[Family with progressive myelopathic muscular atrophy with proximal distribution and onset in adulthood]

The Authors present two brothers suffering from proximal progressive muscular atrophy arising in adulthood and describe its clinical, bioptic and electromyographic characteristics. Electromyographic and bioptic examinations demonstrate the neurogenic nature of the amyotrophy and localize the causal lesion at the level of the anterior horns of the spinal medulla. With regard to the differential diagnosis, the nosographic position of the disorder in question is described, and the hypothesis advanced that it may represents the late onset variety of Wohlfart-Kugelberg-Welander disease.