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Modelling progressive autonomic failure in MSA: where are we now?

  • Movement Disorders - Review Article
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Abstract

Multiple system atrophy (MSA) is a fatal late-onset α-synucleinopathy that presents with features of ataxia, Parkinsonism, and pyramidal dysfunction in any combination. Over the last decade, efforts have been made to develop preclinical MSA testbeds for novel interventional strategies. The main focus has been on murine analogues of MSA-linked motor features and their underlying brainstem, cerebellar and basal ganglia pathology. Although progressive autonomic failure (AF) is a prominent clinical feature of patients with MSA, reflecting a disruption of both central and peripheral autonomic networks controlling cardiovascular, respiratory, urogenital, gastrointestinal and sudomotor functions, attempts of modelling this aspect of the human disease have been limited. However, emerging evidence suggests that AF-like features may occur in transgenic MSA models reflecting α-synucleinopathy lesions in distributed autonomic networks. Further research is needed to fully characterize both autonomic and motor features in optimized preclinical MSA models.

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Acknowledgments

Supported by grants of the Austrian Research Foundation graduate program SPIN (FWF W1206).

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  1. Division of Clinical Neurobiology, Department of Neurology, Innsbruck Medical University, Anichstr. 35, 6020, Innsbruck, Austria

    Sylvia Stemberger & Gregor K. Wenning

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  1. Sylvia Stemberger
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  2. Gregor K. Wenning
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Correspondence to Gregor K. Wenning.

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Stemberger, S., Wenning, G.K. Modelling progressive autonomic failure in MSA: where are we now?. J Neural Transm 118, 841–847 (2011). https://doi.org/10.1007/s00702-010-0576-3

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