Corticobasal Ganglionic Degeneration

This chapter focuses on the corticobasal ganglionic degeneration (CBD) and describes the clinical features suggestive of the pathological diagnosis of CBD. CBD is a relatively rare neurodegenerative disease. The clinical features associated with CBD consist of any combination of the following signs and symptoms: action and postural tremor, rarely rest tremor, bra­dykinesia, myoclonus, ideomotor apraxia, alien limb phenomena, limb dystonia, gait impairment, dysarthria, aphasia, apraxia of speech, asymmetrical parkinsonism cognitive impairment, neuropsychiatric features, and dementia. The features of CBD, regardless of whether they are predominantly motor or predominantly cognitive, tend to be strongly asymmetrical. The clinical features suggestive of CBD in the absence of pathological confirmation are referred to as “corticobasal syndrome” (CBS). The chapter also discusses the diagnostic criteria for CBD, diagnostic investigations of CBD, macroscopic and microscopic findings of CBD, and pathology and genetics of CBD.