Profiles of neuromuscular diseases. Becker's muscular dystrophy.

Fifteen subjects with Becker's muscular dystrophy (BMD) were studied prospectively over a 10-yr period to provide a profile of impairment and disability. Proximal lower extremity musculature (particularly the hip and knee extensors), ankle dorsiflexors, and neck flexors showed significantly early weakness. Extensor muscle groups were weaker than flexor muscles at the elbow and knee. The mean manual muscle test (MMT) strength grade for all muscle groups combined was 3.7 +/- 0.8 MMT units. There was a slowly progressive decline in strength, only -0.31 MMT units per decade, and the decline was relatively equal in all muscle groups. There was not side dominance. Severe contractures did not appear to be a problem until after transition to a wheelchair, and scoliosis was rare. Restrictive lung disease occurred as a late complication in a small percentage of cases; however, maximal expiratory pressure was significantly reduced early in the disease. Only two individuals (19%) had severe restrictive lung disease and a history of significant respiratory complications. There was a slight but significant decline in forced vital capacity and maximal expiratory pressure with age and disease duration. The cardiomyopathy in BMD appeared to be disproportionately severe in some cases. Eleven individuals (73%) had abnormal electrocardiograms, and five (25%) had a history of significant cardiovascular complication, with no age or disease duration effect. Functional evaluations and timed motor performance tests showed only mild disability in most individuals. Mean intellectual and neuropsychologic function was within normal limits, but with a large variability in intelligence quotient scores. This report and others suggest a tremendous heterogeneity of severity among males with BMD.