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REM Sleep Behavior Disorder Associated with Parkinson’s Disease and Multiple System Atrophy

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Rapid-Eye-Movement Sleep Behavior Disorder

Abstract

Violent and other clinical manifestations of REM sleep behaviour disorder (RBD), presenting in sleep laboratories for diagnostic work-up, have been shown to precede the clinical syndrome of neurodegenerative diseases associated with the misprocessing of α-synuclein. At the time of RBD diagnosis, it is still impossible to foresee whether idiopathic RBD will eventually evolve to Parkinson’s disease (PD), multiple system atrophy (MSA) or dementia with Lewy bodies (DLB). Conversely, in newly diagnosed PD, a meta-analysis of published studies has shown that RBD has been identified in a mean 23.6% (range, 4.3–69.4%) of patients and appears to indicate a specific phenotypically defined subtype of the disease. Although not all PD patients with RBD show violent dream-enacting behaviours and RBD severity may vary from night to night, RBD is an important differential diagnosis for abnormal nocturnal behaviours in PD patients as the disease progresses, encompassing also night-time confusional states, hallucinations and periodic limb movements (PLM). Moreover, the appearance of RBD during the course of PD has been shown to indicate the entrance to a more advanced stage of the disease. In MSA the prevalence of RBD has been determined at 88%. While RBD manifestations prodromal to MSA onset are described by many patients, dream-enacting behaviours seem to disappear over time, and physiologic sleep structure becomes severely disturbed. So far, attempts to differentiate the different types of MSA by sleep analysis have failed, and longitudinal sleep laboratory data are insufficient. Recently a small series of patients with pure autonomic failure (PAF) was published, and these patients had developed RBD during the course of the disease, indicating that PAF may be a mild form of CNS α-synucleinopathy.

“... In this stage, the sleep becomes much disturbed. The tremulous motion of the limbs occur during sleep, and augment until they awaken the patient, and frequently with much agitation and alarm. ... but even when exhausted nature seizes a small portion of sleep, the motion becomes so violent as not only to shake the bed-hanging, but even the floor and sashes of the room. …”.

(James Parkinson, 1817) [1]

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Note Added in Proof:

A recently published study merits inclusion, along with its accompanying Editorial: (1) Pagano G, De Micco R, Yousaf T, Wilson H, Chandra A, Politis M. REM behavior disorder predicts motor progression and cognitive decline in Parkinson disease. Neurology 2018 Aug 8; doi: 10.1212/WNL.0000000000006134. (2) Mahowald MW, Schenck CH. The “when” and “where” of α-synucleinopathies: Insights from REM sleep behavior disorder. Neurology. 2018 Aug 8; doi: 10.1212/WNL.0000000000006129.

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Authors and Affiliations

  1. Paracelsus-Elena-Klinik, Kassel, Germany

    Friederike Sixel-Döring & Claudia Trenkwalder

  2. Department of Neurology, Philipps-University, Marburg, Germany

    Friederike Sixel-Döring

  3. Department of Neurosurgery, University Medicine, Göttingen, Germany

    Claudia Trenkwalder

Authors
  1. Friederike Sixel-Döring
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  2. Claudia Trenkwalder
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Correspondence to Friederike Sixel-Döring .

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Editors and Affiliations

  1. Minnesota Regional Sleep Disorders Center, Departments of Psychiatry, University of Minnesota Medical School and Hennepin County Medical Center, Minneapolis, MN, USA

    Carlos H. Schenck

  2. Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria

    Birgit Högl

  3. Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Aleksandar Videnovic

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Sixel-Döring, F., Trenkwalder, C. (2019). REM Sleep Behavior Disorder Associated with Parkinson’s Disease and Multiple System Atrophy. In: Schenck, C., Högl, B., Videnovic, A. (eds) Rapid-Eye-Movement Sleep Behavior Disorder. Springer, Cham. https://doi.org/10.1007/978-3-319-90152-7_5

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  • DOI: https://doi.org/10.1007/978-3-319-90152-7_5

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