[The respiratory function in muscular dystrophies (author's transl)]

The principal cause of death in Duchenne muscular dystrophy (DMD) results from a respiratory involvement, classically defined as a restrictive syndrome. The respiratory function in muscular dystrophies (MD) was studied using spirometer respiratory function tests on 190 patients, classified into five distinct groups, according to accurate clinical and genetic findings. The three adult types of MD (Becker type, limb girdle type, facio-scapulo-humeral type) are characterized by pulmonary function approaching normal. DMD is always characterized by a restrictive syndrome. A high significant difference between adult and DMD types is confirmed by statistical analysis. In DMD, vital risks are variable according to variations of the level of absolute value of vital capacity. It is useful to distinguish three types in DMD: major involvement or type I, severe involvement or type II, moderate or mild involvement or type III.