Congenital insensitivity to pain with anhidrosis: a case report.

Abstract:

:Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare genetic disorder of the peripheral nervous system characterized by recurrent episodes of unexplained fever, generalized anhidrosis, insensitivity to pain and temperature, and accompanied by self-mutilating behavior and mental retardation. We report on a 16 month-old boy with CIPA who exhibited these characteristic clinical features. A sural nerve biopsy revealed markedly reduced numbers of unmyelinated and small myelinated fibers, consistent with the characteristic features of CIPA.

journal_name

J Korean Med Sci

authors

Kim JS,Woo YJ,Kim GM,Kim CJ,Ma JS,Hwang TJ,Lee MC

doi

10.3346/jkms.1999.14.4.460

keywords:

subject

Has Abstract

pub_date

1999-08-01 00:00:00

pages

460-4

issue

4

eissn

1011-8934

issn

1598-6357

journal_volume

14

pub_type

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