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Review: The spectrum of clinical features seen with alpha synuclein pathology.

Accepted version
Peer-reviewed

Repository DOI


Type

Article

Change log

Authors

Barker, RA 
Williams-Gray, CH 

Abstract

It has been recognized for many years that a number of chronic neurodegenerative diseases of the CNS are characterized by the development of intracellular inclusion bodies, but it is only relatively recently that the core proteins defining these pathologies have been defined. One such protein is alpha synuclein, that was found to be the main component of Lewy bodies in the late 1990s, and this discovery reinforced the emerging view that alpha synuclein was intimately linked to diseases characterized by this type of pathology--namely Parkinson's disease (PD) and Dementia with Lewy Bodies (DLB). Furthermore at around this time, this same protein was also found within the glial inclusion bodies of patients dying with multiple system atrophy (MSA). These three disorders constitute the majority of patients with an 'alpha synucleinopathy', although there are a number of rarer conditions that can also cause this pathology including inherited metabolic disorders such as Gaucher's disease (GD). In this review, we will concentrate on PD, the commonest alpha synucleinopathy, and its associated dementia (PDD), as well as discussing DLB and MSA and will highlight how the clinical features of these conditions vary as a function of pathology.

Description

Keywords

Parkinson's disease, alpha synucleinopathies, Humans, Lewy Body Disease, Multiple System Atrophy, Parkinson Disease, alpha-Synuclein

Journal Title

Neuropathol Appl Neurobiol

Conference Name

Journal ISSN

0305-1846
1365-2990

Volume Title

42

Publisher

Wiley
Sponsorship
Medical Research Council (G0001354)
Medical Research Council (G1000183)
Wellcome Trust (093875/Z/10/Z)
Our own work on the natural history of Parkinson’s disease has been supported by the Wellcome Trust; MRC; Patrick Berthoud Trust; Cure Parkinson’s Trust; Parkinson’s UK and the Cambridge NIHR Biomedical Research Centre.