Abstract
Objective: To assess the effects of an intensive, multidisciplinary rehabilitation program for patients with early to mid-stage Huntington’s disease. Design: A prospective intervention study. Setting: Two Norwegian inpatient rehabilitation centers. Subjects: 37 patients, with early- to midstage Huntington’s disease Interventions: A one year rehabilitation program, consisting of three admissions of three weeks each, and a five-day evaluation stay approximately 3 months after the last rehabilitation admission. Focus was on physical exercise, social activities, and group/teaching sessions. There was also emphasis to implement of coordinated health care and social services for the patients. Main outcome measures: standard measures for motor function, including gait and balance, cognitive function, including MMSE and UHDRS cognitive assessment, anxiety and depression, activities of daily living (ADL), health related quality of life and Body Mass Index (BMI). Results: Significant improvements were observed in gait function, balance, in physical quality of life, anxiety and depression, as well as in BMI. ADL-function remained stable with no significant decline. Only one cognitive measure (SDMT) showed significant decline, while no decline was observed for the remaining cognitive measures. Conclusion: A multidisciplinary intensive rehabilitation program in patients with early and mid stage HD is associated with improved balance, gait function, physical quality of life and with reduced depressive and anxiety symptoms. Longer follow-up is needed to assess if these positive effects are sustained. There should be emphasis to establishment of long term and coordinated health care services for the HD patient
Highlights
Huntington’s disease (HD) is a hereditary autosomal neurodegenerative disorder caused by an expanded Cytosine-AdenineGuanine (CAG) repeat in the HD gene 1
Significant improvements were observed in gait function, balance, in physical quality of life, anxiety and depression, as well as in Body Mass Index (BMI)
A multidisciplinary intensive rehabilitation program in patients with early and mid stage HD is associated with improved balance, gait function, physical quality of life and with reduced depressive and anxiety symptoms
Summary
Huntington’s disease (HD) is a hereditary autosomal neurodegenerative disorder caused by an expanded Cytosine-AdenineGuanine (CAG) repeat in the HD gene 1. The disease is characterized by motor disturbances, psychiatric symptoms and cognitive decline [2,3]. A clinical diagnosis of HD is typically made when an individual has overt motor symptoms and a family history of Huntington’s disease. Disease duration is commonly between [15,16,17,18,19,20] years, but symptom development and severity vary greatly between individuals. Motor symptoms are most visible and result in gait and balance problems. Cognitive and behavioral changes are known to frequently occur many years before clinical diagnosis[4,5] resulting in functional decline already early in the disease 6. Current treatment of persons with HD consists mainly of symptom management and improving quality of life 7. A multidisciplinary approach in the management of persons with HD is recommended 9
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