Rapid eye movement sleep behavior disorder

Rapid eye movement sleep behavior disorder (RBD) is a sleep disorder (more specifically a parasomnia) in which people act out their dreams. It involves abnormal behavior during the sleep phase with rapid eye movement (REM) sleep. The major feature of RBD is loss of muscle atonia (i.e., the loss of paralysis) during otherwise intact REM sleep (during which paralysis is not only normal but necessary). REM sleep is the stage of sleep in which most vivid dreaming occurs. The loss of motor inhibition leads to a wide spectrum of behavioral release during sleep. This extends from simple limb twitches to more complex integrated movement. These behaviors can be violent in nature and in some cases will result in injury to either the individual or their bedmates.[1][2]

Rapid eye movement sleep behavior disorder
Other namesRBD, REM sleep behavior disorder
Sleep talking in a person with RBD
SpecialtyPsychiatry, Sleep medicine

RBD is a very strong predictor of progression to a synucleinopathy (usually Parkinson's disease or dementia with Lewy bodies).[3][4] Melatonin is useful in the treatment of RBD.[3] RBD was first described in 1986.

Symptoms

RBD is characterized by the dreamer acting out their dreams, with complex behaviors.[2] These dreams often involve screaming, shouting, laughing, crying, arm flailing, kicking, punching, choking, and even jumping out of bed. The actions in an episode can result in injuries to oneself or one's bedmate.[2][1] The sleeping person may be unaware of these movements.[2][1] Dreams often involve violent or aggressive actions, and an attack theme like being chased by people or animals. Because violence in dreams is more likely to be recalled, this could be an artifact of recall bias or selection bias.[1] The individual with RBD may not be aware of having it.[4]

In a normal sleep cycle, REM sleep may be experienced at intervals of between 90 minutes and two hours every night, which means RBD episodes may occur some four times a night. In a rare case, they may only happen once a week or once a month. Episodes occur more towards the morning hours because that is when REM sleep is more frequent. When awakened, people can usually recall the dream they were having, which will match the actions they were performing.[5]

As the first indication of an underlying neurodegenerative disorder, symptoms of RBD may begin years or decades before other the onset of another condition.[2]

Almost half of those with Parkinson's, at least 88% of those with multiple system atrophy, and about 80% of people with Lewy body dementia have RBD.[1] RBD is a very strong predictor of progression to a synucleinopathy (for example, the Lewy body dementias).[3] On autopsy, up to 98% of individuals with polysomnography-confirmed RBD are found to have a synucleinopathy.[3]

Symptomatic RBD can also be associated with narcolepsy, Guillain Barre syndrome, limbic encephalitis, and Morvan's syndrome.[4]

Other symptoms found in patients with RBD are reduced motor abilities, posture and gait changes, mild cognitive impairment, alterations in the sense of smell, impairments in color vision, autonomic dysfunction (orthostatic hypotension, constipation, urinary problems and sexual dysfunction), and depression.[4]

Causes

Rapid eye movement behavior disorder occurs when there is a loss of normal voluntary muscle atonia during REM sleep resulting in motor behavior in response to dream content. It can be caused by adverse reactions to certain drugs or during drug withdrawal; however, it is most often associated with the elderly and in those with neurodegenerative disorders such as Parkinson's disease and other neurodegenerative diseases, for example multiple system atrophy and the Lewy body dementias.[1][2]

The underlying cause of RBD is not well understood,[2] but it is likely that RBD is an early symptom of synucleinopathy rather than a separate disorder.[6] Brainstem circuits that control atonia during REM sleep may be damaged,[6] including those in the pontomedullary brainstem.[4] REM sleep circuits are located in caudal brainstem structures—the same structures that are known to lead to be implicated in the synucleinopathies.[6] Motor deficits like those seen in RBD are known to result from lesions in those circuits.[6]

Risk factors for developing IRBD are a family history of acting out dreams, prior head injury, farming, exposure to pesticides, low education level, depression, and use of antidepressants.[4]

RBD has also been reported following cerebrovascular accident and neurinoma (tumor), indicating that damage to the brain stem area may precipitate RBD. RBD is usually chronic. However, it may be acute and sudden in onset if associated with drug treatment or withdrawal (particularly with alcohol withdrawal). Monoamine oxidase inhibitors, tricyclic antidepressants, Selective serotonin reuptake inhibitors, and noradrenergic antagonists can induce or aggravate RBD symptoms and should be avoided in individuals with RBD.

Diagnosis

There are two ways to diagnose RBD: by documenting a history of complex, dream-enactment sleep behaviors, or by polysomnography recording of these behaviors along with REM sleep atonia loss.[2]

RBD may be established from clinical interview as well as several validated questionnaires, when sleep studies cannot be performed.[2][6] Questionnaires such as the Rapid Eye Movement (REM) sleep Behavior Disorder Screening Questionnaire (RBDSQ), the REM Sleep Behavior Questionnaires – Hong-Kong (RBD-HK), the Mayo Sleep Questionnaire (MSQ) and the Innsbruck REM Sleep Behavior Disorder Inventory are well-validated.[2]

Individuals with RBD may not be able to provide a history of dream enactment behavior, so bed partners are also consulted.[1][7] The REM Sleep Behavior Disorder Single-Question Screen offers diagnostic sensitivity and specificity in the absence of polysomnography with one question:[2]

"Have you ever been told, or suspected yourself, that you seem to 'act out your dreams' while asleep (for example, punching, flailing your arms in the air, making running movements, etc.)?"[8]

Differential

Other conditions are similar to RBD in that individuals exhibit excessive sleep movement and potentially violent behavior. Such disorders include non-REM parasomnias (sleepwalking, sleep terrors), periodic limb movement disorder, severe obstructive sleep apnea, and disassociative disorders.[4] Because of the similarities between the conditions, polysomnography plays an important role in confirming RBD diagnosis.

Classification

RBD is a parasomnia. It is categorized as either idiopathic or symptomatic.[1] Idiopathic RBD is the term used when RBD is not associated with another ongoing neurological condition.[4] When it results from an identifiable underlying etiology, RBD is referred to as symptomatic RBD (and considered a symptom of the underlying disorder). Up to almost 92% of patients with idiopathic RBD will go on to develop a neurodegenerative disorder. The disorders most strongly associated with RBD are the synucleinopathies, particularly Parkinson's disease, dementia with Lewy bodies, and to a lesser extent, multiple system atrophy.[2][4]

The following diagnostic criteria for RBD are given by the International Classification of Sleep Disorders (ICSD-3)[9][10]:

  1. Repetition of vocalizations and/or complex motor behaviors during sleep
  2. Polysomnography (PSG) show that these behaviors occur during REM sleep
  3. If documentation of these behaviors by PSG is not possible, they must at least be assumed to take place during REM sleep based on records of dream enactment
  4. REM sleep without atonia (RWA) can be seen in polysomnographic recordings
  5. Episodes cannot be explained by another mental disorder, sleep disorder, substance abuse or medication

These ICSD criteria make sure that diagnosis of RBD can only be made with help of polysomnography.[10] This method is not available everywhere and so a lot of questionnaires have been developed for the screening of RBD.[10] However, these questionnaires only allow a preliminary diagnosis.[10]

Treatment

RBD is treatable (even when the underlying synucleinopathies are not). Melatonin and clonazepam are the most frequently used,[2] and are comparably effective,[11] but melatonin offers a safer alternative, because clonazepam can produce undesirable side effects.[7] Other medications and treatments are available, but have only anecdotal evidence.[12]

Medications that may worsen RBD and should be stopped if possible are tramadol, mirtazapine, antidepressants, and beta blockers.[2]

In addition to medication, it is wise to secure the sleeper's environment by removing potentially dangerous objects from the bedroom and either place a cushion round the bed or move the mattress to the floor for added protection against injuries.[2] Some extreme sufferers sleep in a sleeping bag zipped up to their neck, and wear mittens so they can't unzip it until they awake in the morning.[13][14]

Patients are advised to maintain a normal sleep schedule, avoid sleep deprivation, and keep track of any sleepiness they may have. Treatment includes regulating neurologic symptoms and treating any other sleep disorders that might interfere with sleep. Sleep deprivation, alcohol, certain medications, and other sleep disorders can all increase RBD and should be avoided if possible.[15]

Prognosis

Patients with RBD are at risk for sleep-related injury (SRI) : between 33% and 65% of RBD patients reported to have had sleep related injury to self or their partner. [16][17]

Most people with RBD will convert to α synucleinopathy—usually Parkinson's disease or dementia with Lewy bodies—within 4 to 9 years from diagnosis of RBD, and 11 to 16 years from onset of symptoms.[4] For patients with degenerative dementia, symptoms of RBD and/or sleep related injuries can decline over time.[17] Visuospatial and constructional impairments are also found in patient with idiopathic RBD who had no other neurological disorder.[18]

Epidemiology

RBD prevalence as of 2017 is estimated to be 0.5–2% overall, and 5–13% of those aged 60 to 99.[1] It is more common in males overall, but equally frequent among men and women below the age of 50.[2] This may partially be due to a referral bias, as violent activity carried out by men is more likely to result in harm and injury and is more likely to be reported than injury to male bed partners by women, or it may reflect a true difference in prevalence as a result of genetic or androgenic factors. Typical onset is in the 50s or 60s.[2]

History

In the 1960s and 1970s, Michel Jouvet described brain lesions in cats that led to loss of atonia in REM sleep.[2][19][20] Carlos Schenck and Mark Mahowald and their team in Minnesota first described RBD in 1986.[2][21]

In animals

RBD has also been diagnosed in animals; specifically dogs.[22]

See also

References

  1. St Louis EK, Boeve AR, Boeve BF (May 2017). "REM Sleep Behavior Disorder in Parkinson's Disease and Other Synucleinopathies". Mov. Disord. (Review). 32 (5): 645–658. doi:10.1002/mds.27018. PMID 28513079.
  2. St Louis EK, Boeve BF (November 2017). "REM Sleep Behavior Disorder: Diagnosis, Clinical Implications, and Future Directions". Mayo Clin. Proc. (Review). 92 (11): 1723–1736. doi:10.1016/j.mayocp.2017.09.007. PMC 6095693. PMID 29101940.
  3. Boot BP (2015). "Comprehensive treatment of dementia with Lewy bodies". Alzheimers Res Ther (Review). 7 (1): 45. doi:10.1186/s13195-015-0128-z. PMC 4448151. PMID 26029267. Lay summary Family Practice News (April 17, 2013). Original study here.
  4. Arnaldi D, Antelmi E, St Louis EK, Postuma RB, Arnulf I (December 2017). "Idiopathic REM sleep behavior disorder and neurodegenerative risk: To tell or not to tell to the patient? How to minimize the risk?". Sleep Med Rev (Review). 36: 82–95. doi:10.1016/j.smrv.2016.11.002. PMID 28082168.
  5. "REM sleep behavior disorder - Symptoms and causes - Mayo Clinic". Retrieved 2019-07-11. Movement, such as kicking, punching, arm flailing or jumping from bed, in response to action-filled or violent dreams, such as being chased or defending yourself from an attack ... Being able to recall the dream if you awaken during the episode
  6. McKenna D, Peever J (May 2017). "Degeneration of rapid eye movement sleep circuitry underlies rapid eye movement sleep behavior disorder". Mov. Disord. (review). 32 (5): 636–644. doi:10.1002/mds.27003. PMID 28394031.
  7. McKeith IG, Boeve BF, Dickson DW, et al. (July 2017). "Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium" (PDF). Neurology (Review). 89 (1): 88–100. doi:10.1212/WNL.0000000000004058. PMC 5496518. PMID 28592453.
  8. Tousi B (October 2017). "Diagnosis and management of cognitive and behavioral changes in dementia with Lewy bodies". Curr Treat Options Neurol (Review). 19 (11): 42. doi:10.1007/s11940-017-0478-x. PMID 28990131.
  9. American Academy of Sleep Medicine (2014). International classification of sleep disorders, 3rd edn. American Academy of Sleep Medicine, Darien, IL
  10. Högl, B., & Stefani, A. (2017). REM sleep behavior disorder (RBD): Update on diagnosis and treatment. Somnologie : Schlafforschung und Schlafmedizin = Somnology : sleep research and sleep medicine, 21(Suppl 1), 1–8. doi:10.1007/s11818-016-0048-6
  11. McCarter SJ, et al. (March 2013). "Treatment Outcomes in REM Sleep Behavior Disorder". Sleep Medicine (Review). 14 (3): 237–242. doi:10.1016/j.sleep.2012.09.018. PMC 3617579. PMID 23352028.
  12. Jung Y, St Louis EK (November 2016). "Treatment of REM Sleep Behavior Disorder". Curr Treat Options Neurol (Review). 18 (11): 50. doi:10.1007/s11940-016-0433-2. PMID 27752878.
  13. Birbiglia Mike and Ira Glass (2008-08-08). "Fear of Sleep". This American Life. Retrieved 2016-09-07.
  14. American Academy of Sleep Medicine (2012-01-26). "Sleepwalk with Me: Comedian's sleep disorder experience comes to film". sleepeducation.org. Retrieved 2016-09-07.
  15. Schutte-Rodin S. "REM Sleep Behavior Disorder". yoursleep.aasmnet.org. American Academy of Sleep Medicine. Retrieved 1 October 2011.
  16. Comella, C.L., Nardine T.M., Diederich N.J., Stebbins G.T. (1998). Sleep-related violence, injury, and REM sleep behavior disorder in Parkinson’s disease. Neurology. 51:526-9. doi:10.1212/wnl.51.2.526
  17. Boeve, B. F., Silber, M. H., Ferman, T. J., et al. (1998). REM sleep behavior disorder and degenerative dementia: An association likely reflecting Lewy body disease. Neurology. 51:363-70. doi:10.1212/wnl.51.2.363
  18. Ferini-Strambi, L., Di Gioia, M. R., Castronovo, V., Oldani, A., Zucconi, M., Cappa, S. F. (2004). Neuropsychological assessment in idiopathic REM sleep behavior disorder (RBD): Does the idiopathic form of RBD really exist? Neurology. 62:41-5. doi:10.1212/01.wnl.0000101726.69701.fa
  19. Jouvet M (April 1967). "Neurophysiology of the states of sleep". Physiol. Rev. (Review). 47 (2): 117–77. doi:10.1152/physrev.1967.47.2.117. PMID 5342870.
  20. Sakai K, Sastre JP, Salvert D, Touret M, Tohyama M, Jouvet M (November 1979). "Tegmentoreticular projections with special reference to the muscular atonia during paradoxical sleep in the cat: an HRP study". Brain Res. 176 (2): 233–54. doi:10.1016/0006-8993(79)90981-8. PMID 227527.
  21. Schenck CH, Bundlie SR, Ettinger MG, Mahowald MW (June 1986). "Chronic behavioral disorders of human REM sleep: a new category of parasomnia". Sleep. 9 (2): 293–308. doi:10.1093/sleep/9.2.293. PMID 3505730.
  22. Carey S (2001-02-13). "Dog with Rare Sleeping Disorder Sent Home After Unique Diagnosis at UF's Veterinary Medical Teaching Hospital". University of Florida. Retrieved 2010-01-02.
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