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Myopathy in Primary Systemic Amyloidosis

Published online by Cambridge University Press:  18 September 2015

M.E. Roke ,
W.F.E. Brown ,
D. Boughner ,
L.C. Ang  and
G.P.A. Rice
M.E. Roke
Affiliation:
Department of Clinical Neurological Sciences, University Hospital, London
W.F.E. Brown
Affiliation:
Department of Clinical Neurological Sciences, University Hospital, London
D. Boughner
Affiliation:
Department of Clinical Neurological Sciences, University Hospital, London
L.C. Ang
Affiliation:
Department of Clinical Neurological Sciences, University Hospital, London
G.P.A. Rice*
Affiliation:
Department of Clinical Neurological Sciences, University Hospital, London
*
Clinical Neurological Science Department, University Hospital, P.O. Box 5339, Postal Station A, London, Ontario, Canada N6A 5A5
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Abstract:

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Involvement of the peripheral nervous system by amyloidosis is common. It is less well recognized that amyloid can directly infiltrate and weaken skeletal muscle. We report a case of a 73-year-old woman, known to have cardiac amyloidosis, who developed profound weakness secondary to amyloid myopathy. Review of the 8 other well documented cases in the literature has revealed a rather homogeneous syndrome. Proximal weakness, muscle stiffness, pseudohypertrophy and myalgia constitute the principal features. This syndrome usually develops in cases with well recognized generalized amyloidosis. Amyloid is deposited within the basal lamina of blood vessels and muscle fibers.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 15 , Issue 3 , August 1988 , pp. 314 - 316
Copyright
Copyright © Canadian Neurological Sciences Federation 1988

References

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