Congenital sensory neuropathy is a term which is useful to denote a small group of patients in whom pain, temperature, and touch sensibility is absent or diminished on the extremities and varying portions of the trunk. Deep tendon reflexes are absent or hypoactive, and skin or sensory nerve biopsy or both usually reveal some abnormality of neural structures.1,2 The condition is sporadic and nonprogressive, unlike hereditary sensory radicular neuropathy, syringomyelia, and the acquired sensory neuropathies. Normal autonomic function distinguishes it from the dysautonomias and an interesting group of recently reported cases of analgesia and hypohidrosis.3,4 The presence of the histologic abnormalities mentioned above and involvement of sensory modalities other than pain should adequately separate the condition from congenital indifference to pain, though the information in some case reports is not sufficient to permit this distinction to be made clearly.
We report here what we believe to be the
Linarelli LG, Prichard JW. Congenital Sensory NeuropathyComplete Absence of Superficial Sensation. Am J Dis Child. 1970;119(6):513–520. doi:10.1001/archpedi.1970.02100050515012
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