Strength and functional performance of patients with limb-girdle muscular dystrophy

This study was design to reach a better understanding of muscle strength, motor function and activity of daily living in patients of limb-girdle muscular dystrophy. Forty-eight patients diagnosed as cases of limb-girdle muscular dystrophy were included in this study. Manual muscle testing was used to evaluate muscle strength. The Brooke and Vignos scales were used to grade upper and lower extremities function, respectively, and the ability of daily living activity was measured by Barthel index. Our patients showed progressive symmetrical limb-girdle muscular weakness. Upon regression analysis we found that mean muscle strength was inversely related to disease duration (years) as follows: mean muscle strength = 0.6052 + (0.6309/disease duration). According to the Brooke functional scale, 89.6% of our patients were graded as 1-3 and 10.4% were graded as 5. On the Vignos functional scale, 79.1% of patients fell into the grades 1-5, one person (2.1%) in grade 6 and 18.8% in grade 9 category. The average Barthel index was 85.3 +/- 20.7. Mean muscle strength was significantly correlated with the average Barthel, Vignos and Brooke functional scales. Our study could offer the strength and functional performance of limb-girdle muscular dystrophy on natural history. The muscle strength declined in Taiwanese patients of limb-girdle muscular dystrophy in a typical pattern. Regression analysis showed that the strength was inversely related to disease duration. These findings demonstrate that most of our patients suffered from mild or moderate physical disability.