[Progressive supranuclear palsy: description of a case of early occurrence associated with muscular atrophies (author's transl)].

A case of Progressive Supranuclear Palsy (P.S.P.) of early onset (38 years) is described, with somewhat atypical features: the patient was oligophrenic, had no pseudobulbar signs, but there was some muscular atrophy, particularly of the shoulders girdle. On examination there was a slight cortical and cerebellar atrophy on the air encephalogram. Electromyography showed signs of damage to the lower motor neurone which was confirmed by muscular biopsy. In the literature a muscular atrophy in association with P.S.P. is rarely reported clinically although in some cases studied histologically there was degeneration in the nuclei of the cranial and spinal motor nerves. The present case may be an example of such widespread degeneration.