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Cardiomyopathy Associated with the Syndrome of Amyotrophic Chorea and Acanthocytosis

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Grant support: provided by research training grant HL07220, National Heart, Lung, and Blood Institute, National Institute of Health. Dr. Faillace was a recipient of the John C. Sable Memorial Heart Fund Fellowship 1981-1982.

▸Requests for reprints should be addressed to Navin C. Nanda, M.D.; Box 679, Cardiology Unit, University of Rochester Medical Center; 601 Elmwood Avenue; Rochester, NY 14642.

University of Rochester School of Medicine, Strong Memorial Hospital; Rochester, New York

Ann Intern Med. 1982;96(5):616-617. doi:10.7326/0003-4819-96-5-616
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Cardiac disease occurs in many forms of neuromuscular diseases (1). We report the occurrence of idiopathic congestive cardiomyopathy associated with amyotrophic chorea and acanthocytosis. Three neurologic disorders are associated with acanthocytosis: the Bassen-Kornzweig syndrome (abetalipoproteinemia); a syndrome characterized by symptoms and signs of a progressive demyelinating disorder of the central nervous system and hypobetalipoproteinemia; and a syndrome of amyotrophic chorea and normobetalipoproteinemia (2-4). Amyotrophic chorea with acanthocytosis is a hereditary neuromuscular syndrome consisting of choreiform movements, limb wasting, and areflexia associated with normolipoproteinemic acanthocytes. However, no person affected with this syndrome has been reported to have coexistent cardiac disease (2-4).


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