[Corticobasal degeneration: clinical and neuropsychological profile]. / La dégénérescence corticobasale : aspects cliniques et neuropsychologiques.

ABSTRACT

Corticobasal degeneration (CBD) is a rare neurodegenerative disorder, primarily defined on clinical and pathological grounds. CBD may present with a large set of cognitive and motor symptoms in relation with asymmetrically distributed lesions in the fronto-parietal cortex and basal ganglia. CBD has long been considered as a motor system disease, with a prominent alteration of movements and gestures. In recent years, however, cognitive and behavioral disturbances have increasingly been recognized. In some instances, they are the prevalent manifestations of the disorder, leading to diagnostic misclassifications and uncertainties. This article reviews the evolution of the nosological concept of CBD across the past decade, along with describing the main disturbances involving cognition, behavior, body and gesture representations that may occur throughout the course of this illness.