Insights into REM sleep behavior disorder pathophysiology in brainstem-predominant Lewy body disease

B. F. Boeve, D. W. Dickson, E. J. Olson, J. W. Shepard, M. H. Silber, T. J. Ferman, J. E. Ahlskog, E. E. Benarroch

Research output: Contribution to journalArticle

131 Scopus citations

Abstract

Background and purpose: Rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia reflecting changes in the brain, but which specific neuronal networks are involved in human RBD pathogenesis has not yet been determined. To date, only one case of idiopathic RBD has undergone autopsy, in which "incidental Lewy body disease" was found. Due to the severe neuronal loss and gliosis in the substantia nigra (SN) and locus ceruleus (LC) in this case, degeneration of brainstem monoaminergic neurons was postulated as the underlying substrate for RBD. Additional cases of idiopathic RBD with neuropathologic examination may help clarify which key brainstem structures are involved. Patient and methods: Case report with neuropathologic analysis. Results: A man with polysomnographically proven RBD (onset age 57 years), but no other neurologic signs or symptoms, underwent neuropathologic examination upon his death at age 72. Histopathologic analysis showed Lewy body disease, but no significant neuronal loss or gliosis was present in the SN or LC. Conclusions: This case represents another example of Lewy body disease associated with RBD. The minimal degenerative changes in the SN and LC call into question the role of these nuclei in RBD, at least in our case. We suggest additional cases of idiopathic RBD undergo neuropathologic analyses to better delineate the neurologic substrate of this intriguing parasomnia.

Original languageEnglish (US)
Pages (from-to)60-64
Number of pages5
JournalSleep Medicine
Volume8
Issue number1
DOIs
StatePublished - Jan 2007

Keywords

  • Lewy bodies
  • Lewy body disease
  • Parasomnia
  • REM sleep behavior disorder
  • Synuclein

ASJC Scopus subject areas

  • Medicine(all)

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