Definition: a rare, progressive disease (considered by some a variant of Parkinson disease) involving both cerebral cortex and extrapyramidal structures; clinically manifest as disturbances of voluntary movements and rigidity; pathologic characteristics include degeneration of the cerebral cortex with balloon neurons and degeneration of the substantia nigra; apraxia, cortical sensory loss, myoclonus, and phantom limb syndrome have been reported.
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