المؤلفون: Cornwall KM; Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT, USA., Butterfield RJ; Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT, USA.; Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA., Hernandez A; Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT, USA., Heatwole C; Department of Neurology, University of Rochester Medical Center School of Medicine and Dentistry, Rochester, NY, USA., Johnson NE; Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT, USA.; Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA.

المصدر: Journal of neuromuscular diseases [J Neuromuscul Dis] 2018; Vol. 5 (2), pp. 251-255.

نوع المنشور: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: IOS Press Country of Publication: Netherlands NLM ID: 101649948 Publication Model: Print Cited Medium: Print ISSN: 2214-3599 (Print) NLM ISO Abbreviation: J Neuromuscul Dis Subsets: MEDLINE

مواضيع طبية MeSH: Health Status* , Mental Health* , Quality of Life* , Social Participation*, Contracture/*physiopathology , Muscular Dystrophies/*congenital , Sclerosis/*physiopathology, Adolescent ; Adult ; Child ; Child, Preschool ; Contracture/psychology ; Female ; Humans ; Male ; Middle Aged ; Muscular Dystrophies/physiopathology ; Muscular Dystrophies/psychology ; Parents ; Qualitative Research ; Role ; Sclerosis/psychology ; Young Adult

مستخلص: Congenital muscular dystrophies (CMD) cause progressive muscle weakness resulting in severe motor disabilities. Previous studies focused on the effects of motor disability. Here, we explore other factors affecting health related quality-of-life (HRQOL) in CMD. Qualitative interviews were conducted with participant-parent dyads to identify symptoms having the greatest impact on HRQOL. Symptoms were classified into themes and domains representing physical, mental, social health, and disease specific issues. Social role limitations and specific activity impairment were frequently mentioned. A greater understanding of symptoms impacting HRQOL will provide a framework for improved clinical care and patient centered outcomes as new therapies are developed.

SCR Disease Name: Bethlem myopathy; Muscular dystrophy congenital, merosin negative; Scleroatonic muscular dystrophy

المؤلفون: Van Heugten C; School for Mental Health and Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands.; Department of Neuropsychology and Psychopharmacology, Maastricht University, Maastricht, The Netherlands., Meuleman S; Merem Rehabilitation Center De Trappenberg, Almere, The Netherlands., Hellebrekers D; School for Mental Health and Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands., Kruitwagen-van Reenen E; Center of Excellence in Rehabilitation Medicine, Brain Center Rudolf Magnus, University Medical Center Utrecht, and De Hoogstraat Rehabilitation, Utrecht, The Netherlands., Visser-Meily J; Center of Excellence in Rehabilitation Medicine, Brain Center Rudolf Magnus, University Medical Center Utrecht, and De Hoogstraat Rehabilitation, Utrecht, The Netherlands.

المصدر: Journal of neuromuscular diseases [J Neuromuscul Dis] 2018; Vol. 5 (2), pp. 205-214.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: IOS Press Country of Publication: Netherlands NLM ID: 101649948 Publication Model: Print Cited Medium: Print ISSN: 2214-3599 (Print) NLM ISO Abbreviation: J Neuromuscul Dis Subsets: MEDLINE

مواضيع طبية MeSH: Activities of Daily Living* , Apathy* , Family* , Personal Satisfaction*, Fatigue/*psychology , Myotonic Dystrophy/*psychology , Social Participation/*psychology, Adult ; Age Factors ; Aged ; Cross-Sectional Studies ; Emotions ; Fatigue/physiopathology ; Female ; Household Work ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Myotonic Dystrophy/physiopathology ; Netherlands ; Sports

مستخلص: Background: Myotonic Dystrophy type 1 (DM1) is primarily a neuromuscular disease but can also have neuropsychological consequences (i.e.cognitive, emotional and behavioural) which can influence daily living and societal participation. Not much is known about the level of participation of DM1 patients and their relatives and the factors influencing participation. This information can guide the development of rehabilitation programs and identify those at risk of long-term participation problems.
Objective: To investigate the level of participation of DM1 patients and their partners and the determinants.
Methods: Cross-sectional study using the Utrecht Scale for Evaluation of Rehabilitation-Participation. Determinants were demographic, disease related, and neuropsychological characteristics. Spearman correlations and backward multiple regression analyses were performed.
Results: Of the 66 DM1 participants, 54% reported severe fatigue and 47% apathy. Experienced participation restrictions were most prevalent in housekeeping (74%), sports (68%) and outdoor activities (55%). Participants were relatively satisfied with their level of participation, but dissatisfaction occurred mostly in housekeeping (58%), outdoor activities (55%), and sports (54%). Age and apathy were significant predictors of frequency of (R2 = 10.4) and restrictions in participation in the multivariate model (R2 = 14.4). Emotional functioning was the only significant predictor of satisfaction with participation (R2 = 23.8).
Conclusions: A considerable number of DM1 patients have Central Nervous System-related problems such as fatigue and apathy in addition to neuromuscular restrictions. Risk factors for lower participation are age, apathy, and emotional problems. Physical as well as neuropsychological rehabilitation programs are recommended.

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المؤلفون: Johanna M A Visser-Meily, Caroline M. van Heugten, Susan Meuleman, Esther T. Kruitwagen-van Reenen, Danique M. J. Hellebrekers

المساهمون: Section Neuropsychology, Psychiatrie & Neuropsychologie, RS: MHeNs - R1 - Cognitive Neuropsychiatry and Clinical Neuroscience, RS: FPN NPPP I, Promovendi MHN, Klinische Neurowetenschappen

المصدر: Journal of neuromuscular diseases, 5(2), 205-214. I O S Press

مصطلحات موضوعية: Adult, Male, 030506 rehabilitation, Activities of daily living, Neuromuscular disease, medicine.medical_treatment, Apathy, Emotions, Personal Satisfaction, Disease, 03 medical and health sciences, 0302 clinical medicine, Activities of Daily Living, Journal Article, medicine, Humans, Myotonic Dystrophy, Family, Fatigue, Aged, Netherlands, Rehabilitation, business.industry, Age Factors, Neuropsychology, Middle Aged, Social Participation, medicine.disease, Social engagement, Household Work, Cross-Sectional Studies, Neurology, Housekeeping, Multivariate Analysis, Female, Neurology (clinical), medicine.symptom, 0305 other medical science, business, 030217 neurology & neurosurgery, Sports, Clinical psychology

الوصف: BACKGROUND: Myotonic Dystrophy type 1 (DM1) is primarily a neuromuscular disease but can also have neuropsychological consequences (i.e.cognitive, emotional and behavioural) which can influence daily living and societal participation. Not much is known about the level of participation of DM1 patients and their relatives and the factors influencing participation. This information can guide the development of rehabilitation programs and identify those at risk of long-term participation problems.OBJECTIVE: To investigate the level of participation of DM1 patients and their partners and the determinants.METHODS: Cross-sectional study using the Utrecht Scale for Evaluation of Rehabilitation-Participation. Determinants were demographic, disease related, and neuropsychological characteristics. Spearman correlations and backward multiple regression analyses were performed.RESULTS: Of the 66 DM1 participants, 54% reported severe fatigue and 47% apathy. Experienced participation restrictions were most prevalent in housekeeping (74%), sports (68%) and outdoor activities (55%). Participants were relatively satisfied with their level of participation, but dissatisfaction occurred mostly in housekeeping (58%), outdoor activities (55%), and sports (54%). Age and apathy were significant predictors of frequency of (R2 = 10.4) and restrictions in participation in the multivariate model (R2 = 14.4). Emotional functioning was the only significant predictor of satisfaction with participation (R2 = 23.8).CONCLUSIONS: A considerable number of DM1 patients have Central Nervous System-related problems such as fatigue and apathy in addition to neuromuscular restrictions. Risk factors for lower participation are age, apathy, and emotional problems. Physical as well as neuropsychological rehabilitation programs are recommended.

وصف الملف: application/pdf

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b5c92dc59eaa68e6ed5b090485f284d7
https://doi.org/10.3233/jnd-170246

المؤلفون: Chad Heatwole, Kylie M. Cornwall, Russell J. Butterfield, Nicholas E. Johnson, Antonio Hernandez

المصدر: J Neuromuscul Dis

مصطلحات موضوعية: Adult, Male, Parents, Contracture, Adolescent, Ullrich congenital muscular dystrophy, Health Status, Muscular Dystrophies, Article, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Quality of life (healthcare), medicine, Humans, 030212 general & internal medicine, Social determinants of health, Muscular dystrophy, Child, Qualitative Research, Health related quality of life, Sclerosis, business.industry, Patient-centered outcomes, Role, Bethlem myopathy, Middle Aged, Social Participation, medicine.disease, Mental Health, Neurology, Child, Preschool, Quality of Life, Congenital muscular dystrophy, Female, Neurology (clinical), business, 030217 neurology & neurosurgery, Clinical psychology

الوصف: Congenital muscular dystrophies (CMD) cause progressive muscle weakness resulting in severe motor disabilities. Previous studies focused on the effects of motor disability. Here, we explore other factors affecting health related quality-of-life (HRQOL) in CMD. Qualitative interviews were conducted with participant-parent dyads to identify symptoms having the greatest impact on HRQOL. Symptoms were classified into themes and domains representing physical, mental, social health, and disease specific issues. Social role limitations and specific activity impairment were frequently mentioned. A greater understanding of symptoms impacting HRQOL will provide a framework for improved clinical care and patient centered outcomes as new therapies are developed.

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cf6c24068ec81c70e4230b28c0621fae
https://doi.org/10.3233/jnd-170252