Lewy body diseases and multiple system atrophy as α-synucleinopathies
نویسندگان
چکیده
منابع مشابه
Pathology of Neuro-glial Α-synucleinopathy (lewy Body Disease and Multiple System Atrophy)
α-Synucleinopathies comprise a group of neurodegenerative disorders that share α-synuclein (αS) accumulation in selected vulnerable neurons and glia, i.e. Parkinson’s disease (PD), dementia with Lewy bodies and multiple system atrophy (MSA). The histological hallmark of PD is neuronal αS aggregates called Lewy bodies (LBs). LB formation has been considered to be a marker for neuronal degenerati...
متن کاملPost 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
Differentiating clinically between Parkinson's disease (PD) and the atypical parkinsonian syndromes of Progressive supranuclear palsy (PSP), corticobasal syndrome (CBS) and multiple system atrophy (MSA) is challenging but crucial for patient management and recruitment into clinical trials. Because PD (and the related disorder Dementia with Lewy bodies (DLB)) and MSA are characterised by the dep...
متن کاملAccumulation of NACP/alpha-synuclein in lewy body disease and multiple system atrophy.
OBJECTIVES NACP/alpha-synuclein is an aetiological gene product in familial Parkinson's disease. To clarify the pathological role of NACP/alpha-synuclein in sporadic Parkinson's disease and other related disorders including diffuse Lewy body disease (DLBD) and multiple system atrophy (MSA), paraffin sections were examined immunocytochemically using anti-NACP/alpha-synuclein antibodies. METHOD...
متن کاملAlpha-synuclein accumulates in Purkinje cells in Lewy body disease but not in multiple system atrophy.
Alpha-synuclein has an important role in the pathogenesis of Parkinson disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), comprising a new disease concept, that of alpha-synucleinopathies. Cerebellar degeneration with Purkinje cell depletion is present in the majority of MSA cases. By contrast, cerebellar pathology has not been demonstrated unequivocally in either...
متن کاملMolecular Pathology of Lewy Body Diseases
Lewy body diseases are characterized by the presence of Lewy bodies, alpha-synuclein(AS)-positive inclusions in the brain. Since their main component is conformationally modified AS, aggregation of the latter is thought to be a key pathogenic event in these diseases. The analysis of inclusion body constituents gives additional information about pathways also involved in the pathology of synucle...
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ژورنال
عنوان ژورنال: Molecular Psychiatry
سال: 1998
ISSN: 1359-4184,1476-5578
DOI: 10.1038/sj.mp.4000458