated by correlation between the QoL-AD (disease-specific instrument) and the dimensions of the Duke Health Profile (generic instrument), was also good for eight dimensions in the Duke profile (p < 0.05). Discriminant validity showed a significant difference for depression (p = 0.0025) and did not show significant difference for two groups of dementia (p = 0.11). Conclusion: There is now a validated French version of the QoL-AD available, following transcultural adaptation according to international recommendations, which possesses good psychometric qualities.

Health status in patients with Alzheimer's disease: An investigation of inter-rater agreement

Objective: The purpose of the present study was to examine the level of agreement between health status ratings provided by patients with Alzheimer’s disease and by their proxies. Background: Because proxy-completed responses are often necessary in assessing health outcomes for the elderly, it is necessary to determine the feasibility and potential limitations of using proxies as a patient substitutes. Methods: To assess the potential utility of proxy responses on health status when subjects present a cognitive impairment, this study compared the responses of 70 subjects with Alzheimer‘s disease and those of their family and/or care provider proxy using the SF-36. Agreement between proxies and patients was measured by intraclass correlation coefficients (ICCs). Results: The proportion of exact agreement between patients and proxies on the 36 items ranged from 3.3 to 41.7%. Results reveal poor to moderate agreement between patient and proxy reports. Proxy reliability varied according to the relationship of the proxy to the index subject. Agreement decreased significantly with increasing severity of dementia and with increasing severity of Physical status (Katz ADL). Agreement was better for measures of functions that are directly observable and relatively poor for more subjective measures. Conclusions: Our results confirm the importance of the information source used for patient health status.

Predictors of rapid cognitive decline among demented subjects aged 75 or more: ('Sujet Agé Fragile--Evaluation et Suivi' Cohort-SAFES).

To identify factors predictive of rapid cognitive decline (RCD) among elderly subjects aged 75 or over suffering from dementia.

Aging and inhibition processes: the case of metaphor treatment.

The inhibitory deficit hypothesis has often been cited as a possible explanation for cognitive changes related to age. The aim of this study was to develop a new procedure for evaluating effortful inhibition on the basis of the comprehension of metaphors. Our experiment was carried out on younger and older adults, in whom we also measured inhibitory capacity, working memory, and processing speed. The results show that older participants required a longer time and made more frequent errors in rejecting metaphors versus literally false statements. The interference effect was predicted by the psychometric tests designed to evaluate inhibition.

Comparison of QoL-AD and DQoL in elderly with Alzheimer's disease

Objective: The Dementia Quality of Life (DQoL) and the Quality of Life in Alzheimers Disease (QoL-AD) are the two most widely used dementia-specific QoL instruments in the world. We aimed to compare the psychometric properties of these two instruments and identify which is most adapted to use in geriatric consultations. Methods: To evaluate the psychometric properties of the French language validation of DQoL and QoL-AD, 123 patients aged 65 years and over suffering from AD (Mini Mental State Examination score ≥10) were recruited in seven French hospitals and one Switzerland hospital. The DQoL comprises 29 items, ranked on a five-point Likert scale and measuring five QoL domains: self-esteem, positive affect, negative affect, feeling of belonging and sense of aesthetics. The QoL-AD contains 13 items giving an overall score ranging from 13 to 52 and evaluating the domains of interpersonal relationships, financial difficulties, physical condition, memory, mood and overall health. Results: Both questionnaires showed adequate reproducibility at 2 weeks interval (intra-class correlation coefficient >0.80), good internal consistency (Cronbachs alpha coefficient >0.70) and good convergent validity with the general health dimension of the Duke Health Profile. The time required to complete the QoL-AD was significantly shorter (p < 0.0001). DQoL had better discriminant capacity (with at least one dimension significant for each subgroup of severity of cognitive decline, dependency, presence of depression or behavioural disorders). Conclusions: For quick evaluation of QoL during consultations in geriatric care, the QoL-AD is preferable, whereas for the purposes of research and more in-depth evaluation, the DQoL is more suitable.

Qualité de vie et démence : état des connaissances

BACKGROUND Dementia is a chronic and evolutive disease, for which no curative treatment exists. Evaluating the quality of life of patients suffering from dementia is therefore an important component of management. AIMS To summarise existing literature regarding quality of life instruments specific to dementia, and to identify factors associated with quality of life. METHODS We analysed studies published between November 1996 and December 2010 and referenced in PubMed, and focusing on the development and/or validation of quality of life instruments specific to dementia, or studies having used any such instrument to evaluate a therapeutic intervention. RESULTS Nine quality of life instruments were identified. Place of residence and level of education do not appear to play any significant role. Results were conflicting for age and sex. There appears to be a relation, albeit a weak one, with cognitive function. The factors most frequently associated with lower quality of life were behavioural disorders, dependence, and caregiver burden. Quality of life of patients under pharmacological treatment is poorly documented, while occupational therapy and cognitive stimulation appear to have moderate positive effects.

Predictive Factors of Rapid Cognitive Decline in Patients with Alzheimer Disease

Aim: To determine predictive factors associated with rapid cognitive decline (RCD) in elderly patients suffering from Alzheimer disease (AD). Methods: Patients suffering from mild to moderate AD were included. RCD was defined as the loss of at least 3 points on the Mini-Mental State Examination (MMSE) over 12 months. Factors associated with RCD were identified by logistic regression. Results: Among 123 patients included, 61 were followed up until 12 months. RCD occurred in 46% of patients (n = 28). Polymedication (p < 0.0001), the fact that the caregiver was the child or spouse of the patient (p < 0.0001) and autonomy for washing (p < 0.0001) were protective factors against RCD, while the presence of caregiver burden (p < 0.0001) was shown to be a risk factor for RCD. Conclusion: Early detection of the RCD risk in AD patients could make it possible to anticipate the patient’s medical needs and adjust the care plan for caregiver burden.


ATYPICAL CASE OF ALZHEIMER'S DISEASE MIMICKING CREUTZFELDT-JAKOB DISEASE: INTEREST OF CEREBROSPINAL FLUID BIOMARKERS IN THE DIFFERENTIAL DIAGNOSIS

ACKNOWLEDGMENTS Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Author Contributions: Ryuji Sakakibara: study concept and design, acquisition of subjects and data, analysis and interpretation of data, preparation of manuscript. Masahiko Kishi: acquisition of subjects and data, analysis and interpretation of data. Emina Ogawa, Fuyuki Tateno, Tsuyoshi Ogata, Hiroyuki Haruta, Yasuo Matsuzawa, and Kohji Shirai: acquisition of subjects and data. Kohji Shirai: supervision of manuscript. Sponsor’s Role: None.

Exploring quality of life in people with slowly-progressive neuromuscular disease

Abstract Purpose: A qualitative work is conducted to enable later the construction of a health-related quality of life (HRQL) questionnaire for patients with slowly-progressive neuromuscular disease (NMD) such as myopathies and muscular dystrophies. Methods: The formation of focus groups is an efficient method to perform an in-depth exploration of the aspects of HRQL potentially impaired by NMD. Patients were recruited in France by 4 NMD reference centers. To ensure adequate representativeness in terms of severity, three types of focus groups were formed: (1) Patients able to walk (WP). (2) Patients using a wheelchair (WCP). (3) Patients using a wheelchair and requiring continuous mechanical ventilation (WCMVP). All verbal interactions among group participants were recorded. A qualitative analysis of the verbatim was performed using the framework of the International Classification of Functioning, Disability and Health model (ICF). Results: A total of 41 patients distributed across five focus groups were interviewed. The verbatim provided 2424 ICF categories. The percentages of mentions of the different ICF categories were calculated and graphically displayed. Conclusion: The results enabled to identify and quantify the aspects of life that are altered by NMD according to patients. This qualitative work was the first phase of a more ambitious project to develop a new NMD-specific HRQL questionnaire. Implication of rehabilitation Patients with NMD have more to say about the quality of their environment, their social relationships and their psychological state than about their physical symptoms. This observation should be compared to clinician perceptions which often focus mainly on the physical symptoms, overlooking those elements that they cannot assess directly. Many patients reported relationship issues with various people from their surrounding (e.g., health professionals, acquaintances, colleagues, and strangers). In particular, it is essential that health professionals are careful not to make adult patients with NMD feel infantilized. This issue can be addressed by making all relevant medical information available and asking for the patients opinion on any important change in their medical care.