scholarly journals Post mortem cerebrospinal fluid α-synuclein levels are raised in multiple system atrophy and distinguish this from the other α-synucleinopathies, Parkinson's disease and Dementia with Lewy bodies

2012 ◽  
Vol 45 (1) ◽  
pp. 188-195 ◽  
Author(s):  
P.G. Foulds ◽  
O. Yokota ◽  
A. Thurston ◽  
Y. Davidson ◽  
Z. Ahmed ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Cerebrospinal Fluid ◽  
Multiple System Atrophy ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
The Other ◽  
Post Mortem

scholarly journals Structures of α-synuclein filaments from multiple system atrophy

2020 ◽  
Author(s):  
Manuel Schweighauser ◽  
Yang Shi ◽  
Airi Tarutani ◽  
Fuyuki Kametani ◽  
Alexey G. Murzin ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Human Brain ◽  
Multiple System Atrophy ◽  
Recombinant Proteins ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Two Dimensional ◽  
Filamentous Inclusions

Synucleinopathies are human neurodegenerative diseases that include multiple system atrophy (MSA), Parkinson’s disease, Parkinson’s disease dementia (PDD) and dementia with Lewy bodies (DLB) (1). Existing treatments are at best symptomatic. These diseases are characterised by the presence in brain cells of filamentous inclusions of α-synuclein, the formation of which is believed to cause disease (2, 3). However, the structures of α-synuclein filaments from human brain are not known. Here we show, using electron cryo-microscopy, that α-synuclein inclusions from MSA are made of two types of filaments, each of which consists of two different protofilaments. Non-proteinaceous molecules are present at the protofilament interfaces. By two-dimensional class averaging, we show that α-synuclein filaments from the brains of patients with MSA and DLB are different, suggesting that distinct conformers (or strains) characterise synucleinopathies. As was the case of tau assemblies (4–9), the structures of α-synuclein filaments extracted from the brains of individuals with MSA differ from those formed in vitro using recombinant proteins, with implications for understanding the mechanisms of aggregate propagation and neurodegeneration in human brain. These findings have diagnostic and potential therapeutic relevance, especially in view of the unmet clinical need to be able to image filamentous α-synuclein inclusions in human brain.

scholarly journals Clinical Manifestations of Parkinson's Disease and Parkinsonism

2003 ◽  
Vol 30 (S1) ◽  
pp. S2-S9 ◽  
Author(s):  
Doug Everett Hobson
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Differential Diagnosis ◽  
Multiple System Atrophy ◽  
Dementia With Lewy Bodies ◽  
Clinical Manifestations ◽  
Lewy Bodies ◽  
Accurate Diagnosis ◽  
Additional Information ◽  
Diagnosis And Prognosis

The most common disorder in a patient presenting to a movement disorder clinic will be parkinsonism. The challenge is to provide the patient with the most accurate diagnosis and prognosis possible. The assumption at the time of initial presentation of the clinical diagnosis of Parkinson's disease is often wrong (20-25%). Waiting to see the pattern of progression, and response to medication provides invaluable additional information. This manuscript summarizes the clinical manifestations of Parkinson's disease and the main akinetic-rigid syndromes (progressive supranuclear palsy, multiple system atrophy, cortical-basal ganglionic degeneration, and dementia with Lewy bodies) that make up the differential diagnosis.

Filamentous α-synuclein inclusions link multiple system atrophy with Parkinson's disease and dementia with Lewy bodies

1998 ◽  
Vol 251 (3) ◽  
pp. 205-208 ◽  
Author(s):  
Maria Grazia Spillantini ◽  
R. Anthony Crowther ◽  
Ross Jakes ◽  
Nigel J. Cairns ◽  
Peter L. Lantos ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Multiple System Atrophy ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies
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