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Review
. 2025 Apr 11.
doi: 10.1007/s00702-025-02921-3. Online ahead of print.

Neuroimaging in multiple system atrophy: clinical implications and novel developments

Wolfgang H Jost  1   2 , Alexander Rau  3 , Joachim Brumberg  4 , Horst Urbach  3 , Philipp T Meyer #  4 , Nils Schröter #  5   6
Affiliations
Review

Neuroimaging in multiple system atrophy: clinical implications and novel developments

Wolfgang H Jost et al. J Neural Transm (Vienna). .

Abstract

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by cerebellar dysfunction, a Parkinsonian syndrome with poor response to levodopa and autonomic failure. The diagnosis of MSA is particularly challenging in its early stages due to symptom overlap with other neurodegenerative Parkinson syndromes. Recent advances in neuroimaging have greatly improved the accuracy of the diagnosis in clinical routine and provided valuable insights into the pathophysiology and progression of MSA. Macrostructural MRI shows atrophy in regions such as the putamen and pontocerebellar regions, distinguishing MSA from other Parkinson syndromes. Advanced imaging techniques, including diffusion tensor imaging (DTI), free water imaging and quantitative susceptibility mapping, add further value in assessing disease progression. While dopamine transporter (DAT) imaging is the mainstay for confirmation of nigrostriatal degeneration in suspected neurodegenerative Parkinson syndromes and may enable to identify prodromal cases, cardiac sympathetic imaging with [123I]MIBG scintigraphy may be used for delineation of MSA from Parkinson's disease (PD). Positron emission tomography (PET) with the glucose analogue [18F]FDG depicts disease-specific metabolic patterns in MSA and various neurodegenerative diseases, which do not only enable a highly accurate differential diagnosis of MSA (e.g., from PD and other atypical Parkinson syndromes) but also carry important prognostic and pathophysiological information. Various other PET radiopharmaceuticals currently under investigation in MSA provide novel insights into neurotransmitter system changes, glial pathology and, most recently, α-synuclein pathology. These imaging modalities considerably expand the diagnostic and prognostic capabilities in MSA and may provide important biomarkers for tracking disease development, progression and treatment.

Keywords: DAT SPECT; Imaging; MRI; MSA; PET.

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Conflict of interest statement

Declarations. Relevant conflicts of interest/financial disclosures: The authors have nothing to disclose. The authors declare no competing nonfinancial or financial interests. Dedication: We wrote this work in memory of Gregor Wenning. He has achieved extraordinary success in his scientific work with multiple system atrophy. We will all miss his ideas, his commitment and his enthusiasm. But we want to continue this work together so that the disease can be diagnosed earlier and safely and so that we can treat the disease better and perhaps even cure it. This would have been in the spirit of Gregor Wenning. Funding agency: AR and JB were funded by Berta-Ottenstein-Programme for Advanced Clinician Scientists, Faculty of Medicine, University of Freiburg. Ethics declarations: All authors declare that they have no conflicts of interest in regard to this work. Conflict of interest: All authors contributed to the study conception and design. The first draft of the manuscript was written by NSc and AR and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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References

    1. Antonini A, Benti R, De Notaris R et al (2003) 123I-Ioflupane/SPECT binding to striatal dopamine transporter (DAT) uptake in patients with Parkinson’s disease, multiple system atrophy, and progressive supranuclear palsy. Neurol Sci 24:149–150. https://doi.org/10.1007/s10072-003-0103-5 - DOI - PubMed
    1. Arnaldi D, Chincarini A, Hu MT et al (2021) Dopaminergic imaging and clinical predictors for phenoconversion of REM sleep behaviour disorder. Brain 144:278–287. https://doi.org/10.1093/brain/awaa365 - DOI - PubMed
    1. Ballweg A, Klaus C, Vogler L et al (2023) [18F]F-DED PET imaging of reactive astrogliosis in neurodegenerative diseases: preclinical proof of concept and first-in-human data. J Neuroinflammation 20:68. https://doi.org/10.1186/s12974-023-02749-2 - DOI - PubMed - PMC
    1. Braune S (2001) The role of cardiac Metaiodobenzylguanidine uptake in the differential diagnosis of parkinsonian syndromes. Clin Auton Res 11:351–355. https://doi.org/10.1007/BF02292766 - DOI - PubMed
    1. Bürk K, Skalej M, Dichgans J (2001) Pontine MRI hyperintensities (the cross sign) are not pathognomonic for multiple system atrophy (MSA). Mov Disord 16:535. https://doi.org/10.1002/mds.1107 - DOI - PubMed

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