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Nemaline Myopathy Appearing in Adults as CardiomyopathyA Clinicopathologic Study
Claus Meier, MD;
Werner Voellmy, MD;
Mark Gertsch, MD;
Arthur Zimmermann, MD;
Josef Geissbühler, MD
Arch Neurol. 1984;41(4):443-445.
Abstract
• We examined a 29-year-old woman with nemaline myopathy that appeared as cardiomyopathy. Clinical examination showed dilated cardiomyopathy, but no neuromuscular abnormalities of the skeletal muscles. Electromyography showed neither neurogenic nor myopathic abnormalities. A biopsy specimen from the quadripecs muscle showed typical nemaline bodies in about 50% of the muscle fibers. The patient died six months later of biventricular heart insufficiency. Autopsy revealed nemaline bodies in the working and conducting tissues of the myocardium. Earlier, the patient's mother and one of her sisters died unexplained, sudden deaths at the ages of 47 and 37 years, respectively. Sections of the myocardium taken from the sister at autopsy were available, and also disclosed nemaline bodies after restaining with trichrome.
Author Affiliations
From the Departments of Neurology (Dr Meier), Cardiology (Dr Gertsch), and Pathology (Dr Zimmermann), University of Bern (Switzerland); and the Division of Medicine, Regionalspital, Langenthal (Switzerland) (Drs Voellmy and Geissbiihler).
Footnotes
Accepted for publication June 11, 1983.
Reprint requests to Department of Neurology, Inselspital, CH-3010 Bern, Switzerland.
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