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Neuropediatrics 1981; 12(1): 83-91
DOI: 10.1055/s-2008-1059642
© Georg Thieme Verlag KG Stuttgart · New York

CHANGES IN THE VOLUNTARY MUSCLES AND THE PERIPHERAL NERVES IN AN AUTOPSY CASE OF MPS TYPE II (HUNTER)

H. P. Schmitt
  • Institute of Neuropathology, University of Heidelberg (GFR), Im Neuenheimer Feld 220, D-6900 Heidelberg 1, GFR
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Publikationsdatum:
19. März 2008 (online)

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Abstract

Skeletal muscles and peripheral nerves obtained at autopsy from a fifteen-year-old boy who had mucopolysaccharidosis type II (Hunter) were examined. The muscles showed severe neurogenic atrophy, chiefly from a distal motor neuropathy due to damage of the intramuscular nerve terminals. There was only a very slight loss of motor neurons in the spinal cord, which itself could not explain the severe atrophy of the muscles. Contrary to former observations in Tay-Sachs disease, in which the telodendra of peripheral nerves had exhibited abundant intraaxonal storage phenomena, similar axonal distensions were not found in the present case.

Key words

Mucopolysaccharidosis - skeletal muscles - peripheral nerves