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]
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Parkinson J. An essay on the shaking palsy. London: Whittingham and Rowland; 1817.
Schenck CH, Bundlie SR, Mahowald MW. Delayed emergence of a parkinsonian disorder in 38% of 29 older men initially diagnosed with idiopathic rapid eye movement sleep behaviour disorder. Neurology. 1996;46:388–93.
Trenkwalder C. Sleep dysfunction in Parkinson’s disease. Clin Neurosci. 1998;5:107–14.
Getting a good night sleep? The importance of recognizing and treating nocturnal hypokinesia in Parkinson’s disease.
Comella CL, Nardine TM, Diederich NJ, Stebbins GT. Sleep-related violence, injury, and REM sleep behavior disorder in Parkinson’s disease. Neurology. 1998;51:526–9.
Schenck CH, Lee SA, Bornemann MA, Mahowald MW. Potentially lethal behaviors associated with rapid eye movement sleep behavior disorder: review of the literature and forensic implications. J Forensic Sci. 2009;54:1475–84.
Chaudhuri KR, Yates L, Martinez-Martin P. The non-motor symptom complex of Parkinson's disease: a comprehensive assessment is essential. Curr Neurol Neurosci Rep. 2005;5:275–83.
Barone P, Antonini A, Colosimo C, et al. The PRIAMO study: a multicenter assessment of nonmotor symptoms and their impact on quality of life in Parkinson's disease. Mov Disord. 2009;24:1641–9.
Martinez-Martin P, Rodriguez-Blazquez C, Kurtis MM, Chaudhuri KR. The impact of non-motor symptoms on health-related quality of life of patients with Parkinson’s disease. Mov Disord. 2011;26:399–406.
Schenck CH, Boeve BF, Mahowald MW. Delayed emergence of a parkinsonian disorder or dementia in 81% of older men initially diagnosed with idiopathic rapid eye movement sleep behavior disorder: a 16-year update on a previously reported series. Sleep Med. 2013;14(8):744.
Iranzo A, Molinuevo JL, Santamaria J, et al. Rapid-eye-movement sleep behaviour disorder as an early marker for a neurodegenerative disorder: a descriptive study. Lancet Neurol. 2006;5(7):572.
Postuma RB, Gagnon JF, Vendette M, Fantini ML, Massicotte-Marquez J, Montplaisir J. Quantifying the risk of neurodegenerative disease in idiopathic REM sleep behavior disorder. Neurology. 2009;72:1296–300.
Iranzo A, Tolosa E, Gelpi E, et al. Neurodegenerative disease status and post-mortem pathology in idiopathic rapid-eye-movement sleep behaviour disorder: an observational cohort study. Lancet Neurol. 2013;12:443–53.
Iranzo A, Valldeoriola F, Lomena F, et al. Serial dopamine transporter imaging of nigrostriatal function in patients with idiopathic rapid-eye-movement sleep behaviour disorder: a prospective study. Lancet Neurol. 2011;10:797–805.
Wu P, Yu H, Peng S, et al. Consistent abnormalities in metabolic network activity in idiopathic rapid eye movement sleep behaviour disorder. Brain. 2014;137:3122–8.
Berg D, Marek K, Ross GW, Poewe W. Defining at-risk populations for Parkinson's disease: lessons from ongoing studies. Mov Disord. 2012;27:656–65.
Iranzo A, Santamaria J, Rye DB, et al. Characteristics of idiopathic REM sleep behavior disorder and that associated with MSA and PD. Neurology. 2005;65:247–52.
Boeve BF, Silber MH, Saper CB, et al. Pathophysiology of REM sleep behaviour disorder and relevance to neurodegenerative disease. Brain. 2007;130:2770–88.
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;14:754–62.
Hughes AJ, Daniel SE, Blankson S, Lees AJ. A clinicopathologic study of 100 cases of Parkinson’s disease. Arch Neurol. 1993;50:140–8.
Braak H, Del Tredici K, Rub U, de Vos RA, Jansen Steur EN, Braak E. Staging of brain pathology related to sporadic Parkinson's disease. Neurobiol Aging. 2003;24:197–211.
Lai YY, Hsieh KC, Nguyen D, Peever J, Siegel JM. Neurotoxic lesions at the ventral mesopontine junction change sleep time and muscle activity during sleep: an animal model of motor disorders in sleep. Neuroscience. 2008;154:431–43.
Zhang J, Xu C-Y, Liu J. Meta-analysis on the prevalence of REM sleep behavior disorder symptoms in Parkinson’s disease. BMC Neurol. 2017;17:23.
Sixel-Doring F, Trautmann E, Mollenhauer B, Trenkwalder C. Rapid eye movement sleep behavioral events: a new marker for neurodegeneration in early Parkinson disease? Sleep. 2014;37:431–8.
Frauscher B, Iranzo A, Gaig C, et al. Normative EMG values during REM sleep for the diagnosis of REM sleep behavior disorder. Sleep. 2012;35:835–47.
Muntean ML, Trenkwalder C, Walters AS, Mollenhauer B, Sixel-Doring F. REM sleep behavioral events and dreaming. J Clin Sleep Med. 2015;11:537–41.
Sixel-Doring F, Zimmermann J, Wegener A, Mollenhauer B, Trenkwalder C. The evolution of REM sleep behavior disorder in early Parkinson disease. Sleep. 2016;39:1737–42.
Stefani A, Gabelia D, Hogl B, et al. Long-term follow-up investigation of isolated rapid eye movement sleep without atonia without rapid eye movement sleep behavior disorder: a pilot study. J Clin Sleep Med. 2015;11:1273–9.
Chahine LM, Xie SX, Simuni T, et al. Longitudinal changes in cognition in early Parkinson's disease patients with REM sleep behavior disorder. Parkinsonism Relat Disord. 2016;27:102–6.
Sforza E, Zucconi M, Petronelli R, Lugaresi E, Cirignotta F. REM sleep behavioral disorders. Eur Neurol. 1988;28:295–300.
Fantini ML, Gagnon JF, Filipini D, Montplaisir J. The effects of pramipexole in REM sleep behavior disorder. Neurology. 2003;61:1418–20.
Gagnon JF, Bedard MA, Fantini ML, et al. REM sleep behavior disorder and REM sleep without atonia in Parkinson’s disease. Neurology. 2002;59:585–9.
Consens FB, Chervin RD, Koeppe RA, et al. Validation of a polysomnographic score for REM sleep behavior disorder. Sleep. 2005;28:993–7.
Frauscher B, Gschliesser V, Brandauer E, et al. Video analysis of motor events in REM sleep behavior disorder. Mov Disord. 2007;22:1464–70.
Sixel-Doring F, Schweitzer M, Mollenhauer B, Trenkwalder C. Intraindividual variability of REM sleep behavior disorder in Parkinson’s disease: a comparative assessment using a new REM sleep behavior disorder severity scale (RBDSS) for clinical routine. J Clin Sleep Med. 2011;7:75–80.
Sixel-Döring F, Trautmann E, Mollenhauer B, Trenkwalder C. Phenomenology of rapid eye movement (REM) sleep behavior disorder in Parkinson’s disease: a descriptive study using the REM sleep behavior disorder severity scale (RBDSS). Sleep Biol Rhythms. 2013;11:35–9.
Zhang J, Lam SP, Ho CK, et al. Diagnosis of REM sleep behavior disorder by video-polysomnographic study: is one night enough? Sleep. 2008;31:1179–85.
Bolitho SJ, Naismith SL, Terpening Z, et al. Investigating rapid eye movement sleep without atonia in Parkinson’s disease using the rapid eye movement sleep behavior disorder screening questionnaire. Mov Disord. 2014;29:736–42.
De Cock VC, Vidailhet M, Leu S, et al. Restoration of normal motor control in Parkinson’s disease during REM sleep. Brain. 2007;130:450–6.
Stiasny-Kolster K, Sixel-Doring F, Trenkwalder C, et al. Diagnostic value of the REM sleep behavior disorder screening questionnaire in Parkinson’s disease. Sleep Med. 2015;16:186–9.
Marelli S, Rancoita PM, Giarrusso F, et al. National validation and proposed revision of REM sleep behavior disorder screening questionnaire (RBDSQ). J Neurol. 2016;263:2470–5.
Iranzo A, Santamaria J, Tolosa E. The clinical and pathophysiological relevance of REM sleep behavior disorder in neurodegenerative diseases. Sleep Med Rev. 2009;13:385–401.
Hogl B, Stefani A. REM sleep behavior disorder (RBD): update on diagnosis and treatment. Somnologie (Berl). 2017;21:1–8.
Sixel-Doring F, Trautmann E, Mollenhauer B, Trenkwalder C. Associated factors for REM sleep behavior disorder in Parkinson disease. Neurology. 2011;77:1048–54.
Kumru H, Santamaria J, Tolosa E, Iranzo A. Relation between subtype of Parkinson's disease and REM sleep behavior disorder. Sleep Med. 2007;8:779–83.
Postuma RB, Gagnon JF, Vendette M, Charland K, Montplaisir J. REM sleep behaviour disorder in Parkinson’s disease is associated with specific motor features. J Neurol Neurosurg Psychiatry. 2008;79:1117–21.
Ozekmekci S, Apaydin H, Kilic E. Clinical features of 35 patients with Parkinson’s disease displaying REM behavior disorder. Clin Neurol Neurosurg. 2005;107:306–9.
Sinforiani E, Pacchetti C, Zangaglia R, Pasotti C, Manni R, Nappi G. REM behavior disorder, hallucinations and cognitive impairment in Parkinson’s disease: a two-year follow up. Mov Disord. 2008;23:1441–5.
Romenets SR, Gagnon JF, Latreille V, et al. Rapid eye movement sleep behavior disorder and subtypes of Parkinson’s disease. Mov Disord. 2012;27:996–1003.
Videnovic A, Marlin C, Alibiglou L, Planetta PJ, Vaillancourt DE, Mackinnon CD. Increased REM sleep without atonia in Parkinson disease with freezing of gait. Neurology. 2013;81:1030–5.
Marras C, Chaudhuri KR. Nonmotor features of Parkinson’s disease subtypes. Mov Disord. 2016;31:1095–102.
Ansari M, Rahmani F, Dolatshahi M, Pooyan A, Aarabi MH. Brain pathway differences between Parkinson’s disease patients with and without REM sleep behavior disorder. Sleep Breath. 2017;21:155–61.
Rahmani F, Ansari M, Pooyan A, Mirbagheri MM, Aarabi MH. Differences in white matter microstructure between Parkinson's disease patients with and without REM sleep behavior disorder. In: Engineering in Medicine and Biology Society (EMBC), 2016 IEEE 38th Annual International Conference of the; 2016: IEEE, 2016. p. 1124–6.
Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71:670–6.
Plazzi G, Corsini R, Provini F, et al. REM sleep behavior disorders in multiple system atrophy. Neurology. 1997;48:1094–7.
Palma JA, Fernandez-Cordon C, Coon EA, et al. Prevalence of REM sleep behavior disorder in multiple system atrophy: a multicenter study and meta-analysis. Clin Auton Res. 2015;25:69–75.
Nomura T, Inoue Y, Hogl B, et al. Comparison of the clinical features of rapid eye movement sleep behavior disorder in patients with Parkinson’s disease and multiple system atrophy. Psychiatry Clin Neurosci. 2011;65:264–71.
Tachibana N, Oka Y. Longitudinal change in REM sleep components in a patient with multiple system atrophy associated with REM sleep behavior disorder: paradoxical improvement of nocturnal behaviors in a progressive neurodegenerative disease. Sleep Med. 2004;5:155–8.
De Cock VC, Debs R, Oudiette D, et al. The improvement of movement and speech during rapid eye movement sleep behaviour disorder in multiple system atrophy. Brain. 2011;134:856–62.
Muntean ML, Sixel-Doring F, Trenkwalder C. No difference in sleep and RBD between different types of patients with multiple system atrophy: a pilot video-polysomnographical study. Sleep Disord. 2013;2013:258390.
Vetrugno R, Alessandria M, D’Angelo R, et al. Status dissociatus evolving from REM sleep behaviour disorder in multiple system atrophy. Sleep Med. 2009;10:247–52.
Miglis MG, Muppidi S, During E, Jaradeh S. A case series of REM sleep behavior disorder in pure autonomic failure. Clin Auton Res. 2017;27:41–4.
Hague K, Lento P, Morgello S, Caro S, Kaufmann H. The distribution of Lewy bodies in pure autonomic failure: autopsy findings and review of the literature. Acta Neuropathol. 1997;94:192–6.
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.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer International Publishing AG, part of Springer Nature
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-319-90152-7_5
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-90151-0
Online ISBN: 978-3-319-90152-7
eBook Packages: MedicineMedicine (R0)