Multiple system atrophy: cure and care

Multiple system atrophy (MSA) is a rare and rapidly progressive neurodegenerative disorder characterized by a variable combination of autonomic failure, parkinsonism, and cerebellar ataxia, with a median survival of 8-10 years from symptom onset. Its aetiology remains poorly understood, as most cases are sporadic and environmental contributors remain unclear. Neuropathologically, MSA is defined by the accumulation of α-synuclein within oligodendroglial cells, forming glial cytoplasmic inclusions that drive widespread neurodegeneration in striatonigral and olivopontocerebellar systems. Recent advances in diagnostic criteria and biomarker development have improved disease recognition; however, early diagnosis remains challenging, particularly during the prodromal phase, when clinical features overlap with other α-synucleinopathies. Advances in fluid biomarkers and multimodal imaging are expected to facilitate earlier detection, improve diagnostic accuracy, and provide more robust tools for monitoring disease progression. Therapeutic development has largely focused on targeting α-synuclein pathology, but these approaches have not yet demonstrated consistent clinical benefit, highlighting the biological complexity of MSA. In the absence of curative treatments, management remains largely supportive, aimed at symptom control, particularly addressing autonomic dysfunction and motor impairment. Emerging care models, including telemedicine and multidisciplinary management, are reshaping clinical practice and may improve access to specialized care. Bridging advances in pathophysiology with patient-centred care will be essential for improving outcomes in MSA.

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Publication Details

Journal
Journal of Neural Transmission
Published
2026-07-25
DOI
https://doi.org/10.1007/s00702-026-03235-8
Primary Topic
Parkinson's Disease Mechanisms and Treatments
Type
article
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article

Multiple system atrophy: cure and care

Fabian Leys, Franziska Hopfner, Bianca Caliò, Günter U. Höglinger et al.
Journal of Neural Transmission
Parkinson's Disease Mechanisms and Treatments
article

Multiple system atrophy: cure and care

Fabian Leys, Franziska Hopfner, Bianca Caliò, Günter U. Höglinger, Andrea Quattrone, Jolanda Buonocore, Alessandra Fanciulli, Nadia Stefanova
article en

Abstract

Multiple system atrophy (MSA) is a rare and rapidly progressive neurodegenerative disorder characterized by a variable combination of autonomic failure, parkinsonism, and cerebellar ataxia, with a median survival of 8-10 years from symptom onset. Its aetiology remains poorly understood, as most cases are sporadic and environmental contributors remain unclear. Neuropathologically, MSA is defined by the accumulation of α-synuclein within oligodendroglial cells, forming glial cytoplasmic inclusions that drive widespread neurodegeneration in striatonigral and olivopontocerebellar systems. Recent advances in diagnostic criteria and biomarker development have improved disease recognition; however, early diagnosis remains challenging, particularly during the prodromal phase, when clinical features overlap with other α-synucleinopathies. Advances in fluid biomarkers and multimodal imaging are expected to facilitate earlier detection, improve diagnostic accuracy, and provide more robust tools for monitoring disease progression. Therapeutic development has largely focused on targeting α-synuclein pathology, but these approaches have not yet demonstrated consistent clinical benefit, highlighting the biological complexity of MSA. In the absence of curative treatments, management remains largely supportive, aimed at symptom control, particularly addressing autonomic dysfunction and motor impairment. Emerging care models, including telemedicine and multidisciplinary management, are reshaping clinical practice and may improve access to specialized care. Bridging advances in pathophysiology with patient-centred care will be essential for improving outcomes in MSA.

Journal of Neural Transmission
Innsbruck Medical University (AT), Universität Innsbruck (AT), German Center for Neurodegenerative Diseases (DE), LMU Klinikum (DE), Magna Graecia University (IT), Munich Cluster for Systems Neurology (DE), Ludwig-Maximilians-Universität München (DE)
Good health and well-being
Openalex Percentile: Top 8%
Parkinson's Disease Mechanisms and Treatments
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