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Semantic dementia: Brazilian study of nineteen cases

Demência semântica: estudo brasileiro de 19 casos

Abstract

The term semantic dementia was devised by Snowden et al. in 1989 and nowadays, the semantic dementia syndrome is recognized as one of the clinical forms of frontotemporal lobar degeneration (FTLD) and is characterized by a language semantic disturbance associated to non-verbal semantic memory impairment. Objectives: The aim of this study was to describe a Brazilian sample of 19 semantic dementia cases, emphasizing the clinical characteristics important for differential diagnosis of this syndrome. Methods: Nineteen cases with semantic dementia were evaluated between 1999 and 2007. All patients were submitted to neurological evaluation, neuroimaging exams and cognitive, language and semantic memory evaluation. Results: All patients presented fluent spontaneous speech, preservation of syntactic and phonological aspects of the language, word-finding difficulty, semantic paraphasias, word comprehension impairment, low performance in visual confrontation naming tasks, impairment on tests of non-verbal semantic memory and preservation of autobiographical memory and visuospatial skills. Regarding radiological investigations, temporal lobe atrophy and/or hypoperfusion were found in all patients. Conclusions: The cognitive, linguistic and of neuroimaging data in our case series corroborate other studies showing that semantic dementia constitutes a syndrome with well defined clinical characteristics associated to temporal lobe atrophy.

Key words:
semantic dementia; semantic memory; fluent progressive aphasia; primary progressive aphasia; word comprehension; temporal lobe

Resumo

O termo demência semântica foi lançado por Snowden et al. em 1989 e, atualmente, a síndrome da demência semântica é reconhecida como uma das formas clínicas da degeneração lobar fronto-temporal (DLFT) e é caracterizada por distúrbio semântico da linguagem associado a comprometimento semântico não-verbal. Objetivos: Este trabalho teve como objetivo descrever uma amostra brasileira de 19 casos de demência semântica, ressaltando as características clínicas importantes para a realização do diagnóstico diferencial desta síndrome. Métodos: Foram estudados 19 casos com demência semântica avaliados entre 1999 e 2007. Todos os pacientes foram submetidos à avaliação neurológica, exames de neuroimagem e avaliação cognitiva, da linguagem e da memória semântica. Resultados: Todos os pacientes apresentaram produção espontânea fluente, preservação dos aspectos sintáticos e fonológicos da linguagem, dificuldade em encontrar palavras, parafasias semânticas, dificuldade de compreensão de palavras, baixo desempenho em provas de nomeação por confrontação visual, falhas em provas que avaliam a memória semântica não-verbal e preservação da memória autobiográfica e de habilidades visuoespaciais. Em relação aos achados de neuroimagem, o comprometimento do lobo temporal (atrofia e/ou hipoperfusão) foi encontrado em todos os pacientes. Conclusões: Os achados cognitivos, lingüísticos e de neuroimagem do nosso grupo de pacientes corroboram outros estudos que mostram que a demência semântica constitui uma síndrome com características clínicas bem definidas associada à atrofia do lobo temporal.

Palavras-chave:
demência semântica; memória semântica; afasia progressiva fluente; afasia progressiva primária; compreensão de palavras; lobo temporal

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Publication Dates

  • Publication in this collection
    Oct-Dec 2007

History

  • Received
    31 Oct 2007
  • Reviewed
    09 Nov 2007
  • Accepted
    27 Nov 2007
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
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