HEREDITARY SENSORY RADICULAR NEUROPATHY

Abstract

11 of 36 members of a family were affected by "hereditary per forating ulcer of the foot," transmitted as a simple Mendelian dominant. The syndrome includes dissociated disorder of sensation beginning in the feet, and progressive deafness. In sporadic and familial forms deafness is absent. Autopsy of one patient disclosed primary degeneration of dorsal root ganglia with secondary emyloid deposits in the most severely affected ganglia.