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Corticobasal degeneration

Authors
Stewart A Factor, DO
Deborah G Walls, DNP, APRN-BC
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

Corticobasal degeneration (CBD) is a rare neurodegenerative disorder that poses significant challenges to clinical diagnosis and treatment. The classic description of CBD is that of a progressive asymmetric movement disorder characterized by various combinations of akinesia, rigidity, dystonia, focal myoclonus, ideomotor apraxia, and alien-limb phenomena. However, a wider range of clinical presentations is increasingly apparent, including onset with cognitive or behavioral abnormalities.

This topic will review clinical aspects of CBD. Other atypical parkinsonian disorders are reviewed elsewhere. (See "Progressive supranuclear palsy (PSP)" and "Multiple system atrophy: Clinical features and diagnosis".)

HISTORICAL ASPECTS AND NOMENCLATURE

The first description of corticobasal degeneration (CBD) in 1967 reported the disorder in three patients who had an asymmetric, akinetic-rigid neurodegenerative syndrome with cerebral cortical dysfunction [1,2]. The original name given to this was "corticodentatonigral degeneration with neuronal achromasia," based upon its neuropathologic appearance. It would be another two decades before the next series of six patients was described [3]. However, as subsequent cases were evaluated, it became apparent that the dentate pathology was infrequent and the nomenclature evolved to the more accurate terminology of corticobasal or corticobasal ganglionic degeneration [4,5].

It is now recognized that pathologically proven CBD often begins as a cognitive or behavioral disturbance. Additionally, the cognitive and motor features considered characteristic of CBD are not specific to CBD, but may occur with other pathologically proven neurodegenerative disorders, including progressive supranuclear palsy, frontotemporal dementia, and Alzheimer disease [4,6-8]. This has prompted some experts to the use the term "corticobasal syndrome" (CBS) for cases with a clinical diagnosis, while reserving "corticobasal degeneration" for cases with neuropathologic confirmation.

A fundamental abnormality of the cytoskeletal protein tau is the underlying pathologic substrate, which has led to CBD being labeled as a tauopathy. Other tauopathies include Alzheimer disease, Pick disease (frontotemporal dementia), and progressive supranuclear palsy [9,10]. The shared tau protein pathology may explain the overlap of clinical symptoms and the complex neuropathology present in these diseases.

                      

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Literature review current through: Nov 2016. | This topic last updated: Wed Jun 15 00:00:00 GMT+00:00 2016.
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