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Maladie de Creutzfeldt-Jakob
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Définition:
A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ; , visual loss, weakness, muscle atrophy, , 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 ) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of . (From N Engl J Med, 1998 Dec 31;339(27))
Synonyme(s):
Encéphalopathie spongiforme subaiguë /
Syndrome de Creutzfeldt-Jakob /
MCJ (Maladie de Creutzfeldt-Jakob) /
Pseudo-sclérose spastique de Jakob /
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(NLM)®
de l'INSERM (version française)
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PSYCHIATRIE ET PSYCHOLOGIE
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