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Multiple System Atrophy

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Hybrid PET/MR Neuroimaging

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Abstract

Multiple system atrophy (MSA) is a rapidly progressive sporadic neurodegenerative disease that may share clinical and imaging features with Parkinson’s disease and other atypical parkinsonian disorders. Patients present with autonomic failure usually in the form of genitourinary syndromes, severe orthostatic hypotension, and sleep disorders, among others. The disease is classified based on the predominant motor manifestations into MSA-P (parkinsonian) or MSA-C (cerebellar), although patients may have overlapping clinical and imaging findings. Poor sustained response to levodopa is a hallmark of MSA. Certain imaging features support a diagnosis of MSA, including atrophy of the putamen (posterolateral predominance), pons, brainstem, or cerebellum on MRI and hypometabolism of the putamen, brainstem, or cerebellum on FDG-PET imaging. There are other characteristic features of MSA-P on MRI such as putaminal hypointensity on T2 or T2*/SWI sequences due to iron deposition and the presence of a T2-FLAIR hyperintense putaminal rim. In MSA-C, there is profound atrophy of the pons, medulla, cerebellum, and middle cerebellar peduncles with a characteristic cruciform T2-FLAIR hyperintensity in the pons. In addition to FDG, several other PET tracers have been studied in MSA which allow evaluation of the dopaminergic and cholinergic systems as well as changes in microglial activation secondary to neuroinflammation, among others.

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Authors and Affiliations

  1. Division of Neuroradiology, Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, NC, USA

    Carlos Zamora & Mauricio Castillo

  2. Division of Molecular Imaging and Therapeutics, Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, NC, USA

    Mitchel A. Muhleman

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  1. Carlos Zamora
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  2. Mitchel A. Muhleman
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Correspondence to Carlos Zamora .

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Editors and Affiliations

  1. Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, The Feinstein Institutes for Medical Research, Manhasset, NY, USA

    Ana M. Franceschi

  2. Stony Brook University Hospital, Stony Brook, NY, USA

    Dinko Franceschi

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Zamora, C., Muhleman, M.A., Castillo, M. (2022). Multiple System Atrophy. In: Franceschi, A.M., Franceschi, D. (eds) Hybrid PET/MR Neuroimaging. Springer, Cham. https://doi.org/10.1007/978-3-030-82367-2_31

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