Cognitive dysfunction in corticobasal degeneration

Laís Machado de Oliveira; Igor Barcellos; Hélio A.G. Teive; Renato Puppi Munhoz

doi:10.1590/0004-282X20170077

Arquivos de Neuro-Psiquiatria, Table of Contents

CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(08): 570-579
DOI: 10.1590/0004-282X20170077

VIEWS AND REVIEWS

Cognitive dysfunction in corticobasal degeneration

Disfunção cognitiva na degeneração corticobasal

Laís Machado de Oliveira

¹University Health Network, Toronto Western Hospital, Morton and Gloria Shulman Movement Disorders Centre and the Edmond J. Safra Program in Parkinson’s Disease, Toronto, ON, M5T 2S8, Canada.

,

Igor Barcellos

²Pontifícia Universidade Católica do Paraná, Hospital Universitário Cajuru, Serviço de Neurologia, Curitiba PR, Brasil;

,

Hélio A.G. Teive

³Universidade Federal do Paraná, Hospital de Clínicas, Departamento de Medicina Interna, Serviço de Neurologia, Setor de Distúrbios do Movimento, Curitiba, PR, Brasil.

,

Renato Puppi Munhoz

¹University Health Network, Toronto Western Hospital, Morton and Gloria Shulman Movement Disorders Centre and the Edmond J. Safra Program in Parkinson’s Disease, Toronto, ON, M5T 2S8, Canada.

› Author Affiliations

Abstract

ABSTRACT

Corticobasal degeneration (CBD) was originally described as a distinct clinicopathological entity in 1967. Since then, different phenotypic presentations have emerged as possible manifestations of CBD histopathological findings. In addition, pathophysiological findings and the molecular basis have been delineated and several aspects of its cognitive manifestations have been clarified. Thus, not only the spectrum of what is currently designated as CBD has expanded, but overlap with other degenerative and even secondary disorders has made clinical diagnostic certainty even more challenging in the absence of specific and readily-available markers. Cognitive deficits in CBD are now recognized as a frequent initial presentation and may appear up to eight years before the motor symptoms, depending on the phenotypic variant. Characteristic cognitive features of CBD involve language deficits, visuospatial and executive dysfunctions, apraxia, and behavioral disorders. Semantic and episodic memories are usually preserved, while language is often impaired in the early stages.

RESUMO

A degeneração corticobasal (DCB) foi originalmente descrita como uma entidade clínico-patológica distinta em 1967. Desde então, nossa compreensão sobre DCB evoluiu substancialmente. Diferentes apresentações fenotípicas emergiram refletindo possíveis manifestações das anormalidades histopatológicos da DCB. Adicionalmente, dados fisiopatológicos e moleculares foram delineados e aspectos das manifestações cognitivas foram explorados. Assim, não só o espectro do que é atualmente designado DCB foi expandido, mas a sobreposição com outras doenças degenerativas e até mesmo secundárias tornaram o diagnóstico clínico ainda mais desafiador na ausência de marcadores específicos e prontamente disponíveis. Déficits cognitivos na DCB são agora reconhecidos frequentemente como apresentações iniciais e podem surgir até 8 anos antes dos sintomas motores, dependendo da variante fenotípica. O quadro cognitivo envolve característicamente déficits de linguagem, disfunção visuoespacial e executiva, apraxia, e distúrbios comportamentais. Anormalidades da linguagem são frequentemente descritas nos estágios iniciais da DCB.

dementia - cognition - corticobasal degeneration

demência - cognição - degeneração corticobasal

Full Text

References

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