A Case of Charcot-Marie-Tooth Disease type 1A with Guillain-Barre Syndrome.
- Author:
Bong Ho LEE
1
;
Byeong Cheol OH
;
Shin Kwang KHANG
;
Kwang Kuk KIM
Author Information
1. Department of Neurology, Asan Medical Center, College of Medicine, University of Ulsan, Korea. kkkim@www.amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Charcot-Marie-Tooth;
Guillain-Barre syndrome;
PMP-22 gene
- MeSH:
Adolescent;
Charcot-Marie-Tooth Disease*;
Exons;
Female;
Foot Deformities;
Guillain-Barre Syndrome*;
Humans;
Immunoglobulins;
Neural Conduction;
Quadriplegia
- From:Journal of the Korean Neurological Association
2002;20(6):721-724
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Charcot-Marie-Tooth disease (CMTD) is a hereditary neuropathy with slow progression, whereas Guillain-Barre syndrome is an acute acquired neuropathy of immunopathogenesis. A 17 year-old girl with hammertoes and pes cavus developed an acute quadriparesis. The duplication of PMP-22 exons in 17p12-p11.2 was confirmed by genetic study. After Intravenous ingection of immunoglobulin, she recovered to normal activity within one month with improvement of nerve conduction study. We should be concerned with the possibility of Guillain-Barre syndrome if there is rapidly progressive exacerbation on CMTD.