NR ARNN
AU Ashwath,M.L.; DeArmond,S.J.; Culclasure,T.
TI Prion-associated dilated cardiomyopathy
QU Archives of Internal Medicine 2005 Feb 14; 165(3): 338-40
PT case reports; journal article
AB 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.
MH Adult; Cardiomyopathy, Dilated/*complications/pathology; Creutzfeldt-Jakob Syndrome/*complications/pathology; Female; Humans; Myocardium/pathology; PrPsc Proteins/metabolism
AD Mercer University School of Medicine, Macon, GA, USA
SP englisch
PO USA