Peripheral Nerve Involvement in Hunter Syndrome (Mucopolysaccharidosis II)

Thomas Robert Swift, Thomas F. McDonald

Research output: Contribution to journalArticle

14 Citations (Scopus)

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 - Jan 1 1976

Fingerprint

Mucopolysaccharidosis II
Sural Nerve
Equidae
Gangliosides
Median Nerve
Schwann Cells
Myelin Sheath
Glycosaminoglycans
Wrist
Peripheral Nerves
Fibroblasts
Biopsy
Inclusion
Nerve
Hunters
Syndrome
Cells

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

Cite this

Peripheral Nerve Involvement in Hunter Syndrome (Mucopolysaccharidosis II). / Swift, Thomas Robert; McDonald, Thomas F.

In: Archives of Neurology, Vol. 33, No. 12, 01.01.1976, p. 845-846.

Research output: Contribution to journalArticle

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