[Clinico-morphologic characteristics of X-linked pseudohypertrophic myopathy].

A clinical, genealogical, EMG and morphological study of the development of pseudohypertrophic myopathy linked with X-chromosome allowed one to distinguish 2 variants: 1) the malignant (Duchenne form) and 2) benign (Baker form). Although both forms have similar clinical symptoms, the Baker form has a later onset, a more favourable development and more preserved motor functions. The activity of fructosodiphosphataldolase and creatinphosphokinase in the blood serum of these patients was significantly lower than in Duchenne' myopathy.