Rapid eye movement sleep behavior disorder

Rapid eye movement sleep behavior disorder
Rapid eye movement sleep behavior disorder
Other names	REM behavior disorder
	Sleep talking in a person with RBD
Specialty	Psychiatry, Sleep medicine

Last updated June 03, 2024

Rapid eye movement sleep behavior disorder or REM sleep behavior disorder (RBD) is a sleep disorder 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). The loss of motor inhibition leads to sleep behaviors ranging from simple limb twitches to more complex integrated movements that can be violent or result in injury to either the individual or their bedmates.^[1]^[2]

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 cause, RBD is referred to as symptomatic RBD, and considered a symptom of the underlying disorder.^[4]

Characteristics

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 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] When awakened, people may be able to recall the dream they were having, which will match the actions they were performing.^[6]

As the first indication of an underlying neurodegenerative disorder or synucleinopathy, symptoms of RBD may begin years or decades before the onset of another condition.^[2] Abnormal sleep behaviors may begin decades before any other symptoms, often as the first clinical indication of another condition.^[1]

Symptomatic RBD can also be associated with narcolepsy, Guillain–Barré syndrome, limbic encephalitis, and Morvan's syndrome.^[7]

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.^[8] Brainstem circuits that control atonia during REM sleep may be damaged,^[8] 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.^[8] Motor deficits like those seen in RBD are known to result from lesions in those circuits.^[8]

Risk factors for developing RBD 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 may be acute and sudden in onset if associated with drug treatment or withdrawal (particularly with alcohol withdrawal). Antidepressant medications can induce or aggravate RBD symptoms.^[9]

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]^[8] 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]^[10] 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.)?"^[11]

Diagnostic criteria for RBD from the International Classification of Sleep Disorders (ICSD-3) are:^[12]

Repetition of vocalizations and/or complex motor behaviors during sleep
Polysomnography (PSG) show that these behaviors occur during REM sleep
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
REM sleep without atonia (RWA) can be seen in polysomnographic recordings
Episodes cannot be explained by another mental disorder, sleep disorder, substance abuse or medication

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 dissociative disorders.^[4] Because of the similarities between the conditions, polysomnography plays an important role in confirming RBD diagnosis.

Treatment

RBD is treatable (even when the underlying synucleinopathies are not). Melatonin and clonazepam are the most frequently used,^[2] and are comparably effective,^[13] but melatonin offers a safer alternative, because clonazepam can produce undesirable side effects.^[10]

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 around the bed or move the mattress to the floor for added protection against injuries.^[2] In extreme cases, an affected individual has slept in a sleeping bag zipped up to their neck, wearing mittens so they cannot unzip it until they awake.^[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.^[7]

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] Most people with RBD will convert to a 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]

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]

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).^[5] On autopsy, up to 98% of individuals with polysomnography-confirmed RBD are found to have a synucleinopathy.^[5]

History

In the 1960s and 1970s, Michel Jouvet described brain lesions in cats that led to loss of atonia in REM sleep.^[2]^[16]^[17] Carlos Schenck and Mark Mahowald and their team in Minnesota first described RBD in 1986.^[2]^[18]

In animals

RBD has also been diagnosed in animals, specifically dogs.^[19]

Related Research Articles

Dementia is a syndrome associated with many neurodegenerative diseases, characterized by a general decline in cognitive abilities that impacts a person's ability to perform everyday activities. This typically involves problems with memory, thinking, behavior, and motor control. Aside from memory impairment and a disruption in thought patterns, the most common symptoms of dementia include emotional problems, difficulties with language, and decreased motivation. The symptoms may be described as occurring in a continuum over several stages. Dementia ultimately has a significant effect on the individual, their caregivers, and their social relationships in general. A diagnosis of dementia requires the observation of a change from a person's usual mental functioning and a greater cognitive decline than might be caused by the normal aging process.

A sleep disorder, or somnipathy, is a medical disorder of an individual's sleep patterns. Some sleep disorders are severe enough to interfere with normal physical, mental, social and emotional functioning. Sleep disorders are frequent and can have serious consequences on patients' health and quality of life. Polysomnography and actigraphy are tests commonly ordered for diagnosing sleep disorders.

Parkinsonism is a clinical syndrome characterized by tremor, bradykinesia, rigidity, and postural instability. Both hypokinetic as well as hyperkinetic features are displayed by Parkinsonism.These are the four motor symptoms found in Parkinson's disease (PD) – after which it is named – dementia with Lewy bodies (DLB), Parkinson's disease dementia (PDD), and many other conditions. This set of symptoms occurs in a wide range of conditions and may have many causes, including neurodegenerative conditions, drugs, toxins, metabolic diseases, and neurological conditions other than PD.

Dementia with Lewy bodies (DLB) is a type of dementia characterized by changes in sleep, behavior, cognition, movement, and regulation of automatic bodily functions. Memory loss is not always an early symptom. The disease worsens over time and is usually diagnosed when cognitive impairment interferes with normal daily functioning. Together with Parkinson's disease dementia, DLB is one of the two Lewy body dementias. It is a common form of dementia, but the prevalence is not known accurately and many diagnoses are missed. The disease was first described on autopsy by Kenji Kosaka in 1976, and he named the condition several years later.

Lewy body dementia (LBD) is an umbrella term for two similar and common subtypes of dementia: dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD). Both are characterized by changes in thinking, movement, behavior, and mood. The two conditions have similar features and may have similar causes, and are believed to belong on a spectrum of Lewy body disease that includes Parkinson's disease. As of 2014, they were more often misdiagnosed than any other common dementia.

Nightmare disorder is a sleep disorder characterized by repeated intense nightmares that most often center on threats to physical safety and security. The nightmares usually occur during the REM stage of sleep, and the person who experiences the nightmares typically remembers them well upon waking. More specifically, nightmare disorder is a type of parasomnia, a subset of sleep disorders categorized by abnormal movement or behavior or verbal actions during sleep or shortly before or after. Other parasomnias include sleepwalking, sleep terrors, bedwetting, and sleep paralysis.

Hypersomnia is a neurological disorder of excessive time spent sleeping or excessive sleepiness. It can have many possible causes and can cause distress and problems with functioning. In the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5), hypersomnolence, of which there are several subtypes, appears under sleep-wake disorders.

Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by tremors, slow movement, muscle rigidity, and postural instability, autonomic dysfunction and ataxia. This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum.

Parkinson-plus syndromes (PPS) are a group of neurodegenerative diseases featuring the classical features of Parkinson's disease with additional features that distinguish them from simple idiopathic Parkinson's disease (PD). Parkinson-plus syndromes are either inherited genetically or occur sporadically.

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Parasomnias are a category of sleep disorders that involve abnormal movements, behaviors, emotions, perceptions, and dreams that occur while falling asleep, sleeping, between sleep stages, or during arousal from sleep. Parasomnias are dissociated sleep states which are partial arousals during the transitions between wakefulness, NREM sleep, and REM sleep, and their combinations.

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Parkinson's disease (PD), or simply Parkinson's, is a long-term neurodegenerative disease of mainly the central nervous system that affects both the motor system and non-motor systems. The symptoms usually emerge slowly, and as the disease progresses, non-motor symptoms become more common. Usual symptoms are tremor, slowness of movement, rigidity, and difficulty with balance, collectively known as parkinsonism. Parkinson's disease dementia, falls and neuropsychiatric problems such as sleep abnormalities, psychosis, mood swings, or behavioral changes may arise in advanced stages.

Synucleinopathies are neurodegenerative diseases characterised by the abnormal accumulation of aggregates of alpha-synuclein protein in neurons, nerve fibres or glial cells. There are three main types of synucleinopathy: Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). Other rare disorders, such as various neuroaxonal dystrophies, also have α-synuclein pathologies. Additionally, autopsy studies have shown that around 6% of sporadic Alzheimer's Disease exhibit α-synuclein positive Lewy pathology, and are sub-classed as Alzheimer's Disease with Amygdalar Restricted Lewy Bodies (AD/ALB).

The REM Sleep Behavior Disorder Screening Questionnaire (RBDSQ) is a specific questionnaire for rapid eye movement behavior disorder (RBD) developed by Stiasny-Kolster and team, to assess the most prominent clinical features of RBD. It is a 10-item, patient self-rating instrument with short questions to be answered by either 'yes' or 'no'. The validity of the questionnaire was studied by researchers and they have observed it to perform with high sensitivity and reasonable specificity in the diagnosis of RBD.

Parkinson's disease dementia (PDD) is dementia that is associated with Parkinson's disease (PD). Together with dementia with Lewy bodies (DLB), it is one of the Lewy body dementias characterized by abnormal deposits of Lewy bodies in the brain.

Corticobasal syndrome (CBS) is a rare, progressive atypical Parkinsonism syndrome and is a tauopathy related to frontotemporal dementia. CBS is typically caused by the deposit of tau proteins forming in different areas of the brain.

The REM Sleep Behavior Disorder Single-Question Screen (RBD1Q) is a one-question screening tool for dream enactment behaviors associated with the parasomnia REM sleep behavior disorder (RBD). It screens for RBD with a simple yes/no response.

Rapid eye movement sleep behaviour disorder and Parkinson's disease is rapid eye movement sleep behavior disorder (RBD) that is associated with Parkinson's disease. RBC is linked genetically and neuropathologically to α- synuclein, a presynaptic neuronal protein that exerts deleterious effects on neighbouring proteins, leading to neuronal death. This pathology is linked to numerous other neurodegenerative disorders, such as Lewy body dementias, and collectively these disorders are known as synucleinopathies. Numerous reports over the past few years have stated the frequent association of synucleinopathies with REM sleep behaviour disorder (RBD). In particular, the frequent association of RBD with Parkinson's. In the general population the incidence of RBD is around 0.5%, compared to the prevalence of RBD in PD patients, which has been reported to be between 38% and 60%. The diagnosis and symptom onset of RBD typically precedes the onset of motor or cognitive symptoms of PD by a number of years, typically ranging anywhere from 2 to 15 years prior. Hence, this link could provide an important window of opportunity in the implementation of therapies and treatments, that could prevent or slow the onset of PD.

Yo-El Ju is the Barbara Burton and Reuben Morriss III Professor of Neurology at the Washington University School of Medicine. She co-directs the Center on Biological Rhythms and Sleep (COBRAS) and is a member of the Hope Center for Neurological Diseases at Washington University. Clinically, she sees patients at Barnes-Jewish Hospital for parasomnia, narcolepsy, restless legs syndrome, and obstructive sleep apnea. Ju's team has made multiple significant contributions to the field of sleep medicine and neurology in unveiling the complex relationship between sleep, amyloid deposition and neurodegenerative diseases such as Alzheimer's, opening new possibilities for clinical treatment. As of April 2023, the most cited work from her lab is their 2017 paper in Brain: A Journal of Neurology that showed cerebrospinal fluid (CSF) amyloid-beta protein level increases due to slow-wave sleep disruption.

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