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Lesional RBD

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Rapid-Eye-Movement Sleep Behavior Disorder

Abstract

REM sleep behavior disorder (RBD) is a potentially injurious parasomnia characterized by dream enactment behavior and polysomnographic REM sleep atonia loss (REM sleep without atonia, RSWA), most commonly seen as a manifestation of an early alpha-synuclein-mediated neurodegenerative disease in older adults. However, RBD has also been reported to evolve following lesions in the brainstem or limbic system. To date, 44 cases of “lesional RBD” have been reported in the literature. Age of lesional RBD onset and male predominance is similar to presumably synuclein-mediated RBD. Lesions to either the left or right dorsal pons, superior medulla, or limbic system appear to be most strongly associated with development of RBD, supporting the localization of REM sleep atonia control to the locus subcoeruleus/sublateral dorsal nucleus (SubC/SLD) of the pons. A variety of etiologies including infarction/encephalomalacia, inflammatory/demyelinating disorders, autoimmune disease, vascular malformation, and neoplasm have been associated with the development of lesional RBD, suggesting lesion location is more important than etiology in the development of RBD. It remains unknown whether brain lesions interact with preexisting covert pontine synuclein deposits that are already present (i.e., whether lesional RBD requires a “two-hit” pathophysiology) or perhaps with other vulnerability factors that could predispose toward RBD development, since lesional RBD is much rarer than pontine lesions in the general population. The development of RBD following either a discrete lesion to the dorsal pontine location of the SubC/SLD or extraaxial lesions exerting mass effect on the brainstem suggests that disruption or disturbance of the SubC/SLD or its afferent/efferent projections to other structures in the REM atonia control network can cause RBD. Lesional RBD may be refractory to pharmacologic treatment with or without continued presence of the lesion, and resolution of RBD following effective treatment of the underlying etiology is variable, likely depending upon the degree of reversibility of damage or possibly due to permanent impact and alteration of REM atonia control networks following disturbance by a lesion even after its resolution. Patients who develop RBD along with focal neurologic deficits or in the context of subacutely evolving syndromic neurologic manifestations of encephalopathy, cognitive, autonomic, or movement disorder impairments should undergo brain magnetic resonance imaging to rule out a structural lesion as the cause of their RBD.

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Acknowledgments

The project described was supported by the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health, through Grant Number 1 UL1 RR024150-01. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.

Disclosures

No off-label medication use.

The project described was supported by the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health, through Grant Number 1 UL1 RR024150-01. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.

SJ McCarter reports no disclosures.

EK St. Louis reports that he receives research support from the Mayo Clinic Center for Translational Science Activities (CTSA), supported by the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health, through Grant Number 1 UL1 RR024150-01.

Author information

Authors and Affiliations

  1. Mayo Center for Sleep Medicine, Rochester, MN, USA

    Stuart J. McCarter M.D. & Erik K. St. Louis M.D., M.S.

  2. Department of Neurology, Mayo Clinic and Foundation, Rochester, MN, USA

    Stuart J. McCarter M.D. & Erik K. St. Louis M.D., M.S.

  3. Department of Medicine, Mayo Clinic and Foundation, Rochester, MN, USA

    Stuart J. McCarter M.D. & Erik K. St. Louis M.D., M.S.

  4. Mayo Clinic College of Medicine, Rochester, MN, USA

    Erik K. St. Louis M.D., M.S.

Authors
  1. Stuart J. McCarter M.D.
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Correspondence to Erik K. St. Louis M.D., M.S. .

Editor information

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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McCarter, S.J., St. Louis, E.K. (2019). Lesional RBD. 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_9

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  • DOI: https://doi.org/10.1007/978-3-319-90152-7_9

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