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Creutzfeldt-Jakob disease: literature review based on three case reports

DOENÇA DE CREUTZFELDT-JAKOB: REVISÃO DA LITERATURA BASEADA NO RELATO DE TRÊS CASOS

ABSTRACT.

Creutzfeldt-Jakob disease (CJD) is one of the transmissible spongiform encephalopathies that lead to rapidly progressive dementia. CJD has a low prevalence, and the average survival is only 1 year after the onset of symptoms. As the patients with CJD develop rapidly progressive dementia, associated with myoclonus, visual or cerebellar problems, pyramidal or extrapyramidal features, and akinetic mutism, the hypothesis of CJD must be raised. Classic magnetic resonance imaging (MRI) findings are hypersignals in the caudate nucleus, putamen, and cortical region. CJD must be considered a differential diagnosis of other types of dementia, and there is no effective treatment for this disease. In this article, we present a literature review based on the report of three cases of the sporadic form of this disease.

Keywords:
Creutzfeldt-Jakob Syndrome; Prion Diseases; Myoclonus

Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices, Torre Norte, São Paulo, SP, Brazil, CEP 04101-000, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revistadementia@abneuro.org.br | demneuropsy@uol.com.br