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Parkinsonism Relat Disord. 2014 Jan;20 Suppl 1:S62-7. doi: 10.1016/S1353-8020(13)70017-8.

α-Synucleinopathy phenotypes.

Author information

1
Neuroscience Research Australia, Sydney, Australia.

Abstract

α-Synucleinopathies are neurodegenerative diseases characterised by the abnormal accumulation of α-synuclein aggregates in neurons, nerve fibres or glial cells. While small amounts of these α-synuclein pathologies can occur in some neurologically normal individuals who do not have associated neurodegeneration, the absence of neurodegeneration in such individuals precludes them from having a degenerative α-synucleinopathy, and it has yet to be established whether such individuals have a form of preclinical disease. There are three main types of α-synucleinopathy, Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), with other rare disorders also having α-synuclein pathologies, such as various neuroaxonal dystrophies. Multiple clinical phenotypes exist for each of the three main α-synucleinopathies, with these phenotypes differing in the dynamic distribution of their underlying neuropathologies. Identifying the factors involved in causing different α-synuclein phenotypes may ultimately lead to more targeted therapeutics as well as more accurate clinical prognosis.

KEYWORDS:

Dementia with Lewy bodies; Multiple system atrophy; Parkinson's disease; α-Synuclein

PMID:
24262191
DOI:
10.1016/S1353-8020(13)70017-8
[Indexed for MEDLINE]

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