{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,4,3]],"date-time":"2022-04-03T12:12:44Z","timestamp":1648987964808},"reference-count":25,"publisher":"FapUNIFESP (SciELO)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq. Neuro-Psiquiatr."],"published-print":{"date-parts":[[2003,12]]},"abstract":"<jats:p>INTRODU\u00c7\u00c3O E OBJETIVO: Descri\u00e7\u00e3o das caracter\u00edsticas demogr\u00e1ficas, clinicas e neuropatol\u00f3gicas de 11 doentes com doen\u00e7a de Creutzfeldt-Jakob (DCJ). M\u00c9TODO: Revis\u00e3o cl\u00ednica e neuropatol\u00f3gica de doentes com DCJ diagnosticados entre 1993 e 2002 em hospitais do Norte de Portugal. RESULTADOS: Foram identificados 11 doentes (4 do sexo feminino; idade m\u00e9dia de in\u00edcio dos sintomas, 64 anos; m\u00e9dia de dura\u00e7\u00e3o da doen\u00e7a, 8 meses). Todos apresentaram s\u00edndrome demencial progressiva associada a mioclonias, sendo a s\u00edndrome cerebelar a forma de apresenta\u00e7\u00e3o inicial em quatro deles. O estudo neuropatol\u00f3gico revelou sempre espongiose e gliose reativa associada a perda neuronal. O estudo imunocitoqu\u00edmico para prote\u00edna pri\u00f4nica (PrP) foi positivo nos oito casos em que foi executado. CONCLUS\u00c3O: O grupo de doentes descritos constitui uma s\u00e9rie clinica representativa da heterogeneidade de fen\u00f3tipos poss\u00edveis da DCJ espor\u00e1dica. O estudo neuropatol\u00f3gico \u00e9 ainda indispens\u00e1vel para o diagn\u00f3stico definitivo da doen\u00e7a.<\/jats:p>","DOI":"10.1590\/s0004-282x2003000600012","type":"journal-article","created":{"date-parts":[[2005,5,12]],"date-time":"2005-05-12T20:26:33Z","timestamp":1115929593000},"page":"950-956","source":"Crossref","is-referenced-by-count":2,"title":["Estudo retrospectivo da doen\u00e7a de Creutzfeldt-Jakob diagnosticada no norte de Portugal entre 1993-2002: caracter\u00edsticas demogr\u00e1ficas, cl\u00ednicas e neuropatol\u00f3gicas"],"prefix":"10.1590","volume":"61","author":[{"given":"Ana Martins","family":"Silva","sequence":"first","affiliation":[{"name":"Hospital Geral de Santo Ant\u00f3nio,  Portugal"}]},{"given":"Manuel Melo","family":"Pires","sequence":"additional","affiliation":[{"name":"Hospital Geral de Santo Ant\u00f3nio"}]},{"given":"Antonio J Bastos","family":"Leite","sequence":"additional","affiliation":[{"name":"Hospital Geral de Santo 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