200 case histories were analyzed (108 males and 92 females). 55 patients were under the author’s personal observation, 145 cases were taken from the literature. There were 163 hereditary and 37 ‘sporadic’ cases. Both the patterns of distribution of muscular atrophies in different phases of the disorder and the changes in their extension throughout the whole course of the disease process were studied. According to the data on the clinical manifestation and genetics, the facioscapulohumeral type can be considered as an independent form. The best name for it is ‘facio-scapulo-limb (FSL) muscular dystrophy’, adding the eponym ‘Duchenne-Lan-douzy-Déjerine’. There are two varieties of the extension of muscular atrophies in the FSL type: the gradually descending variety and the more frequent descending one, characterized by a ‘jump’ from the upper part of the body to the peroneal group of the shin muscles (the descending variety with a ‘jump’). Five clinical syndromes were distinguished, representing various phases in the two varieties of FSL muscular dystrophy. In particular, the facio-scapulo-(humero)-peroneal syndrome may be the principal stage in the disease process. The findings confirm the existence of the autosomal-dominant type of inheritance of FSL muscular dystrophy with complete penetrance and variable expressivity of the gene. Genealogical analysis of 62 families suggests the existence of a clinical and genetic heterogenei
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