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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Vydané v:	The New England journal of medicine Ročník 371; číslo 6; s. 519 - 529
Hlavní autori:	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:	English
Vydavateľské údaje:	Waltham, MA Massachusetts Medical Society 07.08.2014
Predmet:	Aged Allergies Amyloid Biological and medical sciences Biopsy Brain - pathology Cerebrospinal fluid Creutzfeldt-Jakob disease Creutzfeldt-Jakob Syndrome - diagnosis Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Diagnosis Diagnostic tests Epithelium - chemistry Female Fibrils Fluorescence General aspects Humans Indicator Dilution Techniques Infectious diseases Male Medical sciences Middle Aged Nasal Mucosa - chemistry Neurology Olfactory epithelium Polymerization Prion protein Prions Prions - analysis Prions - cerebrospinal fluid Protein seeding Proteins Seeds Sensitivity and Specificity Italy Infection Medicine Prion disease Nervous system diseases Nose Central nervous system disease Creutzfeldt-Jakob disease Brushing Degenerative disease Nasopharynx Prion Cerebral disorder
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 . . .
Bibliografia:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 14 content type line 23 Drs. Orrú and Bongianni contributed equally to this article.
ISSN:	0028-4793 1533-4406 1533-4406
DOI:	10.1056/NEJMoa1315200