Primary progressive aphasia: analisys of 16 cases

Abstract

-Primary progressive aphasia (PPA) is an intriguing syndrome, showing some peculiar aspects that differentiate it from classical aphasic pictures caused by focal cerebral lesions or dementia. The slow and progressive deterioration of language occurring in these cases provides an interesting model to better understand the mechanisms involved in the linguistic process. We describe clinical and neuroimaging aspects found in 16 cases of PPA. Our patients underwent language and neuropsychological evaluation, magnetic resonance imaging (MRI) and single photon emission computerized tomography (SPECT). We observed a clear distinction in oral expression patterns; patients were classified as fluent and nonfluent. Anomia was the earliest and most evident symptom in both groups. Neuroimaging pointed to SPECT as a valuable instrument in guiding the differential diagnosis, as well as in making useful clinical and anatomical correlations. This report and a comparison to literature are an attempt to contribute to a better understanding of PPA.KEY WORDS: primary progressive aphasia, focal cortical degeneration, cerebral perfusion, neuroimaging. Afasia progressiva primária: análise de 16 casosRESUMO -A afasia progressiva primária (APP) é uma síndrome que tem despertado grande interesse devido a aspectos particulares que a diferenciam das afasias clássicas (secundárias a lesões cerebrais focais) e dos quadros demenciais. A deterioração lenta e progressiva da linguagem presente nesses casos fornece um interessante modelo de observação dos mecanismos subjacentes ao processamento linguístico. Descrevemos as características clínicas e de neuroimagem de 16 casos de APP. Os pacientes foram submetidos a exame de linguagem, neuropsicológico, ressonância magnética (RM) e tomografia computadorizada por emissão de fóton único (SPECT). Clinicamente pudemos observar uma nítida distinção nos padrões de produção oral, sendo os pacientes agrupados em fluentes e não-fluentes. Anomia foi o sintoma mais precoce e evidente nos dois subgrupos. Os achados de neuroimagem permitem destacar a sensibilidade do SPECT como instrumento diagnóstico. O registro e a comparação destes casos aos da literatura são uma tentativa de contribuir para a compreensão da APP. PALAVRAS-CHAVE: afasia progressiva primária, degeneração focal cortical, perfusão cerebral, neuroimagem.

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