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Porphyric Neuropathy: An Ultrastructural and Quantitative Case Study

Published online by Cambridge University Press:  18 September 2015

P.S. Thorner
Affiliation:
St. Michael’s Hospital, Toronto General Hospital and The University of Toronto, Canada
J.M. Bilbao
Affiliation:
St. Michael’s Hospital, Toronto General Hospital and The University of Toronto, Canada
A.A.F. Sima*
Affiliation:
St. Michael’s Hospital, Toronto General Hospital and The University of Toronto, Canada
S. Briggs
Affiliation:
St. Michael’s Hospital, Toronto General Hospital and The University of Toronto, Canada
*
Division of Neuropathology, Department of Pathology, Banting Institute, 100 College Street, Toronto, Ontario, Canada M5G 1L5
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Summary:

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We report a case of acute neuropathy in a 46 year old female with porphyria variegata. Histologic, electron microscopic, and quantitative examinations of peripheral nerves were performed at onset of the neuropathy and at autopsy. The results revealed severe qualitative and quantitative changes in myelinated and unmyelinated fibers showing features indicative of an axonopathy with a distribution in keeping with a dying-back phenomenon.

Type
Research Article
Copyright
Copyright © Canadian Neurological Sciences Federation 1981

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