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Nemaline Myopathy With Associated CardiomyopathyReport of Clinical and Detailed Autopsy Findings
A. Jon Stoessl, MD;
Angelika F. Hahn, MD;
David Malott, MSc;
David T. Jones, MD;
Malcolm D. Silver, MD
Arch Neurol. 1985;42(11):1084-1086.
Abstract
• We describe the clinical features and autopsy findings of an adult patient who had nemaline myopathy and an associated progressive cardiomyopathy. The spinal cord and the results of morphometric analysis of multiple peripheral nerves were normal. There was probable intrafusal fiber involvement, in addition to the typical histopathologic features of extrafusal fibers. Cardiac dysfunction was a prominent clinical and autopsy feature, but it has been infrequently recognized in this entity. Our findings suggest that there is a poor correlation between clinical and pathologic features in this disorder, and they support the need for careful cardiac evaluation of affected patients. Furthermore, the constellation of features favors a myopathic basis for the disease, in contradistinction to some previously expressed views.
Author Affiliations
From the Departments of Clinical Neurological Sciences (Drs Stoessl, Hahn, and Malott), Medicine (Dr Jones), and Pathology (Dr Silver), University of Western Ontario, London, Canada.
Footnotes
Accepted for publication Feb 3, 1985.
Reprint requests to Department of Neurological Sciences, Victoria Hospital, 375 South St, London, Ontario, Canada N6A 4G5 (Dr Hahn).
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