[Stark-Kaeser type of chronic scapulo-peroneal amyotrophy. Apropos of 10 cases].
The authors described 10 cases of scapulo-peroneal amyotrophy of spinal origin of the Stark-Kaeser type. The main characteristics are a frequently dominant autosomal heredity, onset in the muscles of the legs, scapulp-peroneal weakness and amyotrophy with extension sometimes to the bulbar muscles, areflexia without sensory disturbances, an electromyogram of the neurogenic type with a normal conduction rate, neurogenic histological aspects with normal peripheral nerve. It does not usually result in severe disability. This syndrome is related to the scapulo-peroneal or facio-scapulo-peroneal myopathies and the scapulo-peroneal neuropathies. The problem of the boundaries between these disorders and their association does not alter the fact that the concept of spinal amyotrophy of the Stark-Kaeser type should be kept as a reference group.
D000328:Adult; D000368:Aged; D004576:Electromyography; D005260:Female; D006801:Humans; D007866:Leg; D008297:Male; D009132:Muscles / Q000473:pathology; D009133:Muscular Atrophy / Q000235:genetics*; D012021:Reflex, Abnormal / Q000235:genetics; D014059:Tongue
Amyotrotrophies scapulo-péronières chroniques de type Stark-Kaeser. (A propos de 10 observations)