A Test for Creutzfeldt–Jakob Disease Using Nasal Brushings

This preliminary study assessed the accuracy of testing nasal brushings for the prion protein (PrPCJD) found in Creutzfeldt–Jakob disease. In 31 patients with disease and 43 controls, the test had 97% sensitivity and 100% specificity. Prion diseases, or transmissible spongiform encephalopathies, are...

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Vydáno v:The New England journal of medicine Ročník 371; číslo 6; s. 519 - 529
Hlavní autoři: Orrú, Christina D, Bongianni, Matilde, Tonoli, Giovanni, Ferrari, Sergio, Hughson, Andrew G, Groveman, Bradley R, Fiorini, Michele, Pocchiari, Maurizio, Monaco, Salvatore, Caughey, Byron, Zanusso, Gianluigi
Médium: Journal Article
Jazyk:angličtina
Vydáno: Waltham, MA Massachusetts Medical Society 07.08.2014
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ISSN:0028-4793, 1533-4406, 1533-4406
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Shrnutí:This preliminary study assessed the accuracy of testing nasal brushings for the prion protein (PrPCJD) found in Creutzfeldt–Jakob disease. In 31 patients with disease and 43 controls, the test had 97% sensitivity and 100% specificity. Prion diseases, or transmissible spongiform encephalopathies, are fatal neurodegenerative disorders in humans and animals. 1 , 2 Prion diseases include Creutzfeldt–Jakob disease, the Gerstmann–Sträussler–Scheinker syndrome, and fatal familial insomnia in humans. The most common form of human prion disease is sporadic Creutzfeldt–Jakob disease, with an incidence of approximately 1 case per million persons per year worldwide. 3 Sporadic Creutzfeldt–Jakob disease is clinically heterogeneous and includes forms characterized by psychotic symptoms, depression, and behavioral and personality changes. 4 , 5 Possible or probable sporadic Creutzfeldt–Jakob disease is defined on the basis of clinical features, as well as periodic sharp and slow wave complexes on electroencephalograms, a . . .
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Drs. Orrú and Bongianni contributed equally to this article.
ISSN:0028-4793
1533-4406
1533-4406
DOI:10.1056/NEJMoa1315200