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Journal of the Korean Academy of Rehabilitation Medicine 1998;22(5):1151-1155.
Congenital Insensitivity to Pain with Anhidrosis: Follow-up of 1 case.
Choi, Eun Seok , Lee, Yeon Soo , Yang, Seung Han , Shin, Ji Nam , Kim, Yong Seog , Jung, Kyung Heui
1Department of Rehabilitation Medicine, The Catholic University of Korea, College of Medicine.
2Department of Radiology, The Catholic University of Korea, College of Medicine.
무한증을 동반한 선천성 동통 무감각증 추적검사 ⁣1례 보고⁣
최은석, 이연수1, 양승한, 신지남, 김용석, 정경희
가톨릭대학교 의과대학 재활의학교실, 1방사선과학교실
Abstract

Congenital insensitivity to pain with anhidrosis (CIPA: a hereditary sensory and autonomic neuropathy, HSAN IV) is a rare disease characterized by the self-mutilation, bone fracture, multiple scars, osteomyelitis, joint deformities and anhidrosis. The pathophysiologic mechanism remains unknown.

This is the report of a twelve years old boy who had been diagnosed as the CIPA at his age of five. Loss of unmyelinated and small myelinated nerve fibers have been noted in an abdominal skin biopsy. On follow up studies, no significant changes were noted in the clinical manifestations and in the findings of laboratory, radiologic and electrophysiologic studies when compared to the initial studies except for the minimally progressed neuropathic ankle joints. Long term follow up study including the sequential electrophysiologic examination and biopsy of nerve and muscle might be necessary to establish the natural course of the disease. Prevention of the injury should be emphasized for the good prognosis.

Key Words: Congenital insensitivity to pain with anhidrosis (CIPA), Electrophysiologic examination, Nerve biopsy, Skin biopsy, Neuropathic joint
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