Corticobasal degeneration

Ivan Donaldson, C. David Marsden, Susanne A. Schneider and Kailash P. Bhatia

in Marsden's Book of Movement Disorders

Published on behalf of Oxford University Press

ISBN: 9780192619112
Published online January 2012 | e-ISBN: 9780199698103 | DOI:
Corticobasal degeneration

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CBD is a progressive disorder of late life characterized usually by the insidious asymmetric onset of apraxia, rigidity, tremor, myoclonus, and dystonia of the limbs, associated with sensory loss and sometimes pain, alien limb behaviour, frontal release, and pyramidal signs. The condition evolves into an akinetic–rigid syndrome, unresponsive to levodopa, with disequilibrium and pseudobulbar symptoms, often with a supranuclear gaze palsy, with some cognitive and language function involvement, although this aspect may be relatively preserved early on. Death occurs within a decade. The characteristic pathology consists of focal, asymmetric frontal, and parietal cortical and subcortical atrophy, associated with gliosis and degeneration marked by pale swollen neurons. With advances in molecular pathology CBD is a now known to be fairly distinct lobar and basal ganglia tauopathy with selective aggregation of four repeat tau (4R tau) protein with characteristic antigenic and ultrastructural characteristics. The cause is unknown and usually there is no evidence of inheritance, although there seems to be a suggestion of a genetic propensity.

Chapter.  13091 words.  Illustrated.

Subjects: Neurology

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