Congenital insensitivity to pain with anhidrosis: a case report
Journal of Korean Medical Science
; : 460-464, 1999.
Article
in En
| WPRIM
| ID: wpr-221953
Responsible library:
WPRO
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.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Atrophy
/
Self Mutilation
/
Sural Nerve
/
Tongue
/
Microscopy, Electron
/
Pain Insensitivity, Congenital
/
Fingers
/
Hypohidrosis
/
Korea
/
Intellectual Disability
Limits:
Humans
/
Infant
/
Male
Country/Region as subject:
Asia
Language:
En
Journal:
Journal of Korean Medical Science
Year:
1999
Type:
Article