Peripheral Nerve Involvement in Hunter Syndrome (Mucopolysaccharidosis II)
- 1 December 1976
- journal article
- case report
- Published by American Medical Association (AMA) in Archives of Neurology
- Vol. 33 (12) , 845-846
- https://doi.org/10.1001/archneur.1976.00500120049008
Abstract
• A 20-year-old man with confirmed Hunter syndrome had entrapments of median and ulnar nerves. Sural nerve biopsy specimen revealed clear, lamellated, and granular inclusions in Schwann cells, fibroblasts, and perineurial cells. The clear inclusions are believed to represent storage of mucopolysaccharide, and the zebra bodies, ganglioside. Focal alterations in myelin were found, associated with moderately dense granular inclusions. Following carpal tunnel release operations, the patient experienced dramatic relief of symptoms.Keywords
This publication has 3 references indexed in Scilit:
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- ELECTRON MICROSCOPY OF TWO CEREBRAL, BIOPSIES IN GARGOYLISMJournal of Neuropathology and Experimental Neurology, 1965
- Mucopolysaccharide Production By Fibroblasts Derived from Human Buffy-Coat Cultures in vitroExperimental Biology and Medicine, 1964