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Journal of the Korean Academy of Rehabilitation Medicine 2001;25(5):791-794.
Nerve Conduction Studies in the Patients with Mucopolysaccharidoses.
Kim, Sang Yong , Kim, Hyeon Sook , Lee, K W , Hwang, Ji Hye , Lee, Yong Taek , Jung, Soon Tak , Lee, Mun Hyang , Jin, Dong Ku
1Department of Physical Medicine and Rehabilitation, Samsung Medical Center, Sungkyunkwan University School of Medicine.
2Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine.
뮤코다당체 침착증 환아에 있어서의 신경전도검사 소견
김상용, 김현숙, 이강우, 황지혜, 이용택, 정순탁, 이문향1, 진동규1
성균관대학교 의과대학 삼성서울병원 재활의학과교실, 1소아과학교실
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.

Key Words: Nerve conduction study, Mucopolysaccharidoses, Polyneuropathy, Carpal tunnel syndrome


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