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Amyotrophic Lateral Sclerosis (ALS) is the most common motor neuron disease MND) among adults. Motor neurons in the spinal cord, brain stem, and cerebral motor cortex degenerate and create a variety of upper (UMN) and lower motor neuron (LMN) clinical signs and symptoms, with the most frequently presenting symptom being focal weakness beginning in the leg, arm, or bulbar muscles, occurring in more than 70% of patients. Despite the high incidence of muscle weakness in patients with ALS, only two case studies evaluating the effects of specific muscle strengthening and endurance exercise programs in this patient population have been published, and the effects of resistive exercise programs in patients with ALS have not been well studied. Some have discouraged exercise programs in patients with ALS because of fear of overuse weakness. Yet, in patients with other neuromuscular diseases, resistive exercise programs have been shown to be beneficial and have not produced overuse weakness. The purpose of this study is to determine the effects of six months strengthening program on strength, function, fatigue and quality of life in individuals with ALS.
The specific aims of this preliminary, prospective randomized study of individuals with ALS are to determine variability, describe trends over a 6-month time frame and to estimate differences between individuals with ALS who perform an upper and lower extremity strengthening program three times per week and a control group of individuals with ALS with respect to the following outcomes:
1. an increase in muscle strength (maximum voluntary isometric contraction) as measured by quantitative muscle testing.
2. an increase in functional activities as measured by The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS)7 and the Schwab and England Rating Scale (SERS).
3. a decrease in fatigue as measured by the Fatigue Severity Scale.
4. an increase in quality of life as measured by the SF-36.Subjects will be randomized into two groups - experimental and control. Subjects in the control group will perform a daily stretching exercise program for a period of six months. Subjects in the experimental groups will perform a strengthening exercise program three times per week for a period of six months. All subjects will be evaluated at baseline and then monthly using the following outcome measures: (1) maximum voluntary isometric contraction; (2) Amyotrophic Lateral Sclerosis Functional Rating Scale; (3) Schwab and England Rating Scale; (3) Fatigue Severity Scale; (4) forced vital capacity; and, (5) the SF-36.
Allocation: Randomized, Control: Active Control, Endpoint Classification: Safety/Efficacy Study, Intervention Model: Parallel Assignment, Masking: Single Blind, Primary Purpose: Treatment
Amyotrophic Lateral Sclerosis
Cleveland Clinic Foundation
University of Saskatchewan
Published on BioPortfolio: 2014-08-27T03:49:35-0400
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Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)
A Poly(A) RNA-binding protein that negatively regulates EGFR ENDOCYTOSIS. An increased risk for developing AMYOTROPHIC LATERAL SCLEROSIS 13 is observed in patients who have more than 23 CAG repeats in the ATXN2 gene coding sequence. Larger CAG expansions in the ATXN2 gene occur in SPINOCEREBELLAR ATAXIA 2 patients.
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