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Peripheral Nerve Involvement in Hunter Syndrome (Mucopolysaccharidosis II)
Thomas R. Swift, MD;
Thomas F. McDonald, PhD
Arch Neurol. 1976;33(12):845-846.
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.
Author Affiliations
From the Department of Neurology, Medical College of Georgia, Augusta.
Footnotes
Accepted for publication March 31, 1976.
Reprint requests to Department of Neurology, Medical College of Georgia, Augusta, GA 30902 (Dr Swift).
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