Premium
Limb girdle muscular dystrophy: Weakness and disease duration as predictors of functional impairment
Author(s) -
Stübgen JoergPatrick,
Lahouter Annette
Publication year - 1994
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880170806
Subject(s) - weakness , medicine , muscle weakness , physical medicine and rehabilitation , muscular dystrophy , functional testing , functional impairment , physical therapy , limb girdle muscular dystrophy , surgery , medline , biology , biochemistry , gene , phenotype
Abstract This cross‐sectional study compared progressive weakness to functional disability in 20 patients with limb girdle muscular dystrophy. Weakness (assessed by manual muscle testing) determined disability (assessed by functional grades, timed functional tests, and an activities of daily living scale) For any functional grade the degree of weakness varied between patients. Yet, at a certain “threshold” weakness the functional level predictably deteriorated. Change in functional grade did not reflect a parallel increase in weakness nor disease progression. Functional “milestones” were reached at varying durations of disease. Only guidelines were offered to individual patients as to the time course of progression in disability. Serial timed tests should be a sensitive, objective method to follow the rate of disease progression. Timely referral of patients to establish physical needs should delay functional deterioration and minimize handicap despite progressive weakness. © 1994 John Wiley & Sons, Inc.