The strength and functional performance in patients with facioscapulohumeral muscular dystrophy.

Abstract

Facioscapulohumeral muscular dystrophy (FSHD) is a slowly progressive myopathy with autosomal dominant inheritance remarkable for its early involvement of facial musculature. The purpose of our study was to assess the rate of strength deterioration, functional condition and performance of activity of daily living of patients with FSHD in Taiwan. Twenty patients diagnosed with FSHD were included in this study. Manual muscle testing (MMT) was used to evaluate muscle strength. The Brooke and Vignos scales were used to assess upper and lower extremity function respectively, and the capability of the activity of daily living was measured by Barthel index. The result of the strength testing was characterized by the presence of a progressive asymmetrical muscular weakness in patients with FSHD. The mean muscular strength of the right extremity was weaker than its left counterparts (p < 0.05) and the shoulder muscle group was the weakest. According to the Brooke functional scale, 20% of our patients were graded as 1, 30% as grade 2, and 50% as grade 3. On the Vignos functional scale, 50% of patients fell into grade 1, 10% in grade 2, and 40% in grades 3-5. Vignos scale was significantly correlated with mean muscle strength (p < 0.05). The average value of Barthel index was 97.8 +/- 4.7. The muscle strength decline in this Taiwanese of FSHD population was more severe in shoulder girdle area. The mean muscle strength of the right extremity was weaker than the left. Most of our patients suffered from mild or moderate physical disability. Finding of these Taiwanese FSHD population is similar to those reported elsewhere in the world.