loading...| Preferred Name | Creutzfeldt-Jakob Syndrome | |
| Synonyms |
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| 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). |
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| ID |
http://uri.neuinfo.org/nif/nifstd/birnlex_12687 |
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| acronym |
CJD |
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| createdDate |
2007-10-05 |
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| definingCitation |
N Engl J Med, 1998 Dec 31;339(27) |
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| 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). |
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| editorial note | ||
| hasBirnlexCurator | ||
| hasCurationStatus | ||
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| hasExternalSource | ||
| label |
Creutzfeldt-Jakob Syndrome |
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| MeshUid |
D007562 |
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| modifiedDate |
April 11, 2009 |
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| nifID |
_8.3_2.8.1 |
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| note |
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). |
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| preferred label |
Creutzfeldt-Jakob Syndrome |
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| synonym |
Jakob-Creutzfeldt Disease Jakob Creutzfeldt Disease Jakob-Creutzfeldt Syndrome Creutzfeldt-Jakob Disease |
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| subClassOf |