ArticleNeuropathology and applied neurobiology2017
Progression of alpha-synuclein pathology in multiple system atrophy of the cerebellar type.
Article in Neuropathology and applied neurobiology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers, 1 of them a synthesis that pooled it.
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Who cites it
43 citing papers in PubMed, 1 synthesis or guideline pooled it, 63 citations in OpenAlex.
- Tau-PET imaging in Parkinson's disease: a systematic review and meta-analysis.Frontiers in neurology · 2023Pooled it
- Neural Correlates of Apathy in Multiple System Atrophy: A Clinical and Perfusion Imaging Study.European journal of neurology · 2026Article
- ActiTect: a generalizable machine learning pipeline for REM sleep behavior disorder screening through standardized actigraphy.NPJ digital medicine · 2026Article
- Deciphering Freezing of Gait: What Neuropathology Reveals About an Episodic Phenomenon.The European journal of neuroscience · 2026Review
- Revisiting 'hot cross bun' sign: a multicentre MRI study of 97 patients with autopsy-confirmed multiple system atrophy.Journal of neurology, neurosurgery, and psychiatry · 2026Article
- Cerebellar and Brainstem White Matter Geometric Alterations in Multiple System Atrophy: A DFA-Based Biomarker for Disease Staging.CNS neuroscience & therapeutics · 2025Article
- Autonomic dysfunction in multiple system atrophy: from pathophysiology to clinical manifestations.Annals of medicine · 2025Review
- Contribution of α-synuclein cytopathologies to distinct seeding of misfolded α-synuclein.Brain pathology (Zurich, Switzerland) · 2025Article
- Review
- The Inferior Cerebellar Peduncle Sign: A Novel Imaging Marker for Differentiating Multiple System Atrophy Cerebellar Type from Spinocerebellar Ataxia.AJNR. American journal of neuroradiology · 2025Article
- The Irony of Iron: The Element with Diverse Influence on Neurodegenerative Diseases.International journal of molecular sciences · 2024Review
- Differentiation between Parkinson's Disease and the Parkinsonian Subtype of Multiple System Atrophy Using the Magnetic Resonance T1w/T2w Ratio in the Middle Cerebellar Peduncle.Diagnostics (Basel, Switzerland) · 2024Article
- Article
- Pharmacological inhibition of FABP7 by MF 6 counteracts cerebellum dysfunction in an experimental multiple system atrophy mouse model.Acta pharmacologica Sinica · 2024Article
- The α-synuclein PET tracer [18F] ACI-12589 distinguishes multiple system atrophy from other neurodegenerative diseases.Nature communications · 2023Article
- Functional brain networks in the evaluation of patients with neurodegenerative disorders.Nature reviews. Neurology · 2023Review
- Stereotyped Relationship Between Motor and Cognitive Metabolic Networks in Parkinson's Disease.Movement disorders : official journal of the Movement Disorder Society · 2022Article
- Quantitative Evaluation of Cerebellar Function in Multiple System Atrophy with Transcranial Magnetic Stimulation.Cerebellum (London, England) · 2022Article
- John Q. Trojanowski: neuropathology icon.Acta neuropathologica · 2022Article
- Passive Immunization in Alpha-Synuclein Preclinical Animal Models.Biomolecules · 2022Review
Corrections and comments
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Authors and funding
16 authors at 4 institutions in 2 countries.
Funding
Abstract
aimsThe aim of this study was to identify early foci of α-synuclein (α-syn pathology) accumulation, subsequent progression and neurodegeneration in multiple system atrophy of the cerebellar type (MSA-C).
methodsWe analysed 70-μm-thick sections of 10 cases with MSA-C and 24 normal controls.
resultsMSA-C cases with the lowest burden of pathology showed α-syn glial cytoplasmic inclusions (GCIs) in the cerebellum as well as in medullary and pontine cerebellar projections. Cerebellar pathology was highly selective and severely involved subcortical white matter, whereas deep white matter and granular layer were only mildly affected and the molecular layer was spared. Loss of Purkinje cells increased with disease duration and was associated with neuronal and axonal abnormalities. Neocortex, basal ganglia and spinal cord became consecutively involved with the increasing burden of α-syn pathology, followed by hippocampus, amygdala, and, finally, the visual cortex. GCIs were associated with myelinated axons, and the severity of GCIs correlated with demyelination.
conclusionsOur findings indicate that cerebellar subcortical white matter and cerebellar brainstem projections are likely the earliest foci of α-syn pathology in MSA-C, followed by involvement of more widespread regions of the central nervous system and neurodegeneration with disease progression.
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