Congenital sensory neuropathy in siblings.

Abstract

Two siblings with congenital sensory neuropathy are discussed whose inheritance appears to be autosomal recessive, an unusual pattern for this disorder. Low cerebrospinal fluid protein was demonstrated in both children early in life, and at present one continues to have low cerebrospinal fluid total protein, while the other has an unusual spinal fluid electrophoretic pattern with very low albumin concentration. Both children suffered from chronic, unexplained diarrhea in their early years. The children9s problems are reviewed, and the importance of classifying the various sensory disorders is stressed.