Congenital sensory neuropathy with selective loss of small myelinated fibers

Ann Neurol. 1978 Feb;3(2):179-82. doi: 10.1002/ana.410030215.


The results of clinical, electrophysiological, autonomic, and histopathological studies in a 6-year-old patient with congenital sensory neuropathy are presented. Selective loss of pain perception affecting the extremities was noted from infancy. Nerve conduction studies were normal. Small myelinated fibers were selectively reduced in the sural nerve, and unmyelinated fibers were normal.

Publication types

  • Case Reports

MeSH terms

  • Autonomic Nervous System / physiopathology
  • Child
  • Female
  • Humans
  • Nerve Fibers, Myelinated / pathology*
  • Pain Insensitivity, Congenital / pathology*
  • Pain Insensitivity, Congenital / physiopathology
  • Sural Nerve / pathology