Review: The spectrum of clinical features seen with alpha synuclein pathology
Neuropathology and Applied Neurobiology
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Barker, R., & Williams-Gray, C. (2016). Review: The spectrum of clinical features seen with alpha synuclein pathology. Neuropathology and Applied Neurobiology, 42 6-19. https://doi.org/10.1111/nan.12303
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.
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.
MEDICAL RESEARCH COUNCIL (G0001354)
Wellcome Trust (093875/Z/10/Z)
External DOI: https://doi.org/10.1111/nan.12303
This record's URL: https://www.repository.cam.ac.uk/handle/1810/253259