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Congenital Insensitivity to Pain with AnhydrosisA Unique Syndrome in Two Male Siblings
AUGUST G. SWANSON, M.D.
Arch Neurol. 1963;8(3):299-306.
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Introduction
The syndrome of congenital indifference to noxious stimuli (congenital universal indifference to pain, congenital indifference to pain, congenital insensitivity to pain, congenital pure analgesia, congenital analgia) consists of failure to react to ordinarily painful stimuli and usually occurs as an isolated sensory abnormality. Most observers have attributed the syndrome to an abnormal affective response rather than to defective perception. Therefore the term, "indifference to pain" is generally applied. However, Kunkle1 recently has emphasized that some patients manifest signs indicating faulty transmission of pain signals. He comments that insensitivity to pain is a more appropriate term if evidence for defective transmission is demonstrated.
While indifference to pain is usually considered congenital, Ervin's report of a family with dominant inheritance2 is the only well-documented case of familial occurrence. One-third of the 36 patients reported in the literature were over 25 years of age when studied, and the congenital nature
. . . [Full Text PDF of this Article]
Author Affiliations
SEATTLE
From the Division of Neurology and the Department of Pediatrics, University of Washington School of Medicine.
Footnotes
Submitted for publication July 2, 1962.
Presented at the American Academy of Neurology Meeting, Detroit, 1961.
Assistant Professor Pediatrics and Medicine for Neurology.
John and Mary R. Markle Scholar in Medicine.
A portion of this work was conducted through the Clinical Research Center facility of the University of Washington supported by the National Institute of Health (Grant OG-13).
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