Hereditary Sensory Neuropathy

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Abstract
Hereditary sensory neuropathy is a condition transmitted by a dominant gene, causing severe peripheral sensory loss, loss of deep reflexes and severe trophic disorders of all 4 extremities. It was originally described as hereditary peforating ulcers of the feet. Five patients of 1 family, 4 of whom are living, are presented. In addition 3 members of the family showed a mild or incomplete form. Family history is traced through 6 generations. The relationship to Morvan''s disease is discussed. Primary pathological lesion seems to be in the posterior root ganglion, however another important factor seems to be the length of the nerve from its ganglion. We feel there must be a metabolic error due to either a defective enzyme system which alters the intermediary metabolism of available food elements, with the accumulation of some toxic product, or a defect of peripheral nerve enzymes in the utilization of normal food sources.

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