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Information- Original Article
- J Korean Acad Rehabil Med. 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
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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.
- 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.
Keywords :Nerve conduction study, Mucopolysaccharidoses, Polyneuropathy, Carpal tunnel syndrome