Multiple system atrophy

Poewe, Werner; Stanković, Iva; Halliday, Glenda M.; Meissner, Wassilios G.; Wenning, Gregor K.; Pellecchia, Maria Teresa; Seppi, Klaus; Palma, Jose‐Alberto

doi:10.1038/s41572-022-00382-6

Cited by 99 publications

(93 citation statements)

References 280 publications

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“…MSA is a devastating, adult-onset NDD in which multiple neuronal pathways degenerate, causing a multifaceted clinical presentation, including parkinsonism, cerebellar impairment, and autonomic dysfunctions in various combinations ( Fanciulli and Wenning, 2015 ; Poewe et al, 2022 ). The pathological hallmark of MSA is the presence of glial cytoplasmic inclusions (GCIs), predominantly in OLs ( Papp et al, 1989 ).…”

Section: Ol and Myelin Dysfunction In Nddsmentioning

confidence: 99%

“…Accumulation of α-Syn in OLs has been shown to cause demyelination, iron overload, and exacerbate autophagy impairment ( Poewe et al, 2022 ). Interestingly, in contrast to the severe neuronal loss, previous studies have reported that MSA brains show only a modest reduction in the number of OLs ( Salvesen et al, 2015 ; Nykjær et al, 2017 ), suggesting that cellular dysfunction of OLs, and not necessarily cell death, induced by GCIs may be sufficient to cause neurodegeneration.…”

Section: Ol and Myelin Dysfunction In Nddsmentioning

confidence: 99%

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Functions and dysfunctions of oligodendrocytes in neurodegenerative diseases

Han

Gim

Jang

et al. 2022

Front. Cell. Neurosci.

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Neurodegenerative diseases (NDDs) are characterized by the progressive loss of selectively vulnerable populations of neurons, which is responsible for the clinical symptoms. Although degeneration of neurons is a prominent feature that undoubtedly contributes to and defines NDD pathology, it is now clear that neuronal cell death is by no means mediated solely by cell-autonomous mechanisms. Oligodendrocytes (OLs), the myelinating cells of the central nervous system (CNS), enable rapid transmission of electrical signals and provide metabolic and trophic support to neurons. Recent evidence suggests that OLs and their progenitor population play a role in the onset and progression of NDDs. In this review, we discuss emerging evidence suggesting a role of OL lineage cells in the pathogenesis of age-related NDDs. We start with multiple system atrophy, an NDD with a well-known oligodendroglial pathology, and then discuss Alzheimer’s disease (AD) and Parkinson’s disease (PD), NDDs which have been thought of as neuronal origins. Understanding the functions and dysfunctions of OLs might lead to the advent of disease-modifying strategies against NDDs.

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Section: Ol and Myelin Dysfunction In Nddsmentioning

confidence: 99%

Section: Ol and Myelin Dysfunction In Nddsmentioning

confidence: 99%

Functions and dysfunctions of oligodendrocytes in neurodegenerative diseases

Han

Gim

Jang

et al. 2022

Front. Cell. Neurosci.

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“…Varied clinical presentations are the results of different pathologies including striatonigral, olivopontocerebellar, and central autonomic degeneration. Clinically, MSA is mainly divided into two subtypes, the parkinsonism subtype (MSA-P) and the cerebellar subtype (MSA-C), according to the predominant motor symptom or the onset sign ( Krismer and Wenning, 2017 ; Poewe et al, 2022 ). The prominent pathological feature of MSA is oligodendroglia cytoplasmic inclusions (GCIs) mainly composed of α-synuclein ( Ahmed et al, 2012 ; Koga et al, 2021 ), which is also the essential histologic hallmark for diagnosis of definite MSA ( Gilman et al, 2008 ).…”

Section: Introductionmentioning

confidence: 99%

Serum vitamin levels in multiple system atrophy: A case-control study

Chen

Wan

Chen

et al. 2023

Front. Aging Neurosci.

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AimThere is increasing evidence suggesting that vitamins may play important roles in the pathogenesis of multiple system atrophy (MSA). The purpose of this study was to detect the changes of serum vitamin levels and investigate their correlation with disease severity in MSA patients.MethodsIn this cross-sectional study, 244 MSA patients, 200 Parkinson’s disease (PD) patients and 244 age-gender matched healthy controls were recruited. Serum vitamin levels were measured, including vitamin A, B1, B2, B9 (folate), B12, C, D, and E. Relevant clinical scales were used to assess the disease severity of MSA patients.ResultsCompared with the healthy controls, decreased serum folate levels and increased serum vitamin A and C levels were detected in MSA patients. Similar differences were also observed in the gender-based subgroup analysis. There were no differences detected between MSA and PD patients. In MSA patients, significant correlation was found between vitamin A, folate, or vitamin C and relevant clinical scales or laboratory findings. In addition, ROC analysis showed potential diagnostic value of the combination of vitamin A, folate, and vitamin C in distinguishing MSA patients from healthy controls.ConclusionThere were significant changes in the blood vitamin spectrums of MSA patients, suggesting that dysregulation of vitamins homeostasis might play an important role in the pathogenesis of MSA.

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“…Medications are often unable or insufficient to alleviate motor symptoms, and they may worsen nonmotor symptoms, especially neurogenic orthostatic hypotension (nOH). 1 The combination of these factors contributes to falls and leads to restricted ambulation. The only US Food and Drug Administration-approved treatments for orthostatic hypotension are midodrine and droxidopa (the latter being only available in the United States and Japan).…”

mentioning

confidence: 99%

“…MSA is rapidly progressive, leading patients to be wheelchair bound within 5 to 6 years of diagnosis. Medications are often unable or insufficient to alleviate motor symptoms, and they may worsen nonmotor symptoms, especially neurogenic orthostatic hypotension (nOH) 1 . The combination of these factors contributes to falls and leads to restricted ambulation.…”

mentioning

confidence: 99%

Epidural Spinal Cord Stimulation to Treat Neurogenic Orthostatic Hypotension in Multiple System Atrophy: A New Hope?

Vernetti

2022

Movement Disord Clin Pract

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Multiple system atrophy

Cited by 99 publications

References 280 publications

Functions and dysfunctions of oligodendrocytes in neurodegenerative diseases

Functions and dysfunctions of oligodendrocytes in neurodegenerative diseases

Serum vitamin levels in multiple system atrophy: A case-control study

Epidural Spinal Cord Stimulation to Treat Neurogenic Orthostatic Hypotension in Multiple System Atrophy: A New Hope?

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