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Creutzfeldtov-Jakobov syndróm

  1. Subject h.Creutzfeldtov-Jakobov syndróm
    Subject h.Creutzfeldt-Jakob Syndrome
    Entry termsJakobov-Creutzfeldtov syndróm
    vCJD
    CJD variantná
    Creutzfeldt-Jakobova choroba, nový variant
    encefalopatia spongiformná subakútna
    encefalopatia spongioformná subakútna
    encefalopatia špongiformná subakútna
    English X referencesNew Variant Creutzfeldt-Jakob Disease
    Spongiform Encephalopathy, Subacute
    Scope note in EnglishA 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. (From N Engl J Med, 1998 Dec 31;339(27))
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