A rare case of rapidly progressive dementia with elevated RT-QuIC and negative 14-3-3 and tau proteins.
Prion
; 10(3): 262-4, 2016 05 03.
Article
em En
| MEDLINE
| ID: mdl-27249661
ABSTRACT
Creutzfeldt-Jakob disease (CJD) is characterized by rapidly progressing dementia with death usually occurring within 6 months. There is no verified disease-specific pre-mortem diagnostic test besides brain biopsy. We describe a 66 y old previously high functioning male who presented with a 5 month history of rapidly progressive dementia. Neurological examination revealed a score of 19/30 on MOCA testing. An extensive workup into various causes of dementia including electroencephalography and imaging studies was unremarkable. The cerebrospinal fluid was sent to National Prion Disease Center and it revealed elevated RT-QuIC levels with negative 14-3-3 and T tau proteins. Based on literature review, our case is one of few living subjects with elevated RT-QuIC levels and negative 14-3-3 and tau proteins.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Príons
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Síndrome de Creutzfeldt-Jakob
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Proteínas tau
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Demência
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Proteínas 14-3-3
Limite:
Aged
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Humans
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Male
Idioma:
En
Revista:
Prion
Assunto da revista:
BIOQUIMICA
Ano de publicação:
2016
Tipo de documento:
Article