Preferred Name

Prion Diseases [Disease/Finding]

Synonyms

Transmissible Spongiform Encephalopathies

Prion Disease

ID

http://purl.bioontology.org/ontology/NDFRT/N0000003530

altLabel

Transmissible Spongiform Encephalopathies

Dementias, Transmissible

Transmissible Dementias

Prion Diseases

Prion-Induced Disorders

Encephalopathies, Spongiform, Transmissible

Prion Protein Diseases

Prion-Associated Disorders

Prion-Induced Disorder

Spongiform Encephalopathies, Transmissible

Prion Disease

cui

C0162534

MESH DEFINITION

A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)

MESH DUI

D017096

MeSH name

Prion Diseases

MESH UI

M0025975

NDFRT kind

DISEASE_KIND

notation

N0000003530

NUI

N0000003530

prefLabel

Prion Diseases [Disease/Finding]

SNOMED CID

20484008

230284004

tui

T047

subClassOf

http://purl.bioontology.org/ontology/NDFRT/N0000000710

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