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Cognitive impairment in multiple system atrophy: Changing concepts

Comprometimento cognitivo na atrofia de múltiplos sistemas: mudando os conceitos

Abstract

Multiple system atrophy (MSA) is characterized by a variable combination of cerebellar ataxia, parkinsonism and pyramidal signs associated with autonomic failure. Classically, cognitive impairment was not considered a clinical feature of MSA and dementia was pointed out as an exclusion diagnostic criteria. Based on comprehensive neuropsychological assessment, cognitive impairment was found to be a frequent feature in MSA, and clinically-defined dementia is now reported in 14-16% of cases. This article reviews the current data on cognitive impairment in MSA along with its neuropsychological profile and pathophysiology.

Key words:
multiple system atrophy; cognitive impairment; dementia; atypical parkinsonism; neuropsychological evaluation.

Resumo

A Atrofia de Múltiplos Sistemas (AMS) é caracterizada por uma combinação variável de ataxia cerebelar, parkinsonismo e sinais piramidais, associados à disfunção autonômica. Classicamente, o comprometimento cognitivo não é considerado como manifestação clínica da AMS e a demência é apontada como um critério de exclusão para o diagnóstico. Baseado na avaliação neuropsicológica ampla, o comprometimento neuropsicológico revelou-se frequente na AMS e a presença de demência é atualmente descrita em 14-16% dos casos. Este artigo revisa os dados atuais sobre o comprometimento cognitivo na AMS, seu perfil neuropsicológico e fisiopatologia.

Palavras-chave:
atrofia de múltiplos sistemas; comprometimento cognitivo; demência; parkinsonismo atípico; avaliação neuropsicológica.

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Publication Dates

  • Publication in this collection
    Oct-Dec 2011

History

  • Received
    03 July 2011
  • Accepted
    02 Nov 2011
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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