Skip to main content

Neuropathology of REM Sleep Behavior Disorder

  • Chapter
  • First Online:
Rapid-Eye-Movement Sleep Behavior Disorder

Abstract

The neuropathology of RBD demonstrates the very strong association of alpha-synucleinopathy neurodegeneration with RBD, with complexities involving clinical synucleinopathy subtypes; prodromal stages; heterogeneous phenoconversion timelines from idiopathic RBD (iRBD) to overt neurodegeneration; distribution of synuclein pathology in the central, peripheral, and autonomic nervous systems; clinical comorbidities; antidepressant medication use; and other clinical factors. This chapter covers (1) a clinical-neuropathological study of 172 RBD cases, (2) neuropathology of iRBD cases, (3) a clinical-neuropathological study of PD and probable RBD, (4) a case report of RBD and the neuropathology of prodromal Lewy body disease, (5) two studies on how RBD is closely related to enteric neuropathology in iRBD and RBD-PD, (6) two studies on skin nerve phosphorylated α-synuclein deposits in iRBD, (7) a study on reduced intraepidermal nerve fiber density in patients with iRBD, (8) a study on α-synuclein deposition in autonomic nerve fibers in the submandibular glands in patients with iRBD, (9) a study on α-synuclein aggregates in labial glands of patients with iRBD, and (10) a study comparing the predictive value of probable RBD vs. absence of probable RBD in post-mortem histopathology-confirmed α-synucleinopathy in patients participating in a longitudinal aging and neurodegenerative disorders study.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Boeve BF, Silber MH, Ferman TJ, et al. Clinicopathologic correlations in 172 cases of rapid eye movement sleep behavior disorder with or without a coexisting neurologic disorder. Sleep Med. 2013;14(8):754–62.

    Article  CAS  Google Scholar 

  2. McKeith IG, Galasko D, Kosaka K, et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology. 1996;47(5):1113–24.

    Article  CAS  Google Scholar 

  3. Fujishiro H, Ferman T, Boeve B, et al. Validation of the neuropathologic criteria of the third consortium for dementia with Lewy bodies for prospectively diagnosed cases. J Neuropathol Exp Neurol. 2008;67(7):649–56.

    Article  Google Scholar 

  4. Boeve BF, Dickson DW, Olson EJ, et al. Insights into REM sleep behavior disorder pathophysiology in brainstem-predominant Lewy body disease. Sleep Med. 2007;8(1):60–4.

    Article  CAS  Google Scholar 

  5. Schenck CH, Mahowald MW. REM sleep behavior disorder: clinical, developmental, and neuroscience perspectives 16 years after its formal identification in SLEEP. Sleep. 2002;25(2):120–38.

    Article  Google Scholar 

  6. Schenck CH, Mahowald MW, Anderson ML, Silber MH, Boeve BF, Parisi JE. Lewy body variant of Alzheimer’s Disease (AD) identified by postmortem ubiquitin staining in a previously reported case of AD associated with REM sleep behavior disorder. Biol Psychiatry. 1997;42(6):527–8.

    Article  CAS  Google Scholar 

  7. Iranzo A, Tolosa E, Gelpi E. Neurodegenerative disease status and post-mortem pathology in idiopathic rapid-eye-movement sleep behaviour disorder: an observational cohort study. Lancet Neurol. 2013;12(5):443–53.

    Article  Google Scholar 

  8. Uchiyama M, Isse K, Tanaka K, et al. Incidental Lewy body disease in a patient with REM sleep behavior disorder. Neurology. 1995;45(4):709–12.

    Article  CAS  Google Scholar 

  9. Postuma RB, Adler CH, Dugger BN, et al. REM sleep behavior disorder and neuropathology in Parkinson’s disease. Mov Disord. 2015;30(10):1413–7.

    Article  CAS  Google Scholar 

  10. Iranzo A, Gelpi E, Tolosa E, Molinuevo JL, Serradell M, Gaig C, Santamaria J. Neuropathology of prodromal Lewy body disease. Mov Disord. 2014;29(3):410–5.

    Article  Google Scholar 

  11. Sprenger FS, Stefanova N, Gelpi E, et al. Enteric nervous system α-synuclein immunoreactivity in idiopathic REM sleep behavior disorder. Neurology. 2015;85(20):1761–8.

    Article  CAS  Google Scholar 

  12. Leclair-Visonneau L, Clairembault T, Coron E, et al. RBD is related to enteric neuropathology in PD. Neurology. 2017;89(15):1612–8.

    Article  Google Scholar 

  13. Antelmi E, Donadio V, Incensi A, Plazzi G, Liguori R. Skin nerve phosphorylated α-synuclein deposits in idiopathic REM sleep behavior disorder. Neurology. 2017;88(22):2128–31.

    Article  CAS  Google Scholar 

  14. Doppler K, Jentschke HM, Schulmeyer L, et al. Dermal phospho-alpha-synuclein deposits confirm REM sleep behaviour disorder as prodromal Parkinson’s disease. Acta Neuropathol. 2017;133(4):535–45.

    Article  CAS  Google Scholar 

  15. Schrempf W, Katona I, Dogan I, et al. Reduced intraepidermal nerve fiber density in patients with REM sleep behavior disorder. Parkinsonism Relat Disord. 2016;29:10–6.

    Article  Google Scholar 

  16. Vilas D, Iranzo A, Tolosa E, et al. Assessment of α-synuclein in submandibular glands of patients with idiopathic rapid-eye-movement sleep behaviour disorder: a case-control study. Lancet Neurol. 2016;15(7):708–18.

    Article  CAS  Google Scholar 

  17. Iranzo A, Borrego S, Vilaseca I, et al. Alpha-synuclein aggregates in labial salivary glands of idiopathic rapid eye movement sleep behavior disorder. Sleep. 2018. https://doi.org/10.1093/sleep/zsy101. [Epub ahead of print].

  18. Shprecher DR, Adler CH, Zhang N, et al. Predicting alpha-synuclein pathology by REM sleep behavior disorder diagnosis. Parkinsonism Relat Disord. 2018. https://doi.org/10.1016/j.parkreldis. [Epub ahead of print].

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Carlos H. Schenck .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Schenck, C.H. (2019). Neuropathology of REM Sleep Behavior Disorder. 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_40

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-90152-7_40

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-90151-0

  • Online ISBN: 978-3-319-90152-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics