Congenital Insensitivity to Pain with Anhydrosis

Abstract

Introduction The syndrome of congenital to noxious stimuli (congenital universal to pain, congenital 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 is generally applied. However, Kunkle1recently 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 to pain is usually considered congenital, Ervin's report of a family with dominant inheritance2is 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