REM Sleep Behavior Disorder
- Author: Syed M S Ahmed, MD; Chief Editor: Selim R Benbadis, MD more...
Rapid eye movement (REM) sleep behavior disorder (RBD) is a sleep disorder characterized by the loss of normal voluntary muscle atonia during REM sleep, in association with complex motor behavior while dreaming.[1, 2, 3, 4]
Diagnostic Criteria (DSM-5 and ICSD-2)
The specific DSM-5 criteria for rapid eye movement sleep behavior disorder are as follows :
Recurrent episodes of arousal during sleep associated with vocalization and/or complex motor behaviors that arise during rapid eye movement (REM) sleep
On waking from these episodes, the individual is not confused or disoriented and is completely alert
Either of the following is present: REM sleep without atonia on polysomnographic recordings; or a history suggestive of REM sleep behavior disorder and an established synucleinopathy diagnosis (e.g., Parkinson’s disease, multiple system atrophy)
The episodes cause significant distress or impairment in social, occupational or other areas of functioning which may include serious injury to self or the bed partner
The disturbance cannot be explained by the effects of a drug of abuse or medication
The episodes cannot be attributed to another mental disorder or medical condition
The International Classification of Sleep Disorders requires the following criteria for the clinical diagnosis of RBD :
Presence of REM sleep without atonia (RSWA) on polysomnography (PSG)
The presence of at least one of the following conditions: (1) sleep-related behaviors, by history, that have been injurious, potentially injurious, or disruptive (e.g., dream enactment behavior); (2) abnormal REM sleep behavior documented during PSG monitoring
Absence of epileptiform activity on electroencephalogram (EEG) during REM sleep (unless RBD can be clearly distinguished from any concurrent REM sleep-related seizure disorder)
Sleep disorder not better explained by another sleep disorder, a medical or neurologic disorder, a mental disorder, medication use, or a substance use disorder
The precise etiology and neural structures involved in RBD are unknown. Based on animal (cats, rats), lesional, and neuropathologic studies, sleep-regulating nuclei, particularly the pontine tegmentum, are thought to be involved in the pathogenesis of RBD. Also, a complex interplay of various neurochemical systems, such as the noradrenergic, cholinergic, and serotonergic systems, seems to exist in the pathogenesis of the condition.[1, 6]
Normally, generalized atonia of skeletal muscles occurs during REM sleep. This atonia results from active inhibition of the final common pathway of spinal motor neurons by way of the medullary magnocellular reticular formation (MCRF); this suppresses anterior horn cell activity via projections of the ventral lateral reticulospinal tract.
Various pontine nuclei are known to influence the REM and non-REM sleep circuits, including the locus coeruleus(LC), pedunculopontine nucleus (PPN), and laterodorsal tegmental nucleus (LDTN). In addition, forebrain cortical and subcortical structures and the substantia nigra, thalamus, hypothalamus, basal forebrain, and frontal cortex are also involved. However, their precise roles are unknown.
RBD may be idiopathic. However, several studies have suggested that idiopathic RBD is a potential marker for the later development of neurodegenerative disorders characterized by alpha-synuclein deposition. These include Parkinson disease, multiple system atrophy, dementia with Lewy bodies, and pure autonomic failure, with the risk varying among different studies. (RBD is less frequently associated with nonsynucleinopathies.)[1, 2, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17]
In fact, studies have suggested that RBD may be associated with alpha-synuclein–mediated degeneration of sleep-regulating nuclei in the brainstem, particularly the pontine tegmentum.
In essence, RBD may be the prodrome of neurodegenerative disease, such as diffuse Lew body (DLB) disease or Parkinson disease.[1, 18] In experimental studies in cats, bilateral pontine lesions resulted in a persistent absence of REM atonia associated with prominent motor activity during REM sleep similar to that observed in RBD in humans.
Studies by Eisensehr et al using iodine 123 (123 I) immunoperoxidase technique (IPT) single photon-emission computed tomography (SPECT) scanning demonstrated that striatal presynaptic dopamine transporters are reduced in idiopathic RBD.
Studies by Fantini et al demonstrated impairment of cortical activity in idiopathic RBD, particularly in the occipital region during both wakefulness and REM sleep, compared with controls. Results were similar to those of functional studies, such as of the perfusion and metabolic impairment pattern, observed in DLB disease and in Parkinson disease. Similar cortical activity in the frontal and temporal regions was impaired only during wakefulness.
Research evidence suggests, therefore, that many cases of idiopathic RBD may not be truly idiopathic, leading some to suggest the term cryptogenic rather than idiopathic.
In addition, RBD may occur in association with various neurologic conditions (ie, secondary RBD), including vascular lesions, brainstem neoplasms, demyelinating disease, autoimmune/inflammatory disorders, and neurodegenerative disorders.
Nightingale et al suggested in a study that 36% of persons with narcolepsy experience symptoms of RBD. This link led to the identification of a strong association of RBD with HLA class II genes.[23, 24, 25]
According to DSM-5, the prevalence of REM sleep behavior disorder is approximately 0.38%-0.5% in the general population. Prevalence in patients with psychiatric disorders is greater, possibly related to medications prescribed for psychiatric disorders.
The prognosis of RBD depends on its etiology. In idiopathic cases, the symptoms are controlled with medications. In secondary cases, the prognosis depends on the underlying primary disease.
No deaths have been reported in idiopathic cases of RBD; however, patients and bed partners may experience serious injury. In the reported cases, 32% of patients had injured themselves and 64% had assaulted their spouse. Subdural hematomas occurred in two patients. In secondary cases, the morbidity and mortality rates depend on the underlying disease itself.
REM sleep disorder is present concurrently in approximately 30% of patients with narcolepsy. When comorbid with narcolepsy, RBD presents in younger patients with equal frequency in males and females. Based on findings from sleep studies, most individuals (.50%) with initially “idiopathic” RBD will eventually develop a neurodegenerative disease, most notably, one of the synucleinopathies (e.g., Parkinson disease, multiple system atrophy, dementia with Lewy bodies). REM sleep behavior disorder often predates any other sign of these disorders by many years (often more than a decade).
Educate the patient and his or her bed partner for environmental safety. Potentially dangerous objects should be removed from the bedroom, and the mattress should be placed on the floor or a cushion placed around the bed. (See Treatment.)
Boeve BF, Silber MH, Saper CB, et al. Pathophysiology of REM sleep behaviour disorder and relevance to neurodegenerative disease. Brain. 2007 Nov. 130:2770-88. [Medline].
Gagnon JF, Postuma RB, Mazza S, Doyon J, Montplaisir J. Rapid-eye-movement sleep behaviour disorder and neurodegenerative diseases. Lancet Neurol. 2006 May. 5(5):424-32. [Medline].
Massicotte-Marquez J, Carrier J, Decary A, et al. Slow-wave sleep and delta power in rapid eye movement sleep behavior disorder. Ann Neurol. 2005 Feb. 57(2):277-82. [Medline].
Olson EJ, Boeve BF, Silber MH. Rapid eye movement sleep behaviour disorder: demographic, clinical and laboratory findings in 93 cases. Brain. 2000 Feb. 123 ( Pt 2):331-9. [Medline].
American Academy of Sleep Medicine. International Classification of Sleep Disorders: Diagnostic and Coding Manual. 2nd ed. Westchester, Ill: 2005.
Hendricks JC, Morrison AR, Mann GL. Different behaviors during paradoxical sleep without atonia depend on pontine lesion site. Brain Res. 1982 May 6. 239(1):81-105. [Medline].
Rye DB. Contributions of the pedunculopontine region to normal and altered REM sleep. Sleep. 1997 Sep. 20(9):757-88. [Medline].
Abad VC, Guilleminault C. Review of rapid eye movement behavior sleep disorders. Curr Neurol Neurosci Rep. 2004 Mar. 4(2):157-63. [Medline].
Boeve BF, Silber MH, Parisi JE, et al. Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism. Neurology. 2003 Jul 8. 61(1):40-5. [Medline].
Massicotte-Marquez J, Decary A, Gagnon JF, et al. Executive dysfunction and memory impairment in idiopathic REM sleep behavior disorder. Neurology. 2008 Apr 8. 70(15):1250-7. [Medline].
Boeve BF, Saper CB. REM sleep behavior disorder: a possible early marker for synucleinopathies. Neurology. 2006 Mar 28. 66(6):796-7. [Medline].
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 Jul. 5(7):572-7. [Medline].
Montplaisir J. Abnormal motor behavior during sleep. Sleep Med. 2004 Jun. 5 Suppl 1:S31-4. [Medline].
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 Feb. 46(2):388-93. [Medline].
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 Mar. 5(2):155-8. [Medline].
Nihei Y, Takahashi K, Koto A, Mihara B, Morita Y, Isozumi K, et al. REM sleep behavior disorder in Japanese patients with Parkinson's disease: a multicenter study using the REM sleep behavior disorder screening questionnaire. J Neurol. 2012 Jan 10. [Medline].
Sasai T, Miyamoto T, Miyamoto M, Iwanami M, Abe T, Matsuura M, et al. Impaired decision-making in idiopathic REM sleep behavior disorder. Sleep Med. 2012 Jan 26. [Medline].
Postuma RB, Bertrand JA, Montplaisir J, Desjardins C, Vendette M, Rios Romenets S, et al. Rapid eye movement sleep behavior disorder and risk of dementia in Parkinson's disease: A prospective study. Mov Disord. 2012 Feb 9. [Medline].
Eisensehr I, Linke R, Noachtar S, et al. Reduced striatal dopamine transporters in idiopathic rapid eye movement sleep behaviour disorder. Comparison with Parkinson's disease and controls. Brain. 2000 Jun. 123 ( Pt 6):1155-60. [Medline].
Fantini ML, Gagnon JF, Petit D, et al. Slowing of electroencephalogram in rapid eye movement sleep behavior disorder. Ann Neurol. 2003 Jun. 53(6):774-80. [Medline].
Ferini-Strambi L, Di Gioia MR, Castronovo V, Oldani A, Zucconi M, Cappa SF. Neuropsychological assessment in idiopathic REM sleep behavior disorder (RBD): does the idiopathic form of RBD really exist?. Neurology. 2004 Jan 13. 62(1):41-5. [Medline].
Nightingale S, Orgill JC, Ebrahim IO, et al. The association between narcolepsy and REM behavior disorder (RBD). Sleep Med. 2005 May. 6(3):253-8. [Medline].
Schenck CH, Garcia-Rill E, Segall M, et al. HLA class II genes associated with REM sleep behavior disorder. Ann Neurol. 1996 Feb. 39(2):261-3. [Medline].
Peever J. Control of motoneuron function and muscle tone during REM sleep, REM sleep behavior disorder and cataplexy/narcolepsy. Arch Ital Biol. 2011 Dec 1. 149(4):[Medline].
Franceschini C, Ferri R, Pizza F, Ricotta L, Vandi S, Detto S, et al. Motor events during REM sleep in patients with narcolepsy-cataplexy: a video-polysomnographic pilot study. Sleep Med. 2011 Dec. 12 Suppl 2:S59-63. [Medline].
Schenck CH, Hurwitz TD, Mahowald MW. Symposium: Normal and abnormal REM sleep regulation: REM sleep behaviour disorder: an update on a series of 96 patients and a review of the world literature. J Sleep Res. 1993 Dec. 2(4):224-231. [Medline].
Gagnon JF, Postuma RB, Montplaisir J. Update on the pharmacology of REM sleep behavior disorder. Neurology. 2006 Sep 12. 67(5):742-7. [Medline].
Comella CL, Nardine TM, Diederich NJ, Stebbins GT. Sleep-related violence, injury, and REM sleep behavior disorder in Parkinson's disease. Neurology. 1998 Aug. 51(2):526-9. [Medline].
Takeuchi N, Uchimura N, Hashizume Y, et al. Melatonin therapy for REM sleep behavior disorder. Psychiatry Clin Neurosci. 2001 Jun. 55(3):267-9. [Medline].
Iranzo A, Santamaria J. Severe obstructive sleep apnea/hypopnea mimicking REM sleep behavior disorder. Sleep. 2005 Feb 1. 28(2):203-6. [Medline].
Kempfner J, Sorensen GL, Sorensen HB, Jennum P. Automatic REM sleep detection associated with idiopathic rem sleep Behavior Disorder. Conf Proc IEEE Eng Med Biol Soc. 2011 Aug. 2011:6063-6. [Medline].
Culebras A, Moore JT. Magnetic resonance findings in REM sleep behavior disorder. Neurology. 1989 Nov. 39(11):1519-23. [Medline].
Plazzi G, Corsini R, Provini F, et al. REM sleep behavior disorders in multiple system atrophy. Neurology. 1997 Apr. 48(4):1094-7. [Medline].
Boeve BF, Silber MH, Ferman TJ. Melatonin for treatment of REM sleep behavior disorder in neurologic disorders: results in 14 patients. Sleep Med. 2003 Jul. 4(4):281-4. [Medline].
Kunz D, Bes F. Exogenous melatonin in periodic limb movement disorder: an open clinical trial and a hypothesis. Sleep. 2001 Mar 15. 24(2):183-7. [Medline].
Winkelman JW, James L. Serotonergic antidepressants are associated with REM sleep without atonia. Sleep. 2004 Mar 15. 27(2):317-21. [Medline].
American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition. Arlington, VA: American Psychiatric Association; 2013.