corticobasal degeneration


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corticobasal degeneration

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.

corticobasal degeneration

A rare idiopathic cerebrodegenerative “tauopathy” which is difficult to diagnose in vivo (less than 25% are correctly diagnosed), as many patients carry the diagnosis of progressive supranuclear palsy whilst alive. Of those with a clinical diagnosis of corticobasal degeneration in vivo, 75% had another diagnosis at autopsy; again, many had progressive supranuclear palsy.

cor·ti·co·ba·sal de·gen·er·a·tion

(kōr'ti-kō-bā'săl dĕ-jen'ĕr-ā'shŭn)
A rare, progressive disease involving both cerebral cortex and extrapyramidal structures; clinically manifested as disturbances of voluntary movements and rigidity; pathologic characteristics include degeneration of the cerebral cortex with balloon neurons and degeneration of the substantia nigra.

corticobasal degeneration

A neurological disorder in which brain cells atrophy and die in the basal ganglia and the cortex of the brain. The disease produces symptoms similar to those found in Parkinson's disease but does not respond to parkinsonian medications.
See also: degeneration
References in periodicals archive ?
c) Atypical variant of corticobasal degeneration with prominent olivopontocerebellar atrophy presents with predominant atrophy of hindbrain structures.
Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau.
A couple of years ago, after Karl was diagnosed with something terrible-sounding called Corticobasal Degeneration, and he could still walk and talk, he took me deep into the woods of his hundred acres to see where his son Karlchen is buried.
Abstract: The Parkinson-plus syndromes, which include multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD), are still not well-known.