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Autonomic dysfunction and phenoconversion in idiopathic REM sleep behavior disorder

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Abstract

Background

REM sleep behavior disorder (RBD) is a common finding among patients with synucleinopathies. We aimed to determine the degree of autonomic dysfunction in patients presenting with idiopathic RBD (iRBD), and the predictive value of autonomic dysfunction for phenoconversion to a defined neurodegenerative disease.

Methods

We searched our electronic medical record for patients diagnosed with iRBD who also underwent standardized autonomic function testing within 6 months of iRBD diagnosis, and who had clinical follow-up of at least 3 years following iRBD diagnosis. The composite autonomic severity score (CASS) was derived and compared between phenoconverters and non-converters using chi-square and Wilcoxon rank-sum tests.

Results

We identified 18 patients who fulfilled inclusion and exclusion criteria. Average age at autonomic testing was 67 ± 6.6 years. Twelve (67%) patients phenoconverted during the follow-up period; six developed Parkinson’s disease (PD), and the other six, dementia with Lewy bodies (DLB). Fifteen (83%) patients had at least mild autonomic dysfunction. There were no significant differences between overall converters and non-converters in total CASS or CASS subscores. However, iRBD patients who developed DLB had significantly higher total and cardiovagal CASS scores compared with those who developed PD (p < 0.05), and a trend for higher adrenergic CASS scores compared to those who developed PD and those who did not phenoconvert.

Discussion

Autonomic dysfunction was seen in 83% of iRBD patients, and more severe baseline cardiovagal autonomic dysfunction in iRBD was associated with phenoconversion to DLB but not PD. Prospective studies are needed to confirm the value of autonomic testing for predicting phenoconversion and disease phenotype in iRBD.

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Funding

This work was supported in part by the National Institutes of Health (R01 NS092625).

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Authors and Affiliations

  1. Department of Neurology, Mayo Clinic, 200 First Street Southwest, Rochester, MN, 55905, USA

    Stuart J. McCarter, Tonette L. Gehrking, Erik K. St. Louis, Mariana D. Suarez, Bradley F. Boeve, Michael H. Silber, Phillip A. Low & Wolfgang Singer

  2. Department of Medicine, Mayo Clinic, Rochester, USA

    Erik K. St. Louis

  3. Department of Sleep Medicine, Mayo Clinic, Rochester, USA

    Erik K. St. Louis, Bradley F. Boeve & Michael H. Silber

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  1. Stuart J. McCarter
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  2. Tonette L. Gehrking
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  3. Erik K. St. Louis
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  4. Mariana D. Suarez
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  5. Bradley F. Boeve
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  6. Michael H. Silber
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  7. Phillip A. Low
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  8. Wolfgang Singer
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Correspondence to Wolfgang Singer.

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McCarter, S.J., Gehrking, T.L., St. Louis, E.K. et al. Autonomic dysfunction and phenoconversion in idiopathic REM sleep behavior disorder. Clin Auton Res 30, 207–213 (2020). https://doi.org/10.1007/s10286-020-00674-5

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  • DOI: https://doi.org/10.1007/s10286-020-00674-5

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