REM Sleep Behavior Disorder


Article Author:
Imran Khawaja


Article Editor:
Shantanu Singh



Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Kyle Blair
Trevor Nezwek
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Daniyal Ameen
Altif Muneeb
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
6/4/2019 7:56:07 PM

Introduction

Rapid eye movement (REM) sleep behavior disorder (RBD) is a REM sleep parasomnia characterized by dream enactment during sleep.[1] Patients act out during their dreams while being in the REM stage of the sleep. This enactment may be violent and can lead to injury to themselves or others without any conscious awareness. A patient can recall the contents of the dream upon awakening. Most patient's with REM behavior disorder will eventually manifest neurodegenerative diseases like parkinsonism, dementia with Lewy body, or multisystem atrophy.

There are also secondary cases of RBD associated with narcolepsy or use of antidepressant medications.

A patient may be warned about the future development of these neurological disorders. Treatment consists of preventive measures while sleeping to avoid injury to the patient as well as the bed partner. In severe cases, the patient may be prescribed melatonin or clonazepam.

Etiology

RBD occurs because of the failure to inhibit spinal motor neuron during REM sleep. A strong association between RBD and future development of a neurodegenerative disorder has been well established.[2] Neurodegenerative disorders linked to alpha-synuclein positive intracellular inclusions, for example, parkinsonism, Lewy body dementia, and multiple system atrophy (MSA), are associated with alpha-synucleinopathies. Nuclei in the pons control REM sleep. Lesions in pons can lead to the development of these synucleinopathies and RBD.[3] A progressive degeneration of these nuclei may explain the RBD as a prodrome before full onset of the disease spectrum. Neuroimaging studies have shown progressive dopaminergic abnormalities in patients with RBD.[4] Dopaminergic agents like antidepressants may, therefore, worsen RBD. In narcolepsy, about 50% of patients may manifest RBD. Lack of orexin in narcolepsy may fail to stabilize REM sleep and results in a lack of muscle atonia during REM sleep.[5]

Epidemiology

The overall prevalence of “spontaneous” RBD is estimated to be about 1% in the general population and 2% in older individuals.[6] It is more prevalent in elderly males than females with a male to female ratio of 9 to 1.[7] There is a strong association of RBD with many neurodegenerative disorders. RBD can be a precursor to more serious conditions involving alpha-synuclein neuropathies such as parkinsonism, multiple system atrophy or dementia with Lewy body where prevalence can be as high as 76% to 81% of affected individuals.[3] In a case series,[8] about half of the patients with RBD converted to a neurologic disorder within 12 years.

Causes of “secondary” RBD, especially in younger individuals, include narcolepsy or use of antidepressant medications. Prevalence of RBD in narcolepsy has been reported to be to be as high as 36%.[5] Antidepressant medications can precipitate RBD-type symptoms in up to 6% of cases [9]. Other secondary causes of RBD may include vascular lesions, tumors, demyelinating disease, autoimmune, or inflammatory disorders.

Obstructive sleep apnea (OSA) may mimic RBD and is referred to as “pseudo RBD.”[10]

Pathophysiology

There are 2 systems involved in normal REM sleep; one generates muscle atonia and other for suppresses motor-skeletal activity. Muscle atonia involves active inhibition by neurons in the medulla. Locomotion involves input from forebrain, and the thalamus influences spinal motor neurons. Several brainstem pontine regions  have been implicated in RBD pathophysiology including the peri-locus coeruleus region, pedunculo-pontine nucleus (PPN) and laterodorsal tegmental nucleus (LDTN).[11] Supra-spinal mechanism handles REM atonia. During REM sleep, nuclei from the pons excite neurons in the medulla, which then transmit descending inhibitory projections to spinal alpha motor-neurons resulting in hyperpolarization and muscle atonia. It is the disinhibition of these neurons that leads to muscle activity during the REM stage of sleep.[2]

History and Physical

Physicians should get a thorough sleep history regarding nocturnal movements to assess the nature of parasomnia. Sleep history may help to identify whether the symptoms are happening during the REM or non-REM stage of the sleep. A history of epileptic activity may be explored. Patients themselves may not know of motor activity during sleep. In a study of 203 patients with RBD,[12] only about half of the patients knew of their symptoms. The movements may be short and range in severity. These may include punching, kicking, falling out of bed, gesturing, or knocking over the nightstand. Patients may have vocalizations during an attack. Schenck et al.[13] reported dream enactment in 87% of their study population. Patients had the vivid, intense, action-filled and, violent dreams coincident with the onset of RBD. Sleep-related injuries occurred in 79% of patients in this series of patients.

Symptoms predominantly occur in the second half of the night when the REM sleep is most prevalent and usually occur during the last REM sleep period.

In milder forms, patients may sleep through the event, but in severe cases, patients may transiently wake up but then fall asleep.

Patients may be examined for the development of any neurodegenerative disorders.

Evaluation

According to the third edition of the International Classification of Sleep Disorders (ICSD-3), a diagnosis of RBD requires all of the following[14]:

  • Repeated episodes of sleep-related vocalization and/or complex motor behaviors
  • Behaviors are documented by polysomnography to occur during REM sleep or, based on the clinical history of dream enactment, are presumed to occur during REM sleep
  • Presence of REM sleep without atonia (RSWA) on polysomnography
  • An absence of epileptiform activity during REM sleep, unless RBD can be clearly distinguished from any concurrent REM sleep-related seizure disorder
  • Sleep disturbance not better explained by another sleep disorder, medical or neurologic disorder, mental disorder, medication use, or substance use disorder

RBD is the only parasomnia which requires an in-facility polysomnogram to diagnose RBD.

The characteristic polysomnographic finding of RBD is REM sleep without atonia (RSWA). It is an elevation of motor tone during REM sleep as measured by electromyography (EMG) activity in the chin and/or limb leads.[15]

Formal polysomnographic criteria for RSWA developed by the American Academy of Sleep Medicine require either of the following[14]:

  • Sustained elevation of chin EMG activity during REM sleep (greater than 50% of the 30-second epoch duration compared with minimum amplitude in non-REM sleep)
  • Excessive bursts of transient muscle activity in the chin or limb EMG during REM sleep, defined by the presence of 5 (50%) or more mini-epochs (30-second epoch is divided into 10 sequential 3-second mini-epochs), containing bursts of transient muscle activity. In RBD, excessive transient muscle activity bursts last 0.1 to 5.0 seconds and are at least 4 times as high in amplitude as the background EMD activity.

Optimally, both upper and lower extremity EMG should be used when evaluating for RBD. Alternate EMG derivations that make use of upper extremity EMG to improve sensitivity for detecting RSWA are reviewed separately.

The disorder needs to be differentiated from other parasomnias including both REM and non-REM sleep parasomnias including nightmares, night terrors, sleepwalking and sleep talking. Periodic leg movements may present with limb movement while sleeping but generally occur during the non-REM stage of the sleep and have distinctive diagnostic criteria on polysomnography.

Epileptic disorders like sleep-related hyperosmolar epilepsy (frontal lobe epilepsy) may present with motor activity during sleep. However, compared with RBD, these patients are generally younger and unaware of their symptoms.

Treatment / Management

The primary goal of treatment is to provide patients with a safe sleeping environment for them and their bed partners. Healthcare professionals can achieve this through non-pharmacologic approaches and pharmacotherapy if needed.

It is important to counsel patients and their bed partners on avoidance of potentially hazardous and injuries objects near the patients, for example, firearms or glass objects. Bed partners should be educated on the disease, and the patient’s enactments during dreams are not under voluntary control.

Sleeping alone may be advisable in severe cases. Many patients may require padded bed rails or must sleep in a sleeping bag.[16]

Patients may be advised to stop SSRI and tricyclic antidepressants that are known to cause or exacerbate RBD.[17]

Regarding pharmacotherapy, melatonin is now considered the first-line therapy in the treatment of RBD.[18] Its mechanism of action is unknown, but in doses between 6 to 18 mg, it augments REM atonia and improves RBD symptoms. Patients are typically started at doses of 3 mg, and then doses are increased in 3-mg increments until the RBD symptoms resolve.

Low-dose clonazepam (0.5 to 1 mg at bedtime) has been traditionally used to control RBD symptoms. Its mechanism of action is not clear either but may help suppress unpleasant dreams.[17] Its use may be limited due to side effects. In one study, 39% of patients reported side effects.[12]

Pearls and Other Issues

RBD can be a disturbing disorder for both patients and their bed partners. Both are prone to injuries due to violent behavior. Most patients with spontaneous RBD can eventually develop a neurodegenerative disorder. The rate of conversion is about 50% every 10 years [8]. Patients should be informed that they are at risk of developing neurodegenerative disorders. Secondary causes of RBD may need to be evaluated through a complete sleep history and review of medications. Any precipitating factors or medications causing or exacerbating RBD need to be avoided. In proper clinical settings, brain imaging with MRI and EEG may be recommended to evaluate for a secondary cause of RBD. This is the only parasomnia where polysomnography is required to diagnose RBD and rule out severe OSA as a cause of “pseudo RBD.” Patients may be advised to create a safer environment in the bedroom to avoid injuries. Melatonin is the first-line treatment, and in refractory cases, clonazepam in lower doses may be tried.

Enhancing Healthcare Team Outcomes

Sleep behavior disorder is often difficult to diagnose and management. Thus, it is best managed with a multidisciplinary team that includes mental health nurses. The primary goal of treatment is to provide patients with a safe sleeping environment for them and their bed partners. Healthcare professionals can achieve this through non-pharmacologic approaches and pharmacotherapy if needed. It is important to counsel patients and their bed partners on avoidance of potentially hazardous and injuries objects near the patients, for example, firearms or glass objects. Bed partners should be educated on the disease, and the patient’s enactments during dreams are not under voluntary control.


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

REM Sleep Behavior Disorder - Questions

Take a quiz of the questions on this article.

Take Quiz
Spontaneous REM behavior disorder (RBD) can precede the development of which medical condition?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
What is the first line medical therapy for treatment of REM behavior disorder?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
In which population is spontaneous REM behavior disorder (RBD) more prevalent?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which one of the following parasomnias requires polysomnography as part of the diagnostic evaluation?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
In which groups of patients should REM sleep behavior disorder (RBD) be suspected the most?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

REM Sleep Behavior Disorder - References

References

Schenck CH,Bundlie SR,Ettinger MG,Mahowald MW, Chronic behavioral disorders of human REM sleep: a new category of parasomnia. 1986 [classical article]. Sleep. 2002 Mar 15     [PubMed]
Boeve BF,Silber MH,Saper CB,Ferman TJ,Dickson DW,Parisi JE,Benarroch EE,Ahlskog JE,Smith GE,Caselli RC,Tippman-Peikert M,Olson EJ,Lin SC,Young T,Wszolek Z,Schenck CH,Mahowald MW,Castillo PR,Del Tredici K,Braak H, Pathophysiology of REM sleep behaviour disorder and relevance to neurodegenerative disease. Brain : a journal of neurology. 2007 Nov     [PubMed]
Boeve BF, REM sleep behavior disorder: Updated review of the core features, the REM sleep behavior disorder-neurodegenerative disease association, evolving concepts, controversies, and future directions. Annals of the New York Academy of Sciences. 2010 Jan     [PubMed]
Shin HY,Joo EY,Kim ST,Dhong HJ,Cho JW, Comparison study of olfactory function and substantia nigra hyperechogenicity in idiopathic REM sleep behavior disorder, Parkinson's disease and normal control. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 2013 Jun     [PubMed]
Nightingale S,Orgill JC,Ebrahim IO,de Lacy SF,Agrawal S,Williams AJ, The association between narcolepsy and REM behavior disorder (RBD). Sleep medicine. 2005 May     [PubMed]
Haba-Rubio J,Frauscher B,Marques-Vidal P,Toriel J,Tobback N,Andries D,Preisig M,Vollenweider P,Postuma R,Heinzer R, Prevalence and Determinants of REM Sleep Behavior Disorder in the General Population. Sleep. 2017 Dec 5     [PubMed]
Bjørnarå KA,Dietrichs E,Toft M, REM sleep behavior disorder in Parkinson's disease--is there a gender difference? Parkinsonism     [PubMed]
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 Apr 14     [PubMed]
Teman PT,Tippmann-Peikert M,Silber MH,Slocumb NL,Auger RR, Idiopathic rapid-eye-movement sleep disorder: associations with antidepressants, psychiatric diagnoses, and other factors, in relation to age of onset. Sleep medicine. 2009 Jan     [PubMed]
Iranzo A,Santamaría J, Severe obstructive sleep apnea/hypopnea mimicking REM sleep behavior disorder. Sleep. 2005 Feb     [PubMed]
Hendricks JC,Morrison AR,Mann GL, Different behaviors during paradoxical sleep without atonia depend on pontine lesion site. Brain research. 1982 May 6     [PubMed]
Fernández-Arcos A,Iranzo A,Serradell M,Gaig C,Santamaria J, The Clinical Phenotype of Idiopathic Rapid Eye Movement Sleep Behavior Disorder at Presentation: A Study in 203 Consecutive Patients. Sleep. 2016 Jan 1     [PubMed]
Schenck CH,Mahowald MW, REM sleep behavior disorder: clinical, developmental, and neuroscience perspectives 16 years after its formal identification in SLEEP. Sleep. 2002 Mar 15     [PubMed]
Berry RB,Brooks R,Gamaldo C,Harding SM,Lloyd RM,Quan SF,Troester MT,Vaughn BV, AASM Scoring Manual Updates for 2017 (Version 2.4). Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine. 2017 May 15     [PubMed]
Neikrug AB,Ancoli-Israel S, Diagnostic tools for REM sleep behavior disorder. Sleep medicine reviews. 2012 Oct     [PubMed]
Howell MJ, Parasomnias: an updated review. Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics. 2012 Oct     [PubMed]
Postuma RB,Gagnon JF,Tuineaig M,Bertrand JA,Latreille V,Desjardins C,Montplaisir JY, Antidepressants and REM sleep behavior disorder: isolated side effect or neurodegenerative signal? Sleep. 2013 Nov 1     [PubMed]
Boeve BF,Silber MH,Ferman TJ, Melatonin for treatment of REM sleep behavior disorder in neurologic disorders: results in 14 patients. Sleep medicine. 2003 Jul     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of your specialty. When it is time for the board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study.