Clinical Syndrome of Corticobasal Degeneration

ABSTRACT

Clinical features of corticobasal degeneration (CBD) can be characterized by an asymmetric akinetic-rigid syndrome with variable combinations of other deficits (corticospinal tract signs, supranuclear gaze palsy, cerebellar ataxia, cortical sensory loss, alien limb behavior, dystonia and myoclonus). Such unique combination of clinical features of CBD have lead to the general agreement that clinical diagnosis of CBD is reliable. We describe 6 patients presenting with clinical features compatible with CBD. Three had characteristic clinical features of CBD; one showed clinical features compatible with progressive supranuclear palsy, but also had apraxia and cortical sensory disturbances; one had an early CBD. The remaining one had clinical features compatible with CBD, but brain magnetic resonance imaging study showed multiple small lesions involving periventricular white matter and basal ganglia bilaterally.