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Corticobasal degeneration 

Corticobasal degeneration
Chapter:
Corticobasal degeneration
Author(s):

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

DOI:
10.1093/med/9780192619112.003.0391
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date: 28 May 2020

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.

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