Juvenile type of distal and segmental muscular atrophy of upper extremities

摘要: Seventy-one cases of distal and segmental muscular atrophy the upper extremities with juvenile onset were studied. The clinical features consisted of: onset, male preponderance, unique distribution in hand forearm, tendon reflexes hypoactive most but hyperactive some, no definite sensory disturbances, involvement cranial nerves, autonomic nerve disorders affected region. There was rapid progression during 2 to 3 years after a slowly progressive course thereafter. abnormal laboratory findings except for electrophysiological morphological muscles. site lesion surmised be from C5 T1 spinal segments intramedullary involvement. cause is unknown.