Abstract
We evaluated progression in the functional disability of extremities in 20 patients with myotonic dystrophy for 10 years. Tentative functional classification were made for eight stages for locomotion ability, four stages for standing-up ability, and four stages for skillfulness of hands (N=16), respectively. The mean stage valuses in locomotion ability test were 2.7±1.7 (M±SD) at the first assessment and 5.7±2.1 at the last, in standing-up ability 2.0±1.1 at the first and 3.3±1.1 at the last, and in skillfulness of hands 1.4±0.8 at the first and 1.9±1.1 at the last assessment. The statistical analysis showed significant decline of the stages in each functional category by Wilcoxon's test (p=0.000 in each category). The progressions of disability of locomotion and standing-up were observed in most cases, and 40-45% of worsening causes were bone f ructure and poor general condition. Eleven out of 16 cases showed no progression of skillfulness of hands stage. In following-up of those patients with myotonic dystrophy, prevention of bone f ructure and complications, and preservation of upper extremity functions are necessary for their QOL
