Hereditary sensory neuropathy: a case with pain and temperature dissociation.

ABSTRACT

A case of hereditary sensory neuropathy is described resembling Dyck's Type I hereditary sensory neuropathy. Sensory testing revealed marked pain impairment in feet and hands shading at mid calf to normal at mid thigh and shading above the wrist to normal at the elbow. Other sensory modalities including temperature were intact except painful heat and painful cold and they produced very little if any discomfort. Stimuli at 0 degrees C or 45 degrees to 70 degrees C elicited a temperature response but not pain. Sural nerve biopsy findings (including electromicroscopy) were consistent with a diagnosis of Type I hereditary sensory neuropathy, but also showed evidence of abortive axonal regeneration and profound Schwann cell vacuolation.