Nerve Conduction Studies in the Patients with Mucopolysaccharidoses.
- Author:
Sang Yong KIM
1
;
Hyeon Sook KIM
;
K W LEE
;
Ji Hye HWANG
;
Yong Taek LEE
;
Soon Tak JUNG
;
Mun Hyang LEE
;
Dong Ku JIN
Author Information
1. Department of Physical Medicine and Rehabilitation, Samsung Medical Center, Sungkyunkwan University School of Medicine.
- Publication Type:Original Article
- Keywords:
Nerve conduction study;
Mucopolysaccharidoses;
Polyneuropathy;
Carpal tunnel syndrome
- MeSH:
Adolescent;
Age Distribution;
Carpal Tunnel Syndrome;
Child;
Electrophoresis;
Enzyme Assays;
Humans;
Mucopolysaccharidoses*;
Neural Conduction*;
Peripheral Nervous System;
Polyneuropathies;
Wrist
- From:Journal of the Korean Academy of Rehabilitation Medicine
2001;25(5):791-794
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
OBJECTIVE: To evaluate the characteristics of peripheral nervous system involvement in patients with mucopolysaccharidoses (MPS). METHOD: Electrophysiologic studies were performed in 26 children with MPS confirmed by semiquantitative MPS study, high resolution electrophoresis and enzyme assay. The age distribution of the patients were 2 to 18 year old (mean 8.2 year old). RESULTS: Of the 26 children, 21 children (80.8%) showed abnormal electrophysiologic finding. Eighteen children had median entrapment neuropathy at wrist level (carpal tunnel syndrome), 3 children had demyelinating peripheral polyneuropathies dominant in motor nerves. CONCLUSION: The most prominent features of the peripheral nervous system involvement in MPS patients were entrapment neuropathy at wrist but concomittent peripheral polyneuropathy. Further studies would be necessary to clarify the characteristics of the peripheral polyneuropathy in MPS.
