Preferred Name

Creutzfeldt-Jakob Syndrome

Synonyms
Definitions

A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS (MeSH).

ID

http://uri.neuinfo.org/nif/nifstd/birnlex_12687

acronym

CJD

createdDate

2007-10-05

definingCitation

N Engl J Med, 1998 Dec 31;339(27)

definition

A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS (MeSH).

hasBirnlexCurator

http://uri.neuinfo.org/nif/nifstd/readable/Bill_Bug

hasCurationStatus

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

hasDefinitionSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH_defSource

hasExternalSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH

label

Creutzfeldt-Jakob Syndrome

MeshUid

D007562

modifiedDate

April 11, 2009

nifID

_8.3_2.8.1

preferred label

Creutzfeldt-Jakob Syndrome

synonym

Jakob-Creutzfeldt Disease

Jakob Creutzfeldt Disease

Jakob-Creutzfeldt Syndrome

Creutzfeldt-Jakob Disease

subClassOf

http://uri.neuinfo.org/nif/nifstd/birnlex_12686

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