Peripheral Nerve Involvement in Hunter Syndrome (Mucopolysaccharidosis II)

Thomas R. Swift, Thomas F. McDonald

Research output: Contribution to journalArticle

15 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)845-846
Number of pages2
JournalArchives of Neurology
Volume33
Issue number12
DOIs
StatePublished - Dec 1976

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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