Respiratory function in the muscular dystrophies

Abstract

The purpose of this investigation was to compare the respiratory function of patients with different clinical types of muscular dystrophy (MD). A total of 190 patients representing facioscapulohumeral MD (n = 20), limb-girdle MD (n = 50), Becker MD (n = 20), Duchenne MD (n = 90), and the "intermediate" type of MD (n = 10) were studied using simple spirometric respiratory function tests. The respiratory modifications observed in the 3 adult forms indicate that pulmonary function was almost normal. Respiratory function in Duchenne muscular dystrophy (DMD) was always characterized by a restrictive syndrome which severely impaired pulmonary function. The vital capacity (VC) underwent ascending, plateau, and descending phases during the course of the disease. The VC at the plateau stage may be used as an estimate of life span. The most interesting finding was the high degree of variability in the restrictive syndrome associated with DMD. In the DMD group 30% presented a severe restrictive respiratory syndrome associated with a life span of less than 20 years (Type 1), 40% had a serious restrictive syndrome and variable mortality (Type 2), and the remaining 30% had a moderate restrictive respiratory syndrome (Type 3).