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Myopathy Associateci with Amyloid Angiopathy

Published online by Cambridge University Press:  03 July 2018

Joseph Bruni ,
Juan M. Bilbao  and
Kenneth P. H. Pritzker
Joseph Bruni
Affiliation:
Departments of Pathology, St. Michael’s Hospital and Mount Sinai Hospital, University of Toronto, Ontario, Canada
Juan M. Bilbao*
Affiliation:
Departments of Pathology, St. Michael’s Hospital and Mount Sinai Hospital, University of Toronto, Ontario, Canada
Kenneth P. H. Pritzker
Affiliation:
Departments of Pathology, St. Michael’s Hospital and Mount Sinai Hospital, University of Toronto, Ontario, Canada
*
Department of Pathology, St. Michael’s Hospital, 30 Bond Street, Toronto, Ontario, Canada M5B 1W8.
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Summary

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A 38-year-old patient with the clinical picture of a progressive myopathy resembling limb girdle muscular dystrophy is presented. Muscle biopsy showed amyloid deposits in the walls of small endomysial blood vessels. There was no clinical or physiological evidence of peripheral nerve involvement, no plasma cell dyscrasia and no generalized amyloidosis. There was no muscle fiber hypertrophy, inflammation or neurogenic change. There was no response to steroid therapy.

The etiopathogenesis of this amyloid angiopathy is undetermined. The extensive vessel involvement with amyloid deposition and the absence of changes indicative of muscular dystrophy or inflammatory myopathy leads us to favor an ischemic basis for this patient’s myopathy.

Résumé

Résumé

Le cas d'un patient de 38 ans avec une myopathie progressive ressemblant á une dystrophie des ceintures est présenté. Une biopsie du muscle montra des dépôts amyloϊdiens sur les parois de petits vaisseaux sanguins de I'endomètre. II n'y avail pas d' évidence clinique ou physiologique d'atteinte du nerf périphérique, pas de dyscrasie des cellules plasmatiques et pas d'amyloϊdose généralisée. II n'y avail pas d'hypertrophie des fibres musculaires, ni d'inflammation ou de changement neurogenique. II n'y eut pas de reponse au traitement aux stéroϊdes.

L'étiopathogénèse de cette angiopathie amyloϊde n'est pas déterminée. L'implication du vaisseau avec depot amyloϊde et I'absence de changements indicatifs d'une dystrophie musculaire ou d'une myopathie inflammatoire nous améne á croire en une raison ischèmique pour la myopathie de ce patient.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 4 , Issue 1 , February 1977 , pp. 77 - 80
Copyright
Copyright © Canadian Neurological Sciences Federation 1977

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