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Prion Disease.
- Source :
-
Seminars in neurology [Semin Neurol] 2019 Aug; Vol. 39 (4), pp. 428-439. Date of Electronic Publication: 2019 Sep 18. - Publication Year :
- 2019
-
Abstract
- Prion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt-Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial insomnia, and Gerstmann-Straussler-Scheinker syndrome), and acquired (kuru, variant CJD, and iatrogenic CJD). This article will review the pathophysiology, genetics, clinical presentations, and diagnostic challenges in patients with prion disease. Case discussions, images, and tables will be used to highlight important characteristics of prion disease and prion mimics.<br />Competing Interests: None.<br /> (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)
- Subjects :
- Aged
Animals
Creutzfeldt-Jakob Syndrome psychology
Diagnosis, Differential
Humans
Male
Middle Aged
Prion Diseases cerebrospinal fluid
Prion Diseases diagnostic imaging
Prion Diseases psychology
Wernicke Encephalopathy psychology
Creutzfeldt-Jakob Syndrome cerebrospinal fluid
Creutzfeldt-Jakob Syndrome diagnostic imaging
Wernicke Encephalopathy cerebrospinal fluid
Wernicke Encephalopathy diagnostic imaging
Subjects
Details
- Language :
- English
- ISSN :
- 1098-9021
- Volume :
- 39
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Seminars in neurology
- Publication Type :
- Academic Journal
- Accession number :
- 31533183
- Full Text :
- https://doi.org/10.1055/s-0039-1687841