Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies

@article{Sikorska2007SynucleinopathyWF,
  title={Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies},
  author={Beata Sikorska and Wielisław Papierz and Matthias Preusser and Pawel P. Liberski and H Budka},
  journal={Neuropathology and Applied Neurobiology},
  year={2007},
  volume={33}
}
Alpha-synuclein is a main component of neuronal inclusions in Parkinson’s disease, dementia with Lewy bodies (DLB) and glial and neuronal inclusions in multiple system atrophy (MSA) – as a group often referred to as alpha-synucleinopathies [1,2]. Dementia with Lewy bodies is the second most frequent neurodegenerative dementing disorder in the elderly after Alzheimer’s disease. Clinically, it is associated with fluctuating cognition and hallucinations in association with neurological features of… 
More frequent Lewy bodies but less frequent Alzheimer-type lesions in multiple system atrophy as compared to age-matched control brains
TLDR
The prevalence of Alzheimer-related pathologic features in synucleinopathies, tauopathies and frontotemporal degeneration in MSA was determined in this study according to postmortem criteria.
Multiple System Atrophy: An Oligodendroglioneural Synucleinopathy1
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  • Medicine, Psychology
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  • 2018
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Multidisciplinary research to elucidate the genetic and molecular background of the deleterious cycle of noxious processes, to develop reliable biomarkers and targets for effective treatment of this hitherto incurable disorder is urgently needed.
Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration: frontotemporal lobar degeneration associated with α-synuclein
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease clinically characterized by cerebellar signs, parkinsonism, and autonomic dysfunction. Pathologically, MSA is an
α-Synucleinopathy associated with G51D SNCA mutation: a link between Parkinson’s disease and multiple system atrophy?
TLDR
A British family with young-onset Parkinson’s disease and a G51D SNCA mutation that segregates with the disease and post-mortem brain examination of the proband revealed atrophy affecting frontal and temporal lobes, which could aid in understanding of α-synuclein biology and its impact on disease phenotype.
Neuropathology of multiple system atrophy: New thoughts about pathogenesis
  • K. Jellinger
  • Medicine, Biology
    Movement disorders : official journal of the Movement Disorder Society
  • 2014
TLDR
Histological core feature are glial cytoplasmic inclusions in all types of oligodendroglia that contain aggregates of misfolded α‐Synuclein (α‐Syn), which are suggested finally to lead to a system‐specific pattern of neurodegeneration.
Diversity of pathological features other than Lewy bodies in familial Parkinson's disease due to SNCA mutations.
TLDR
A diversity of pathological features of genetically determined familial PD are shown, and the presence of oligodendroglial inclusions at the light and electron microscopic levels in familial PD suggests that PD and multiple system atrophy form a continuum of α-synuclein pathology.
Etiology, Pathology, and Pathogenesis
TLDR
The correlation of subregional glial cytoplasmic inclusion (GCI) density and neuronal loss suggests that α-synuclein aggregation is tightly linked to selective neurodegeneration in MSA.
a-Synucleinopathy associated with G51D SNCA mutation: a link between Parkinson's disease and multiple system atrophy? Aoife P. KielyYasmine T. AsiEleanna KaraPatricia LimousinHelen Ling • Patrick LewisChristos ProukakisNiall QuinnAndrew J. LeesJohn Hardy •
We report a British family with young-onset Parkinson's disease (PD) and a G51D SNCA mutation that segregates with the disease. Family history was consistent with autosomal dominant inheritance as
Neuropathological findings in multiple system atrophy with cognitive impairment
TLDR
Mild-to-moderate CI, reported in 35.3% of MSA patients, being significantly older than those without CI, were frequently associated with cortical Alzheimer and Lewy pathologies, while only one with severe dementia had fully developed Alzheimer disease.
Screening for α-synuclein immunoreactive neuronal inclusions in the hippocampus allows identification of atypical MSA (FTLD-synuclein)
TLDR
It was shown that the evaluation of α-synuclein immunoreactive neuronal cytoplasmic inclusions (NCIs) in the hippocampus seems to be of great importance and aimed to determine if these morphological features inThe hippocampus are reliable to identify similar cases in the archives.
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TLDR
Clinical and pathological data derived from these new observations, as well as from literature cases, tend to suggest that this pathological association is of no particular clinical significance, and the comparison of the prevalence of Lewy bodies in normal elderly individuals and in multiple system atrophy suggests a chance association of the two pathologies.
Filamentous alpha-synuclein inclusions link multiple system atrophy with Parkinson's disease and dementia with Lewy bodies.
TLDR
These findings provide an unexpected link between multiple system atrophy and Lewy body disorders and establish that alpha-synucleinopathies constitute a major class of human neurodegenerative disorder.
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TLDR
Findings indicate that alpha-synuclein is also a major component of GCIs and NCIs in MSA and strongly suggest thatalpha- Synuclein aggregation is a common process in certain neurodegenerative diseases, including PD and MSA.
α-Synuclein immunoreactivity in glial cytoplasmic inclusions in multiple system atrophy
TLDR
Findings indicate that alpha-synuclein is also a major component of GCIs and NCIs in MSA and strongly suggest thatalpha- Synuclein aggregation is a common process in certain neurodegenerative diseases, including PD and MSA.
Association of Lewy bodies and glial cytoplasmic inclusions in the brain of Parkinson's disease
Abstract. We report the histopathological and immunohistochemical findings from the brain of an elderly patient diagnosed with Parkinson's disease (PD). Neuropathological examination revealed
Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy
TLDR
A 53-year-old Japanese woman who developed cerebellar ataxia and parkinsonism and was diagnosed as olivopontocerebellar atrophy may present a new variant of MSA since the neuronal inclusions are similar, in many respects, to those of neurons reported in MSA.
Diagnosis and management of dementia with Lewy bodies: Third report of the DLB Consortium
TLDR
It could be more appropriate, for a consensus statement, to conclude that finding EEG abnormalities does not exclude the diagnosis of LBD, instead of asserting that EEG abnormalities support LBD diagnosis.
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TLDR
H hippocampal involvement in MSA differing from ALS-D was clarified and loosely aggregated fibrils without limiting membrane located around the nucleus were confirmed by the results of ubiquitin-immunoelectron microscopy.
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Autonomic dysfunction is frequent in dementia with Lewy bodies and the severity is intermediate between that of multiple system atrophy and Parkinson disease.
[An autopsy case of long-course multiple system atrophy (MSA) with remarkable atrophy and numerous NCI in the temporal lobe].
TLDR
This case was a long-survived MSA with remarkable atrophy of the temporal lobe, and the characteristic neuropathological finding was numerous numbers of neurons containing NCI in the cotices arround the inferior horn.
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