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Chapter-20 Corticobasal Degeneration

BOOK TITLE: Understanding Parkinsonism: The Clinical Perspective

Author
1. Ling Helen
ISBN
9789386322333
DOI
10.5005/jp/books/14125_21
Edition
1/e
Publishing Year
2018
Pages
16
Author Affiliations
1. Reta Lila Weston Institute for Neurological Studies and Queen Square Brain Bank for Neurological Disorders; UCL Institute of Neurology Queen Square, London, United Kingdom
Chapter keywords
Corticobasal degeneration, CBD, corticobasal syndrome, CBS, corticobasal degeneration pathology, neuropathology, clinicopathological correlation, AD, Alzheimer disease, cerebrospinal fluid, CSF

Abstract

The aim of this chapter is to focus on the corticobasal degeneration. In 1968, corticobasal degeneration (CBD) was first described as a distinct clinicopathological entity by Rebeiz et al. Corticobasal syndrome (CBS) was initially described as the pathognomonic clinical presentation of CBD. This chapter covers the different clinical phenotypes associated with corticobasal degeneration pathology, neuropathology and clinicopathological correlations, epidemiology and natural history, clinical presentations, clinical diagnostic criteria and validation, clinical diagnostic approach and investigations, pitfalls in clinical diagnosis, genetics, and treatment. Treatment in CBD and related phenotypes is mainly symptomatic. Evidence-based trials are limited and treatment options suggested in this section are based on anecdotal experience.

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