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The neuropathology of “typical” Friedreich's ataxia in Quebec

Published online by Cambridge University Press:  18 September 2015

J.B. Lamarche ,
B. Lemieux  and
H.B. Lieu
J.B. Lamarche
Affiliation:
Centre Hospitalier Universitaire de Sherbrooke
B. Lemieux
Affiliation:
Centre Hospitalier Universitaire de Sherbrooke
H.B. Lieu
Affiliation:
Centre Hospitalier Universitaire de Sherbrooke
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Abstract

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We present the pathological data from the autopsies performed on 6 Friedreich's disease patients since the start of the Quebec Cooperative Study. All patients met the strict diagnostic criteria of the QCSFA. The anatomical lesions found in the peripheral and central nervous system were similar in all 6 cases and do not differ from those described in the literature. The clinical findings correlate closely with the histological lesions found in the peripheral nervous system and spinal cord. The evidence of segmental demyelination and remyelination in the spinal ganglia and posterior roots further supports the dying-back axonopathy hypothesis.

Type
B—Clinical Physiology and Pathology
Information
Canadian Journal of Neurological Sciences , Volume 11 , Issue S4 , November 1984 , pp. 592 - 600
Copyright
Copyright © Canadian Neurological Sciences Federation 1984

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