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Update on Corticobasal Degeneration

From Bradley F. Boeve, MD, Rochester, MN Corticobasal degeneration (CBD), also known as corticobasal ganglionic degeneration (CBGD), was first described in the late 1960’s by Drs. Rebeiz, Kolodny, and Richardson. Following a lengthy period with no additional reports, several more patients were identified and their symptoms and autopsy findings were described in the 1980’s and 1990’s. Patients typically have symptoms reflecting dysfunction in the cerebral cortex (thus the term “cortical” or “cortico-”) and basal ganglia (thus the terms “basal” or “basal ganglionic”), and symptoms are usually worse on one side of the body. Specifically, cortical dysfunction is manifested as poor coordination of the arms or legs (apraxia), tendency for the arm “to act as if it has a mind of its own” (alien limb phenomenon), numbness or odd sensations (cortical sensory loss), poor comprehension and/or expression of language (aphasia), and quick jerks (myoclonus). Slowness of movement (bradykinesia), stiffness in a limb (rigidity), fixed muscle contractions such as when the fingers curl into a fist (dystonia), and tremor are presumed to reflect basal ganglia dysfunction. Some patients develop memory impairment and/or personality/behavioral changes. Problems with walking eventually occur in almost all. In our studies the duration of illness from onset of symptoms to death has ranged from 3-13 years. The vast majority of patients do not appear to have any family history of dementia or parkinsonism, although there are rare cases in whom a hereditary process may be at play. The cause of CBD is not yet known.

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