Presumed rapid eye movement behavior disorder in Machado‐Joseph disease (spinocerebellar ataxia type 3)

Friedman, Joseph H.

doi:10.1002/mds.10269

Cited by 61 publications

(36 citation statements)

References 11 publications

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“…There are a few recent reports on RBD associated with spinocerebellar atrophy type 3 (Machado-Joseph disease). [63][64][65] As noted above, numerous cases of RBD have been reported in conjunction with MSA, 3,22,23,27,[66][67][68][69][70][71][72] PD, 3,11,22,[24][25][26][27][30][31][32][33][34][35]73 and DLB. 1,10,15,41,45,[47][48][49][50][51][52][53]55,59,60,74 Although frequency data on the presence of RSWA and RBD in the neurodegenerative disorders is based on relatively small numbers of patients thus far, the presence of each in MSA has been reported to be 90% to 95% for RSWA and 68% to 90% for RBD, 66,67 and presence of each in PD is approximately 58% for RSWA and 33% for RBD.…”

Section: The Rbd-synucleinopathy Associationmentioning

confidence: 99%

REM Sleep Behavior Disorder in Parkinson’s Disease and Dementia with Lewy Bodies

Boeve

Silber

Ferman

2004

J Geriatr Psychiatry Neurol

206

106

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Rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia manifested by vivid, often frightening dreams associated with simple or complex motor behavior during REM sleep. Patients appear to "act out their dreams," in which the exhibited behaviors mirror the content of the dreams. Management of RBD involves counseling about safety measures in the sleep environment; in those at risk for injury, clonazepam and/or melatonin is usually effective. In this article, the authors present a detailed review of the clinical and polysomnographic features, differential diagnosis, diagnostic criteria, management strategies, and pathophysiologic mechanisms of RBD. They then review the literature and their institutional experience of RBD associated with neurodegenerative disease, particularly Parkinson's disease and dementia with Lewy bodies. The evolving data suggests that RBD may have clinical diagnostic and pathophysiologic significance in isolation and when associated with neurodegenerative disease. (J Geriatr Psychiatry Neurol 2004; 17:146-157)

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Section: The Rbd-synucleinopathy Associationmentioning

confidence: 99%

REM Sleep Behavior Disorder in Parkinson’s Disease and Dementia with Lewy Bodies

Boeve

Silber

Ferman

2004

J Geriatr Psychiatry Neurol

206

106

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“…Diverse etiologies include cases of tauopathy related parkinsonian syndromes (Progressive supranuclear palsy, Guadaloupean parkinsonism) [149][150][151], TDP43opathies (frontotemporal dementia, amyotrophic lateral sclerosis) [7,152], amyloidopathies (Alzheimer's disease) [7,153]. RBD has also been associated with some trinucleatide repeat disorders including spinal cerebellar ataxia type 3 (SCA3) [154][155][156][157] and Huntington's disease [158]. However, with the notable exception of SCA3, none of these conditions have prevalence rates similar to synuclein disorders.…”

Section: Non-synuclein Neurodegenerative Etiologiesmentioning

confidence: 99%

Parasomnias: An Updated Review

2012

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Parasomnias are abnormal behaviors emanating from or associated with sleep. Sleepwalking and related disorders result from an incomplete dissociation of wakefulness from nonrapid eye movement (NREM) sleep. Conditions that provoke repeated cortical arousals, or promote sleep inertia lead to NREM parasomnias by impairing normal arousal mechanisms. Changes in the cyclic alternating pattern, a biomarker of arousal instability in NREM sleep, are noted in sleepwalking disorders. Sleep-related eating disorder (SRED) is characterized by a disruption of the nocturnal fast with episodes of feeding after an arousal from sleep. SRED is often associated with the use of sedative-hypnotic medications; in particular, the widely prescribed benzodiazepine receptor agonists. Recently, compelling evidence suggests that nocturnal eating may in some cases be a nonmotor manifestation of Restless Legs Syndrome (RLS). rapid eye movement (REM) Sleep Behavior Disorder (RBD) is characterized by a loss of REM paralysis leading to potentially injurious dream enactment. The loss of atonia in RBD often predates the development of Parkinson's disease and other disorders of synuclein pathology. Parasomnia behaviors are related to an activation (in NREM parasomnias) or a disinhibition (in RBD) of central pattern generators (CPGs). Initial management should focus on decreasing the potential for sleep-related injury followed by treating comorbid sleep disorders. Clonazepam and melatonin appear to be effective therapies in RBD, whereas paroxetine has been reported effective in some cases of sleep terrors. At this point, pharmacotherapy for other parasomnias is less certain, and further investigations are necessary.Keywords Parasomnia . Sleepwalking . Sleep terrors . REM sleep behavior disorder . Restless legs syndrome Classification of ParasomniasParasomnias are typically classified by the sleep state from which they arise: non-rapid eye movement sleep (NREM) and rapid eye movement sleep (REM) (see Table 1 NREM parasomnias include: confusional arousals, sleepwalking disorder, and sleep terrors. These behaviors arise when the cortex incompletely arouses from deep NREM sleep, often due to comorbid conditions that provoke repeated arousal or promote sleep inertia. Changes in the cyclic alternating pattern, a biomarker of arousal instability in NREM sleep, are noted in sleepwalking and related disorders [3].Sleep-related eating disorder (SRED) is currently classified in the International Classification of Sleep Disorders, 2nd edition (ICSD-2) under "Other Parasomnia" [1]. However the vast majority of SRED cases emanate from NREM [4], and the complex amnestic behaviors with ambulation are striking similar to sleepwalking [5]. Furthermore, both SRED and sleepwalking are frequently triggered by sedating agents in the setting of underlying motor restlessness [6]. Thus, considering these similarities, SRED will be reviewed immediately after NREM parasomnias.REM sleep behavior disorder is the most clinically relevant REM parasomnia. The loss of atoni...

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“…While sexual dysfunction has been widely noted in patients with PD, there is also indication that the severity of erectile dysfunction may predict conversion from iRBD to neurodegeneration [64]. Erectile dysfunction is higher in patients with iRBD compared to controls [48], although the predictive value remains low. Urinary dysfunction has also been found to a higher degree in iRBD patient than in controls [66], and is considered a prodromal feature of PD [65].…”

Section: Autonomic Dysrhythmias Associated With Rbdmentioning

confidence: 98%

“…In regards to the experimental rat model of PLMs-RBD [7], the impetus for pursuing this model involving the inferior colliculus (IC) was that an abnormal auditory brainstem evoked potential wave V had previously been reported in RBD patients [46], as well as in spinocerebellar ataxia patients [47] who also can have RBD [48][49][50][51]. The external cortex of the IC (ICX) has been demonstrated not only to be involved in auditory processing, but also to participate in the modulation of motor activity.…”

Section: Periodic Leg Movements (Plms) Associated With Rbdmentioning

confidence: 99%

REM sleep behavior disorder rhythms

Irfan¹,

Schenck²

2017

Transl Brain Rhythmicity

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REM sleep behavior disorder (RBD) is an intriguing parasomnia that is characterized by repeated dream enactment associated with increased muscle tone and twitch burst activity in REM sleep. This article explores a unique perspective on the relationship of RBD with various neurophysiological rhythms. We describe the clinical and polysomnographic characteristics of this disorder with diagnostic considerations. Arousal disorders simulating RBD, including Non-REM Sleep Arousal Parasomnias, Obstructive Sleep Apnea and Periodic Limb Movement Disorder, are also described. The pathophysiological basis is discussed with reference to the physiological substrates of RBD subtypes. Periodic Leg Movements associated with RBD, Rhythmic Masticatory Muscle Activity (Sleep Bruxism) and Oromandibular Myoclonus Associated with RBD, Rhythmic Movement Disorder associated with RBD, interface of RBD with epileptic abnormalities, and sleep spindles are also elaborated upon. REM sleep in relation to ultradian sleep rhythmsSleep occurs in ultradian cycles of Non-Rapid Eye Movement (NREM) sleep followed by Rapid Eye Movement sleep, ranging from 90-120 minutes, alternating throughout the night. Typically, it comprises of 3-5 cycles per night. The duration of later REM sleep periods is longer than those noted in the earlier half of the night. REM sleep density, which is a measure of frequency of rapid eye movements in REM sleep, is also higher in the last third of the night. REM sleep has been hypothesized to be involved in memory consolidation through processing between the hippocampus and neocortex [1]. Neurophysiological monitoring of sleep requires recording of the electroencephalogram (EEG), electrooculogram (EOG) and electromyogram (EMG) [2]. Polysomnographic characteristics of normal REM sleepThe REM stage of sleep is normally characterized by low voltage intermixed frequency EEG waves, rapid eye movements, and markedly decreased skeletal muscle tone [2]. REM sleep is also called paradoxical sleep where EEG is desynchronized. On polysomnographic (PSG) monitoring, REMs are characterized as irregular sharp conjugate eye movements with initial duration <500ms. Saw-tooth waves form a distinctive pattern occurring in REM sleep, which are temporally associated with REMs. They are triangular waves with frequency of 2-6 Hz with maximum amplitude in central leads [2]. An example of a 30-sec epoch of REM sleep is shown in Figure 1A.In feline models, ponto-geniculo-occipital (PGO) spikes have been described to originate in the pons, propagate to the lateral geniculate nucleus, and then are observed in the occipital cortex. It is hypothesized that they are related to saw-tooth waves as PGO bursts occur with high probability in relation to REMs [3]. Clusters of PGO-like subthalamic waves occurring before or during REMs have also been recorded in humans, associated with enhancement of fast oscillatory subthalamic activity [4]. PGO waves have been conventionally regarded as neurophysiological correlates of dreaming [4]. REM sleep behavior d...

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Presumed rapid eye movement behavior disorder in Machado‐Joseph disease (spinocerebellar ataxia type 3)

Cited by 61 publications

References 11 publications

REM Sleep Behavior Disorder in Parkinson’s Disease and Dementia with Lewy Bodies

REM Sleep Behavior Disorder in Parkinson’s Disease and Dementia with Lewy Bodies

Parasomnias: An Updated Review

REM sleep behavior disorder rhythms

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