Corticobasal Degeneration: clinical and radiological aspects

Degeneração Corticobasal: aspectos clínico-radiológicos

Abstract

Corticobasal degeneration is a rare and slowly progressive neurodegenerative disorder that generally presents as atypical parkinsonism with early cognitive features. Classically, motor symptoms occur asymmetrically and include akinetic-rigid parkinsonism with dystonia and myoclonic jerks. Cognitive manifestations include apraxia, aphasia, cortical sensory deficits, and alien limb phenomenon. The terms corticobasal syndrome (CBS) and corticobasal degeneration (CBD) represent distinct conditions. The former denotes the clinical phenotype, whereas CBD can generally be confirmed by postmortem anatomopathological analysis. In clinical practice, it is often difficult to make a proper diagnosis because autopsy is rarely performed. Therefore, it would be desirable to develop biomarkers, such as neuroimaging, in this group of patients to establish a more accurate diagnosis. The data in the literature regarding magnetic resonance findings in CBS/CBD are conflicting; however, they remain the most widely used in clinical practice due to their greater availability. This report presents five cases of CBS, assessing clinical progress through motor and cognitive findings, correlated with neuroimaging.