New review on the manifestation of synucleinopathies...
Neuropathol Appl Neurobiol. 2016 Jan 10. doi: 10.1111/nan.12303. [Epub ahead of print]
Barker RA, Williams-Gray CH.
It has been recognised for many years that a number of chronic neurodegenerative diseases of the CNS are characterised 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 characterised by this type of pathology - namely Parkinson's disease (PD) and Lewy body dementia (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.
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