REM and NREM Sleep Parasomnia in Anti-NMDA Receptor Encephalitis.

OBJECTIVES: Encephalitis with anti-N-methyl-d-aspartate receptor antibodies (anti-NMDARe) is a rare disorder characterized by cognitive impairment, psychosis, seizures, and abnormal movements. Abnormal behaviors during REM sleep have not been described in anti-NMDARe. METHODS: Patients were monitored by video-polysomnography on a first night followed by multiple sleep latency tests and 18 hours of bed rest. RESULTS: Two patients with anti-NMDARe developed during the acute and postacute phase parasomnias including REM sleep behavior disorder and continuous finalistic quiet gesturing during a mixed N2/R sleep. The parasomnia disorder was improved by gabapentin and clonazepam. DISCUSSION: Video-polysomnography avoids misdiagnosing these parasomnia behaviors for seizure or movement disorders and allows adequate treatment.

Shared EEG correlates between non-REM parasomnia experiences and dreams.

Sleepwalking and related parasomnias result from incomplete awakenings out of non-rapid eye movement sleep. Behavioral episodes can occur without consciousness or recollection, or in relation to dream-like experiences. To understand what accounts for these differences in consciousness and recall, here we recorded parasomnia episodes with high-density electroencephalography (EEG) and interviewed participants immediately afterward about their experiences. Compared to reports of no experience (19%), reports of conscious experience (56%) were preceded by high-amplitude EEG slow waves in anterior cortical regions and activation of posterior cortical regions, similar to previously described EEG correlates of dreaming. Recall of the content of the experience (56%), compared to no recall (25%), was associated with higher EEG activation in the right medial temporal region before movement onset. Our work suggests that the EEG correlates of parasomnia experiences are similar to those reported for dreams and may thus reflect core physiological processes involved in sleep consciousness.

Lamentations in the night: A systematic review on catathrenia.

Catathrenia is a loud expiratory moan during sleep that is a social embarrassment and is sometimes confused with central apnea on polysomnography. It affects about 4% of adults, but cases are rarely referred to sleep centers. Catathrenia affects males and females, children and adults, who are usually young and thin. A "typical" catathrenia begins with a deep inhalation, followed by a long, noisy exhalation, then a short, more pronounced exhalation, followed by another deep inhalation, often accompanied by arousal. The many harmonics of the sound indicate that it is produced by the vocal cords. It is often repeated in clusters, especially during REM sleep and at the end of the night. It does not disturb the sleepers, but their neighbors, and is associated with excessive daytime sleepiness in one-third of cases. The pathophysiology and treatment of typical catathrenia are still unknown. Later, a more atypical catathrenia was described, consisting of episodes of short (2 s), regular, semi-continuous expiratory moans during NREM sleep (mainly in stages N1 and N2) and REM sleep, often in people with mild upper airway obstruction. This atypical catathrenia is more commonly reduced by positive airway pressure and mandibular advancement devices that promote vertical opening.

"Catatrenia": un trastorno del sueño poco conocido: descripción de tres casos clínicos pediátricos / Catathrenia: unknown sleep disorder: report 3 cases in pediatric patients

Catatrenia (gemido nocturno) es una condición rara caracterizada por sonidos irregulares que ocurren durante el sueño. Los comportamientos ocurren intermitentemente durante cualquiera de las dos etapas de sueño, REM o NREM y se caracterizan por gemidos prolongados, a menudo muy fuertes, socialmente perturbadores, durante la expiración. Es poco conocido y espera más definición y estudios terapéuticos. Hay pocos reportes y en su mayoría de pacientes adultos. Se presentan 3 casos en pacientes pediátricos.

Catathrenia (nocturnal groaning) is a rare condition characterized by irregular sounds that occur during sleep. The behaviors occur intermittently during either REM or NREM sleep and are characterized by prolonged, often very loud, socially disruptive groaning sounds during expiration. It is poorly understood and awaits further definition and therapeutic studies. There are few reports mostly adult patients are presented below 3 cases in pediatric patients.

Rapid Eye Movement Behavior Disorder and Other Parasomnias.

Rapid eye movement (REM) behavior disorder (RBD) is characterized by loss of skeletal muscle atonia that can lead to dream enactment. This condition can cause harm to patients and their bed partners if appropriate safety measures are not ensured. This condition is often the initial presenting symptom in a group of complex neurodegenerative processes. Definitive diagnosis requires a thorough history and an in-laboratory polysomnogram to look for evidence of REM sleep without atonia. Treatment options are limited but consist of sleep safety measures and pharmacotherapy. Patients diagnosed with idiopathic RBD associated with alpha-synucleinopathy are likely to have progression of disease.

Contribution of brainstem circuits in pathophysiology of NREM-sleep parasomnias.

OBJECTIVE: We aimed to investigate the brainstem circuits to reveal if there was any abnormality in these circuits in clinically diagnosed patients with NREM parasomnias during wakefulness. METHODS: Twelve patients with NREM-sleep parasomnia diagnosed according to ICSD-3 criteria and a control group of 16 healthy subjects were enrolled into our study. We analyzed the auditory startle reflex (ASR), blink reflex (BR), prepulse inhibition (PPI) of BR and recovery excitability of BR. RESULTS: There was a trend for longer responses from orbicularis oculi and sternocleidomastoid muscles after auditory stimulation in the patients compared to those in the healthy subjects. The recovery percentages at 200 ms and 300 ms showed a borderline significance in the patients. No significant difference was found in the R2-PPI between the patients and healthy subjects. CONCLUSIONS: Our results suggest a mildly enhanced ASR and relatively early facilitation of BR excitability in patients with NREM-sleep parasomnia during daytime. Although our findings suggest involvement of brainstem networks in NREM-sleep parasomnia during wakefulness, it would be better to study these networks at night and during daytime to see if there is any contribution.

Restless Sleep Disorder.

Restless sleep disorder is (RSD) a condition characterized by frequent large movements during sleep associated with daytime impairment. RSD has been studied in children aged 6 to 18 years. Polysomnography is necessary for the diagnosis of RSD. The current diagnostic criteria include more than 5 large movements per hour of sleep documented by PSG. The pathophysiology is not known yet, but iron deficiency and sleep instability and increased sympathetic activation are suspected to play a role. Iron supplementation is the only treatment option studied so far.

Sleep and Epilepsy, Clinical Spectrum and Updated Review.

Electroencephalogram (EEG) recording is essential in the evaluation of complex movement and behaviors during sleep, but in particular for differentiating epileptic versus nonepileptic events. In general, epileptiform discharges occur with greater density in the first few nonerapid eye movement cycles, and approximately 12% to 20% of seizures occur exclusively at night. This review examines the epilepsy types and syndromes whose presentation is strongly influenced by the sleep state, with an appraisal about the role that sleep plays in facilitating seizures, while deleaneatign EEG findings and clinical manifestation. The review will summarize the typical semiology of sleep-related hypermotor seizures and contrasted with those occurring during none/rapid eye movement parasomnias and sleep-related movement disorders.

Perioperative Management of Insomnia, Restless Legs, Narcolepsy, and Parasomnias.

Obstructive sleep apnea (OSA) has been shown to increase risk of adverse perioperative events. More recently, investigators have begun to examine other common sleep disorders to assess how they may be impacted by the perioperative environment, as well as influence postoperative outcomes. There are a number of mechanisms by which such common sleep disorders (eg, insomnia, restless legs syndrome, narcolepsy, and parasomnias) may have consequences in the perioperative setting, both related to the underlying pathophysiology of the diseases as well as their treatments. This review will highlight the current state of the literature and offer recommendations for management of these conditions during the perioperative journey.

NonREM Disorders of Arousal and Related Parasomnias: an Updated Review.

Parasomnias are abnormal behaviors and/or experiences emanating from or associated with sleep typically manifesting as motor movements of varying semiology. We discuss mainly nonrapid eye movement sleep and related parasomnias in this article. Sleepwalking (SW), sleep terrors (ST), confusional arousals, and related disorders result from an incomplete dissociation of wakefulness from nonrapid eye movement (NREM) sleep. Conditions that provoke repeated cortical arousals, and/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 arousal from sleep. SRED is often associated with the use of sedative-hypnotic medications, in particular the widely prescribed benzodiazepine receptor agonists. Compelling evidence suggests that nocturnal eating may in some cases be another nonmotor manifestation of Restless Legs Syndrome (RLS). Initial management should focus upon decreasing the potential for sleep-related injury followed by treating comorbid sleep disorders and eliminating incriminating drugs. Sexsomnia is a subtype of disorders of arousal, where sexual behavior emerges from partial arousal from nonREM sleep. Overlap parasomnia disorders consist of abnormal sleep-related behavior both in nonREM and REM sleep. Status dissociatus is referred to as a breakdown of the sleep architecture where an admixture of various sleep state markers is seen without any specific demarcation. Benzodiazepine therapy can be effective in controlling SW, ST, and sexsomnia, but not SRED. Paroxetine has been reported to provide benefit in some cases of ST. Topiramate, pramipexole, and sertraline can be effective in SRED. Pharmacotherapy for other parasomnias continues to be less certain, necessitating further investigation. NREM parasomnias may resolve spontaneously but require a review of priming and predisposing factors.

Exploding head syndrome, chronotype, parasomnias and mental health in young adults.

Although inadequate sleep among young people is well documented in the literature, anomalous sleep experiences, such as the parasomnia termed exploding head syndrome (EHS), have received little empirical attention. The current study examined the association of sleep quality, symptoms of psychological distress and other unusual sleep experiences with EHS in a sample of young adults (n = 135, age M = 21.77, SD = 2.08). We also aimed to account for the possible effect of participant chronotype on sleep experiences. The lifetime prevalence of EHS among participants was 20.0%. Three-quarters (75.6%) of participants reported poor quality sleep according to the Pittsburgh Sleep Quality Index (PSQI). Univariate analysis showed that participants with a lifetime prevalence of EHS experienced more symptoms of anxiety and poorer sleep quality; age, gender and symptoms of depression were not significantly related to EHS. Parasomnias (OR [95% CI] = 1.62 [1.02-2.57], p = .040) and action-related sleep disorders (OR [95% CI] = 1.87 [1.09-3.20], p = .023) were associated with lifetime experience of EHS in a logistic regression analysis. Chronotype did not significantly impact mood, sleep quality or presence of EHS. Results suggest that EHS is more common in young people than previously considered and ought to be examined in conjunction with the presence of other unusual sleep disorders. This study provides valuable insight into young peoples' sleep experiences and key factors associated with EHS.

Encephalopathy related to status epilepticus during sleep due to a de novo KCNA1 variant in the Kv-specific Pro-Val-Pro motif: phenotypic description and remarkable electroclinical response to ACTH.

Although the classic phenotype of episodic ataxia type 1 (EA1) caused by variants in KCNA1 includes episodic ataxia and myokymia, further genotype-phenotype correlations are difficult to establish due to highly heterogeneous clinical presentations associated with KCNA1 pathogenic variants. De novo variants in the paralogous Pro-Val-Pro motif (PVP) of KCNA2, an essential region for channel gating, have been reported to be associated with severe epilepsy phenotypes, including developmental and epileptic encephalopathies (DEE). Here, we describe the first patient with a DEE who developed an encephalopathy related to status epilepticus during sleep (ESES) and cerebellar signs, harbouring a variant in the Kv-specific PVP motif of the KCNA1 gene. Interestingly, he showed a remarkable long-term electroclinical response to IM ACTH therapy. This report extends the range of phenotypes associated with KCNA1 variants to include that of ESES, and suggests that ACTH therapy is likely to have a positive effect in patients with these variants.

Nightmare experience and personality disorder functioning styles in healthy volunteers and nightmare disorder patients.

Nightmares are prevalent in psychiatric disorders, and personality disorder features might be associated with nightmare experience, especially in nightmare disorder patients. The authors invited 219 healthy volunteers and 118 nightmare disorder patients to undergo tests of the Nightmare Experience Questionnaire (NEQ), the Parker Personality Measure (PERM), and the Plutchik-van Praag Depression Inventory. Compared to healthy volunteers, nightmare disorder patients scored significantly higher on annual nightmare frequency and NEQ Physical Effect, Negative Emotion, Meaning Interpretation, and Horrible Stimulation, and higher on PERM Paranoid, Schizotypal, Borderline, Histrionic, Narcissistic, Avoidant, and Dependent styles. Borderline, Schizotypal, and Passive-Aggressive styles in healthy volunteers and Dependent, Avoidant, Histrionic, and Paranoid in patients were significant predictors of some NEQ scales. Higher annual nightmare frequency, higher scale scores of nightmare experience and personality disorder styles, and more associations between the two were found in nightmare disorder patients, implying the need for personality-adjustment therapy for nightmare disorder.

Seizures with paroxysmal arousals in sleep-related hypermotor epilepsy (SHE): Dissecting epilepsy from NREM parasomnias.

OBJECTIVE: Sleep-related hypermotor epilepsy (SHE) is a focal epilepsy characterized by seizures occurring mostly during sleep, ranging from brief seizures with paroxysmal arousals (SPAs) to hyperkinetic seizures and ambulatory behaviors. SPAs are brief and stereotypic seizures representing the beginning of a major seizure. Distinguishing SPAs from disorders of arousal (DOAs) and their briefest episodes called simple arousal movements (SAMs) is difficult. We performed a characterization of SPAs and SAMs to identify video-polysomnographic (VPSG) features that can contribute to the diagnosis of SHE or DOA. METHODS: Fifteen SHE, 30 DOA adult patients, and 15 healthy subjects underwent full-night VPSG. Two neurologist experts in sleep disorders and epilepsy classified all the sleep-related movements and episodes recorded. For each SPAs and SAMs, sleep stage at onset, duration, limb involvement, progression, and semiology have been identified. RESULTS: A total of 121 SPAs were recorded, emerging mostly during stage 1-2 non-rapid eye movement (NREM) sleep (median duration: 5 seconds). At the beginning, the SPAs motor pattern was hyperkinetic in 78 cases (64%), involving more than three non-contiguous or all body parts. The standard was a constant progression of movements during SPAs without any motor arrests. In DOA patients a total of 140 SAMs were recorded (median duration: 12 seconds) mostly emerging during stage 3 NREM sleep. In SAMs, we did not observe any tonic/dystonic or hypermotor patterns or stereotypy; motor arrest was present over the course of about half of the episodes. In comparison with both DOA and healthy subjects, SHE patients showed a higher number of sleep-related movements per night and a reduction of sleep efficiency. SIGNIFICANCE: SPAs and SAMs present different semiological and clinical features. Their recognition could be useful to drive the diagnosis when major episodes are not recorded during VPSG in patients with a clear clinical history of SHE or DOA.

Parasomnias Occurring in Non-Rapid Eye Movement Sleep.

PURPOSE OF REVIEW: This article discusses the clinical manifestations, diagnosis and differential diagnosis, pathophysiology, and management of parasomnias occurring in non-rapid eye movement (REM) sleep. RECENT FINDINGS: Disorders of arousal are characterized by dissociated sleep, with wake and sleep phenomena intermingling, and local sleep, in which different areas of the brain exist simultaneously in different states of wakefulness or sleep. The frequency of arousals from slow-wave sleep with delta or mixed-frequency activity has a high sensitivity but relatively low specificity for the diagnosis of arousal parasomnias. SUMMARY: Disorders of arousal (sleepwalking, sleep terrors, and confusional arousals) are characterized by incomplete awakenings from slow-wave sleep, limited recall of imagery, and partial or complete amnesia. They occur most frequently in childhood. Management includes correction of precipitating factors, attention to safety, behavioral techniques, and medications. Sleep-related eating disorder is a variant of arousal disorders and may be associated with the use of short-acting hypnotics and restless legs syndrome. Complex nocturnal visual hallucinations can occur with visual loss, dementia with Lewy bodies, use of ß-adrenergic receptor antagonists, and anxiety. Exploding head syndrome occurs at wake-sleep transition or on waking during the night, is usually benign, and requires treatment only if significant sleep disruption occurs.

Sleep-Wake Disorders in Childhood.

PURPOSE OF REVIEW: The presentation of sleep issues in childhood differs from the presentation in adulthood and may be more subtle. Sleep issues may affect children differently than adults, and distinct treatment approaches are often used in children. RECENT FINDINGS: Sodium oxybate was approved by the US Food and Drug Administration (FDA) in October 2018 for an expanded indication of treatment of sleepiness or cataplexy in patients with narcolepsy type 1 or narcolepsy type 2 aged 7 years or older, with side effect and safety profiles similar to those seen in adults. Restless sleep disorder is a recently proposed entity in which restless sleep, daytime sleepiness, and often iron deficiency are observed, but children do not meet the criteria for restless legs syndrome or periodic limb movement disorder. SUMMARY: Children's sleep is discussed in this article, including normal sleep patterns and effects of insufficient sleep. Sleep disorders of childhood are reviewed, including insomnia, obstructive sleep apnea, restless legs syndrome, parasomnias, narcolepsy, and Kleine-Levin syndrome. Children with neurologic issues or neurodevelopmental disorders frequently have sleep disorders arising from an interaction of heterogeneous factors. Further attention to sleep may often be warranted through a polysomnogram or referral to a pediatric sleep specialist. Sleep disorders may cause indelible effects on children's cognitive functioning, general health, and well-being, and awareness of sleep disorders is imperative for neurologists who treat children.

Association of eszopiclone, zaleplon, or zolpidem with complex sleep behaviors resulting in serious injuries, including death.

PURPOSE: To identify and analyze postmarketing cases of complex sleep behaviors (CSBs) resulting in serious injuries, including death, associated with eszopiclone, zaleplon, or zolpidem (Z-drugs). METHODS: Retrospective analysis of the U.S. Food and Drug Administration (FDA) Adverse Event Reporting System (FAERS) from 16 December 1992 through 27 February 2018 and medical literature using PubMed and EMBASE. We used random sampling and descriptive statistics. RESULTS: We identified 66 cases that met inclusion and exclusion criteria, four of which were identified in the medical literature. Twenty cases reported death and 46 cases reported serious injuries in association with CSBs occurring after the use of a Z-drug. Fatal cases described events, such as carbon monoxide poisoning, drowning, falls, hypothermia, motor vehicle collisions, and apparent completed suicide. Nonfatal cases resulting in serious injuries described events, such as accidental overdoses, falls, gunshot wounds, hypothermia, third-degree burns, and self-injuries or suicide attempts. Twenty-two cases reported a previous episode of a CSB while taking a Z-drug prior to the event reported in this case series. CONCLUSIONS: The FAERS and medical literature cases support the need for increased awareness of the consequences that may occur because of CSBs associated with the use of Z-drugs. Therefore, to protect public health, regulatory actions were taken, including adding a Boxed Warning, a Contraindication in patients who have experienced a prior episode of a CSB with a Z-drug, and updating the existing Warnings and Precautions. An FDA Drug Safety Communication was also disseminated to alert healthcare professionals and the public of this potential risk.

Co-activation of rhythms during alpha band oscillations as an interictal biomarker of exploding head syndrome.

BACKGROUND: Exploding head syndrome is a rarely reported benign sensory parasomnia that may nonetheless have significant impact on patients' quality of life and their perceived well-being. To date, the mechanisms underlying attacks, characterised by a painless perception of abrupt, loud noises at transitional sleep-wake or wake-sleep states, are by and large unclear. METHODS AND RESULTS: In order to address the current gap in the knowledge of potential underlying pathophysiology, a retrospective case-control study of polysomnographic recordings of patients presenting to a tertiary sleep disorders clinic with exploding head syndrome was conducted. Interictal (non-attack associated) electroencephalographic biomarkers were investigated by performing macrostructural and event-related dynamic spectral analyses of the whole-night EEG. In patients with exploding head syndrome, additional oscillatory activity was recorded during wakefulness and at sleep/wake periods. This activity differed in its frequency, topography and source from the alpha rhythm that it accompanied. CONCLUSION: Based on these preliminary findings, we hypothesise that at times of sleep-wake transition in patients with exploding head syndrome, aberrant attentional processing may lead to amplification and modulation of external sensory stimuli.

Mastering nocturnal jigsaws in Parkinson's disease: a dusk-to-dawn review of night-time symptoms.

Finding out about night-time symptoms from Parkinson's disease (PD) patients can be a challenge as many patients and their carers cannot recall many symptoms that occur during the night, resulting in an under-recognition or a large variability of responses from clinical interviews and scales. Moreover, technology-based assessments for most night-time symptoms are still not universally available for use in a patient's home environment. Therefore, most physicians rely on their clinical acumen to capture these night-time symptoms based on pieces of patients' history, bedpartner's reports, clinical features, associated symptoms or conditions. To capture more night-time symptoms, the authors identified common nocturnal symptoms based on how they manifest from dusk to dawn with selected features relevant to PD. While some symptoms occur in healthy individuals, in PD patients, they may impact differently. The authors intend this narrative review to provide a practical guide on how these common night-time symptoms manifest and highlight pertinent issues by focusing on prevalence, clinical symptomatology, and specific relationships to PD. It is also important to recognise that PD-specific sleep disturbances increase with advancing disease with additional contributions from ageing, comorbidities, and medication side effects. However, the relative contribution of each factor to individual symptom may be different in individual patient, necessitating clinical expertise for individual interpretation. While there are debatable issues in certain areas, they underlie the complexity of night-time symptoms. Understanding night-time symptoms in PD is like re-arranging jigsaw pieces of clinical information to create, but never complete, a picture for physicians to instigate appropriate management.

A 3-year observation of excessive daytime sleepiness after subthalamic deep brain stimulation in patients with Parkinson's disease.

OBJECTIVE: Subthalamic nucleus deep brain stimulation (STN DBS) has a positive effect on sleep quality, but its effect on wake functions is controversial. This study evaluated the longitudinal changes of the quality of sleep and excessive daytime sleepiness (EDS) in Parkinson's disease (PD) patients undergoing STN DBS and identify which factors are associated with the presence of EDS before and after STN DBS. PATIENT AND METHODS: A total of 33 PD patients who underwent bilateral STN DBS between July 2011 and October 2015 were recruited. We evaluated subjective sleep quality assessed by Parkinson's Disease Sleep Scale (PDSS) and EDS using Epworth Sleepiness Scale (ESS) preoperatively and 6 months, 1 year, and 3 years postoperatively. RESULTS: There is a significant improvement in PDSS, and a noticeable change occurs immediately after the surgery. After DBS, the number of patients with persistent EDS gradually decreased, but patients with newly developed EDS were added. Baseline ESS score was highly correlated with EDS at 6 months and 1 year postoperatively, and older age of PD onset was highly associated with EDS at 1 year after DBS. At 3 years after DBS, the total PDSS score is a main contributing factor for EDS. There was no significant difference in dopamine agonist dose (agonist LED) and levodopa equivalent daily dose (LEDD) between groups with and without EDS at any time points. CONCLUSION: Bilateral STN DBS improves the subjective sleep quality, but EDS may improve or worsen. The risk factors for EDS change over time after STN DBS. Interestingly, dopaminergic medication did not affect EDS in DBS-treated PD patients.