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Isolated rapid eye movement sleep behaviour disorder: clinical and research implications
  1. Ambra Stefani1,2,
  2. Claudia Trenkwalder3,4,
  3. Isabelle Arnulf5,6,
  4. Donald L Bliwise7,
  5. Bradley F Boeve8,
  6. Yuichi Inoue9,
  7. Alejandro Iranzo10,
  8. Simon JG Lewis11,
  9. Federica Provini12,
  10. Carlos Schenck13,
  11. Gregor K Wenning14,
  12. Yun kwok Wing15,
  13. Birgit Hogl16,
  14. Aleksandar Videnovic17
  1. 1 Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria
  2. 2 Department of Neurology, Harvard Medical School, Boston, Massachusetts, USA
  3. 3 Paracelsus Elena Klinik, Kassel, Germany
  4. 4 Neurologische Klinik, Georg August Universität, Göttingen, Germany
  5. 5 Assistance Publique Hôpitaux de Paris, Service des pathologies du Sommeil, Hôpital Pitié-Salpêtrière, Paris, France
  6. 6 UMR S 1127, CNRS UMR 7225, ICM, Sorbonne Universités, UPMC Univ Paris 06, Paris, France
  7. 7 Neurology, Emory University School of Medicine, Atlanta, Georgia, USA
  8. 8 Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  9. 9 Tokyo Medical University, Shinjuku-ku, Japan
  10. 10 Universitat de Barcelona, Barcelona, Spain
  11. 11 Healthy Brain Ageing Clinic, University of Sydney, Sydney, New South Wales, Australia
  12. 12 Department of Biomedical and Neuromotor Sciences and IRCCS Institute of Neurosciences, Bologna, University of Bologna, Bologna, Italy
  13. 13 Dept. of Psychiatry and Sleep Disorders Center, Minnesota Regional Sleep Disorders Center and University of Minnesota, Minneapolis, Minnesota, USA
  14. 14 Division of Neurobiology, Innsbruck, Austria
  15. 15 Psychiatry, The Chinese University of Hong Kong Faculty of Medicine, Hong Kong, Hong Kong
  16. 16 Department of Neurology, Innsbruck Medical University, Innsbruck, Austria
  17. 17 Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr Aleksandar Videnovic, Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA; AVIDENOVIC{at}mgh.harvard.edu

https://doi.org/10.1136/jnnp-2022-330913

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    Rapid eye movement (REM) sleep behaviour disorder (RBD), initially described in 1986 by Schenck et al as an REM sleep parasomnia,1 is characterised by dream enactment behaviours and increased muscle activity during REM sleep.2 Isolated RBD (iRBD, ie, in the absence of associated neurological disorders like narcolepsy, overt synucleinopathy, anti-IgLON5 disease and other less frequent conditions) has gained increasing relevance in the last decade as numerous studies demonstrated that (1) most iRBD patients develop an overt synucleinopathy over time3 and (2) in patients with iRBD (even those with long-standing iRBD, ie, those not phenoconverting ten years or more after iRBD diagnosis) biomarkers of synuclein-related neurodegeneration are common, including pathological synuclein aggregates in several tissues,4 possibly indicating an underlying neurodegenerative process.5 Based on this evidence, iRBD is now recognised as an early-phase synucleinopathy in most cases, with phenoconversion over time into dementia with Lewy bodies (DLB), Parkinson’s disease (PD) or, less commonly, multiple system atrophy (MSA). A meta-analysis showed that the risk for developing neurodegenerative diseases was 33.5% at 5 years follow-up, 82.4% at 10.5 years and 96.6% at 14 years.3

    In light of such observations, the diagnosis of iRBD has significant implications for individuals with RBD-like symptoms, and it is, therefore, essential that an accurate and timely diagnosis is established. Although it is still debated how and when to disclose the potential implications of iRBD diagnosis on brain health to patients,6 7 this information is …

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    Footnotes

    • Contributors AS and AV: drafting of the manuscript. All other authors: critical review of the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.