the concept of “idiopathic” rbd and the multitude of findings on ancillary studies in those labeled with idiopathic rbd will next be reviewed. there is a large amount of recent data suggesting that many patients with irbd actually represent an evolving neurodegenerative disorder (discussed in detail later in this review), which has fostered many authors to qualify the term idiopathic rbd with surrounding quotation markers (ie, “idiopathic” rbd). while individuals may grunt, speak, laugh, or vocalize in a variety of ways during non-rem (nrem) and rem sleep, and such vocalizations are not necessarily “abnormal,” the vocalizations in rbd tend to be loud and suggest unpleasant dream mentation. those with significant dementia may not be able to recall and/or describe their dreams; in such cases, bedpartner observations of the abnormal behaviors are helpful. this is by no means a sound epidemiologic study of the prevalence of rbd in a population, but the frequency of 20/100,000 could at least be considered a minimum estimate of the frequency of rbd in one county who have come to medical attention, undergone a psg, and had the diagnosis confirmed. some have constructed plywood barriers placed along side the bed and on the bed in between the patient and spouse, with padding affixed to the sides of the plywood facing the patient. as noted above, most patients experience a marked improvement in the frequency and severity of rbd features with medical therapy. the major clinical syndromes and histopathologic disorders which cause dementia and/or parkinsonism are shown in figure 4. as shown in this figure, numerous cases of rbd have been reported in association with certain neurodegenerative disorders, but not reported to date in association with most others. in the study by postuma et al,80 the neuropsychological profile of probable ad cases was indistinguishable to those with probable dlb, which suggests such cases have underlying lbd; these data are consistent with other data showing rbd associated with dementia but not visual hallucinations or parkinsonism likely reflects underlying lbd.83 our updated clinicopathologic experience involving patients evaluated at mayo clinic rochester and mayo clinic jacksonville who had rbd associated with cognitive impairment/dementia and/or parkinsonism is shown in table 4, which demonstrates the preponderance of lbd and msa in this series (41/43=95%). hence, rbd preceding the motor and cognitive features of a neurodegenerative disorder may be particularly common in the synucleinopathies, and there is considerable interest to study patients with “idiopathic” rbd (discussed in much more detail in the sections that follow). they interpreted these findings as showing that the olfactory deficit found in most irbd patients are similar to that described in pd, and that dysnosmia may be a sign of a widespread neurodegenerative process. the authors concluded that the impaired cognitive profile in patients with irbd is similar to that observed in early stages of some synucleinopathies.134 terzaghi et al. similar to the concept of irbd evolving into a pd-predominant phenotype as discussed above, one could hypothesis a similar progression in cognitive functioning with increasing age and disease severity in evolving lbd in the dlb-predominant phenotype (figure 8).
the onset of rbd typically begins years or decades prior to the onset of cognitive decline and a diagnosis of mci, with subtle and often asymptomatic motor signs (ie, mps – represented by the area shaded in light yellow) evolving concurrently or after the onset of cognitive decline. the authors concluded that perfusional abnormalities in patients with irbd were located in the brainstem, striatum, and cortex, and that such findings are consistent with the anatomic metabolic profile of parkinson disease.146 in a study focused on olfactory function in patients with rbd, stiasny-kolner et al. yet there is growing appreciation of the importance of identifying patients with rbd for clinical and particularly research purposes. there is debate as to the terminology and clinical significance of increased emg tone during rem sleep (i.e., rem sleep without atonia or rswa) in patients who have never exhibited dream enactment behavior. the answer to this question is certainly “no.” there are many other medical conditions which have been associated with rbd and some of these may be etiologically related. this author tends to discuss this issue briefly at the time of diagnosis, and in more detail over subsequent visits, again depending on the circumstances with each individual patient. rbd may present years or decades prior to the onset of cognitive impairment in patients diagnosed with dlb or pd. perhaps many of the clinical features and ancillary test findings discussed in this review, and hopefully many others yet to be identified or developed, will function as accurate biomarkers. the challenge for basic scientists is to develop therapies that impact the cascade of events involved in synucleinopathy and other neurodegenerative disorder pathophysiologies. idiopathic rbd is one of the more intriguing clinical curiosities in medicine, and certainly in sleep medicine and neurology. as per the braak staging scheme, the temporal sequence of α-synuclein pathology begins mainly in the medulla and then ascends to the cortex (6 stages). the onset of rbd typically begins years or decades prior to the onset of cognitive decline and a diagnosis of mild cognitive impairment (mci), with subtle and often asymptomatic motor signs (mild parkinsonian signs or mps – represented by the area shaded in light yellow) evolving concurrently or after the onset of cognitive decline. the challenge for investigators at present is to study adequate numbers of patients with “idiopathic” rbd with a spectrum of clinical tests and potential biomarkers longitudinally to assess the natural history and prepare for future clinical trials.
rem sleep behavior disorder (rbd) is a chronic sleep condition characterized by dream enactment and loss of rem atonia. in contrast, rbd individuals will maintain rem sleep during and immediately after most of their dream enactments. figure 1. key brain regions and neurotransmitters involved in regulation and maintenance of the rem sleep stage under healthy normative or pathological rbd conditions. the true prevalence of rbd in the general population is very difficult to gauge. if and why women are susceptible to underreporting rbd, as in the case of snoring and obstructive sleep apnoea (46, 47), therefore needs further investigation. individuals suspected of having rbd should be referred to a specialist sleep medicine or neurology service for a diagnostic assessment (see figure 2 for an outline of the rbd diagnostic process). for patients with rbd presenting as part of a clinically-defined neurodegenerative condition, such as pd, msa, or dlb, the management of their sleep disorder should form part of their holistic care. in pd, for example, the presence of concomitant rbd is associated with a greater non-motor burden and a more adverse prognosis (68–71). there have been no randomized, double-blind, controlled trials of clonazepam in an irbd population, and only a handful of studies have looked at the effects of the drug on sleep and rbd symptoms. clonazepam must be used with caution in the elderly, individuals with a history of depression and those with airways obstruction (90), such as obstructive sleep apnoea which is commonly concomitant with rbd (91). the evidence for melatonin’s efficacy is variable: several studies have found it to reduce rbd motor behavior occurrence (101–103), with long-term use ameliorating rbd symptoms in the majority of patients (104). ultimately, the development of a unified assessment scale for clinical-practice deployment to rbd patients is recommended. from a public health perspective, rbd highlights the importance of sleep for good health and the need for greater awareness of sleep disorders and their detrimental effects. while the prognosis of idiopathic rbd remains uncertain, an increase in basic and clinical research into the condition is already leading to greater understanding and endpoint prediction. video analysis of motor events in rem sleep behavior disorder. doi: 10.1007/s00702-017-1759-y 10. cygan f, oudiette d, leclair-visonneau l, leu-semenescu s, arnulf i. night-to-night variability of muscle tone, movements, and vocalizations in patients with rem sleep behavior disorder. risk and predictors of dementia and parkinsonism in idiopathic rem sleep behaviour disorder: a multicentre study. the association between narcolepsy and rem behavior disorder (rbd). rem sleep behavior disorder in parkinson’s disease: a questionnaire-based survey.
prevalence of rem sleep behavior disorder in multiple system atrophy: a multicenter study and meta-analysis. prevalence and determinants of rapid eye movement sleep behavior disorder in the general population. rem sleep behavior disorder in the korean elderly population: prevalence and clinical characteristics. the persistent gender bias in the diagnosis of obstructive sleep apnea. environmental risk factors for rem sleep behavior disorder: a multicenter case-control study. circadian rhythms of melatonin and peripheral clock gene expression in idiopathic rem sleep behavior disorder. snca 3′utr genetic variants in patients with parkinson’s disease and rem sleep behavior disorder. national validation and proposed revision of rem sleep behavior disorder screening questionnaire (rbdsq). rem sleep behaviour disorder is associated with worse quality of life and other non-motor features in early parkinson’s disease. doi: 10.1016/j.smrv.2016.11.002 75. devnani p, fernandes r. management of rem sleep behavior disorder: an evidence based review. rem sleep eeg instability in rem sleep behavior disorder and clonazepam effects. doi: 10.1093/sleep/32.9.1149 87. nardi ae, perna g. clonazepam in the treatment of psychiatric disorders: an update. doi: 10.1007/s11325-017-1563-9 92. chouinard g. issues in the clinical use of benzodiazepines: potency, withdrawal, and rebound. pramipexole in the treatment of rem sleep behaviour disorder: a critical review. doi: 10.1111/j.1365-2869.2010.00848.x 102. kunz d, bes f. melatonin as a therapy in rem sleep behavior disorder patients: an open-labeled pilot study on the possible influence of melatonin on rem-sleep regulation. a review of sleep disorders and melatonin. alpha-synuclein rt-quic in the csf of patients with alpha-synucleinopathies. alpha-synuclein aggregates in the parotid gland of idiopathic rem sleep behavior disorder. lack of training in sleep and sleep disorders. keywords: remsleep behavior disorder (rbd), parkinson’s disease, prodromal parkinson’s disease, sleep disorders, neurology, neuroscience, sleep citation: roguski a, rayment d, whone al, jones mw and rolinski m (2020) a neurologist’s guide to rem sleep behavior disorder.
rapid eye movement (rem) sleep behavior disorder is a sleep disorder in which you physically act out vivid, often unpleasant dreams with rem sleep behavior disorder is a condition characterized by sudden body movements and vocalizations while a person experiences vivid dreams in a person with rem sleep behavior disorder (rbd), the paralysis that normally happens during rem sleep is incomplete or absent,, .
introduction rapid eye movement (rem) sleep behavior disorder (rbd) is a parasomnia characterized by dream-enactment behaviors that emerge during a loss of rem sleep atonia. rbd dream enactment ranges in severity from benign hand gestures to violent thrashing, punching, and kicking. rapid eye movement (rem) sleep behavior disorder (rbd) is a parasomnia manifested by vivid, often frightening dreams associated with simple or complex motor what is rapid eye movement (rem) sleep? what parasomnias happen during this sleep stage? nightmare disorder: these are vivid dreams that cause rem sleep behavior disorder (rbd) is a chronic sleep condition characterized by dream enactment and loss of rem atonia., .
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