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Article
June 1970

Congenital Sensory NeuropathyComplete Absence of Superficial Sensation

Author Affiliations

Bethesda, Md
From the Children's Diagnostic and Study Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Md. Dr. Linarelli is now at the Depart-; ment of Pediatrics, Mercy Hospital, Pittsburgh.

Am J Dis Child. 1970;119(6):513-520. doi:10.1001/archpedi.1970.02100050515012
Abstract

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

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