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Prion-associated dilated cardiomyopathy
Journal article   Peer reviewed

Prion-associated dilated cardiomyopathy

Mahi Lakshmi Ashwath, Stephen J Dearmond and Talley Culclasure
Archives of internal medicine (1960), Vol.165(3), pp.338-340
02/14/2005
DOI: 10.1001/archinte.165.3.338
PMID: 15710800

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Abstract

Creutzfeldt-Jakob disease is a spongiform encephalopathy affecting 1 individual per million population per year. We report on a previously healthy 43-year-old patient who presented with the simultaneous onset of a movement disorder, encephalopathy, cognitive decline, and dilated cardiomyopathy, and was found to have spongiform encephalopathy on brain biopsy. Although her neurological features could be explained by Creutzfeldt-Jakob disease, the etiology of the dilated cardiomyopathy could not be established. Finally, special staining of the endomyocardial biopsy specimen revealed the presence of abnormal prion, possibly infectious scrapie prion. As an exhaustive search for familial, ischemic, infectious, autoimmune, toxic, and metabolic causes of dilated cardiomyopathy was unrevealing, the presence of abnormal prion in the cardiac muscle suggested the possibility of prion-induced dilated cardiomyopathy in our patient.
 Adult Cardiomyopathy, Dilated - complications Cardiomyopathy, Dilated - pathology Creutzfeldt-Jakob Syndrome - complications Creutzfeldt-Jakob Syndrome - pathology Female Humans Myocardium - pathology PrPSc Proteins - metabolism

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