TY - JOUR
T1 - Peripheral Nerve Involvement in Hunter Syndrome (Mucopolysaccharidosis II)
AU - Swift, Thomas R.
AU - McDonald, Thomas F.
PY - 1976/12
Y1 - 1976/12
N2 - 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.
AB - 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.
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U2 - 10.1001/archneur.1976.00500120049008
DO - 10.1001/archneur.1976.00500120049008
M3 - Article
C2 - 187158
AN - SCOPUS:0017291951
SN - 0003-9942
VL - 33
SP - 845
EP - 846
JO - Archives of Neurology
JF - Archives of Neurology
IS - 12
ER -