{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,12,31]],"date-time":"2024-12-31T19:10:22Z","timestamp":1735672222916,"version":"3.32.0"},"reference-count":42,"publisher":"FapUNIFESP (SciELO)","issue":"2a","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq. Neuro-Psiquiatr."],"published-print":{"date-parts":[[2004,6]]},"abstract":"<jats:p>A doen\u00e7a de Creutzfeldt-Jakob (CJD) \u00e9 uma forma de dem\u00eancia pr\u00e9-senil de r\u00e1pida evolu\u00e7\u00e3o, geralmente fatal em um ano. Casos aut\u00f3ctones no Brasil t\u00eam sido raramente descritos assim como achados de resson\u00e2ncia magn\u00e9tica. Mulher, natural de Ponta Grossa PR, branca , 54 anos , foi admitida no servi\u00e7o em outubro de 2001 com quadro de amaurose bilateral cortical progressiva desde h\u00e1 1 m\u00eas do internamento. Nunca viajou ao exterior e foi somente submetida a uma cirurgia de redu\u00e7\u00e3o do est\u00f4mago, para obesidade. Hist\u00f3ria familial sem relato de casos semelhantes. Logo ap\u00f3s o internamento a paciente desenvolveu quadro de disfasia mista, hemiparesia fl\u00e1cida direita, com movimentos coreoatet\u00f3ticos e crises parciais motoras. Paciente evoluiu com quadro demencial progressivo; atualmente, acamada, torporosa, dependente de alimenta\u00e7\u00e3o enteral, recebendo mepacrina, fenito\u00edna e clorpromazina , estabilizando o quadro at\u00e9 final de maio de 2002. Exames laboratoriais negativos ou normais. Pesquisa de prote\u00edna 14-3-3 no l\u00edquor foi positiva; enolase-neur\u00f4nio-espec\u00edfica no l\u00edquor foi normal. Estudo gen\u00e9tico do gen PRNP n\u00e3o revelou muta\u00e7\u00e3o descrita anteriormente. EEG (23\/10\/2001) revelou intensa atividade irritativa hemisf\u00e9rio cerebral esquerdo. Estudo de resson\u00e2ncia magn\u00e9tica revelou \u00e1reas de hipersinal em T2 e FLAIR em regi\u00f5es temporal esquerda e bioccipital; g\u00e2nglios da base normal. Imagens de DWI mostraram hipersinal nas mesmas \u00e1reas.Outro EEG (15\/03\/2002) revelou padr\u00e3o peri\u00f3dico de ondas trif\u00e1sicas sugestivos de CJD. A paciente fez uso de mepacrina associado a clorpromazina com aparente estabiliza\u00e7\u00e3o do quadro, at\u00e9 seu \u00f3bito por complica\u00e7\u00f5es infecciosas pulmonares em abril de 2003.<\/jats:p>","DOI":"10.1590\/s0004-282x2004000200029","type":"journal-article","created":{"date-parts":[[2005,4,15]],"date-time":"2005-04-15T14:37:52Z","timestamp":1113575872000},"page":"347-352","source":"Crossref","is-referenced-by-count":16,"title":["Doen\u00e7a de Creutzfeldt-Jakob forma Heidenhain: relato de caso com achados de resson\u00e2ncia magn\u00e9tica e DWI"],"prefix":"10.1590","volume":"62","author":[{"given":"Walter Oleschko","family":"Arruda","sequence":"first","affiliation":[{"name":"Instituto de Neurologia de Curitiba, Brasil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kelly C.","family":"Bordignon","sequence":"additional","affiliation":[{"name":"Instituto de Neurologia de Curitiba, 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