Corticobasal Degeneration CBD

CBD is a rare disorder that presents clinically as an asymmetric, akinetic rigid syndrome with apraxia (impaired voluntary execution of symbolic movements and gestures), myoclonus, and sometimes dementia. When dementia is present, the pattern of neuropsy-chological test performance resembles that of PSP, except that apraxia is typically more severe in CBD. Symptoms of cortical sensory loss (e.g., impaired visual or tactile discrimination) are also common early in CBD. Persons with CBD often show the "alien limb'' syndrome, in which the individual reports that their limb is moving without their volitional control, and often are mildly depressed. CBD is associated with markedly asymmetric patterns of cortical metabolism alteration (as measured by PET). Cortical metabolism contralateral to the affected limb is typically lower in inferior parietal, lateral temporal, and sensory motor cortices than that seen in PSP.

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