Development of a Composite Score for the Clinical Assessment of Anti-IgLON5 Disease.

BACKGROUND AND OBJECTIVES: To develop a composite score to assess the severity of the multiple symptoms present in anti-IgLON5 disease. METHODS: The anti-IgLON5 disease composite score (ICS) was designed to evaluate 17 symptoms divided into 5 clinical domains (bulbar, sleep, movement disorders, cognition, and others). Each symptom was scored from 0 (absent/normal) to 3 or 6 (severe) depending on the contribution of the symptom to neurologic disability with a maximum ICS of 69. The ICS was tested in patients from 2 cohorts (Barcelona, Spain, and GENERATE, Germany) that included cases personally seen by the authors (internal) and patients whose ICS was obtained from information of questionnaires completed by the referring neurologists (external). Test-retest and interrater reliabilities of the ICS were assessed by the intraclass coefficient (ICC) and the correlation between the ICS and modified Rankin scale (mRS) with the nonparametric Spearman rank coefficient. The Wilcoxon signed rank test was used to compare the ICS at diagnosis of anti-IgLON5 disease and follow-up in a subset of patients with available clinical information. RESULTS: A total of 86 patients (46 from Barcelona cohort; 40 from GENERATE cohort) were included. The median ICS was 15 (range 2-31). The ICS was higher in the Barcelona cohort than in the German cohort (18 vs 12, p < 0.001), due to higher partial scores in sleep and movement disorder domains. There were no significant differences in the ICS between internal and external patients (15 vs 14, p = 0.96). The ICS correlated with the mRS score (r = 0.429, p < 0.001). Test-retest and interrater reliabilities were excellent with an ICC of 0.997 (95% CI 0.992-0.999) and 0.973 (95% CI 0.925-0.990), respectively. ICS was retested during follow-up in 27 patients, and it was similar to that at diagnosis in 10 clinically stable patients (median ICS at diagnosis 11.5 vs 11.5 at follow-up; p = 1), higher in 8 patients who worsened (12.5 vs 18; p = 0.012), and lower in 9 patients who improved after immunotherapy (14 vs 10; p = 0.007). DISCUSSION: The ICS is a valid method to assess the extension and severity of the different clinical manifestations of anti-IgLON5 disease.

A population-based study exploring association of parasomnia symptoms with sleep onset delay among school-aged children.

OBJECTIVE: Parasomnia is potentially implicated in sleep pattern and sleep architecture, however, evidence is quite limited. This study aimed to investigate the association between parasomnia symptoms and sleep onset delay among children through a large epidemiological study. METHODS: Two rounds of cross-sectional studies were conducted among 21,704 children aged 3-11; one taking place in Shanghai and the other in Sanya, Hainan province. Children's sleep characteristics were evaluated using the Children's Sleep Habits Questionnaire (CSHQ). Propensity score matching was adopted to balance the difference of covariates, and the logistic regression models were implemented to examine the associations between parasomnia symptoms and sleep onset delay. RESULTS: A total of 38.2 % of children had sleep onset delay. Parasomnias, especially non rapid eye movement (NREM) and rapid eye movement (REM) parasomnia symptoms, were associated with an increased risk of sleep onset delay (Sleep Walking: OR = 1.55; Sleep Terror: OR = 1.34; Nightmare: OR = 1.37, all p˂0.001). The similar findings were observed in stratified analyses according to sleep duration, and the association was pronounced in sleep sufficiency group (Sleep Walking: OR = 1.62; Sleep Terror: OR = 1.35; Nightmare: OR = 1.35, all p˂0.001). Moreover, a dose-dependent pattern was observed, in which cumulative parasomnia symptoms were associated with increasing risk of sleep onset delay (2 symptoms: OR = 1.19; ≥3 symptoms: OR = 1.40; by comparison with ≤1 symptom). All these findings were also similarly observed in the propensity score matching sample. Moreover, the associations were generally established in both Shanghai and Sanya children. CONCLUSIONS: Parasomnia symptoms were associated with a higher risk of sleep onset delay independently of sleep duration among children. More studies are needed to enrich the current evidence, thus further clarifying the association and interaction among different sleep parameters.

Parasomnias manifest different phenotypes of sleep-related behaviors in age and sex groups. A YouTube-based video research highlighting the age slope of sleepwalking.

STUDY OBJECTIVE: Finding typical patterns - phenotypes - of sleep behaviors characterizing parasomnias in different age and sex groups. METHODS: We analyzed YouTube videos on sleep-related behaviors likely representing parasomnias. We applied the search terms "sleepwalking", "somnambulism", "sleep eating" "sleep sex", "sleep talking" and "aggression in sleep" in six languages. We classified those persons shown on the videos into estimated biological sex and age (child, adult, elderly) groups. We scored the activity types by a self-made scale and applied binary logistic regression to analyze the association between sleep behaviors versus sex and age groups by the STATA package, providing a 95 % confidence interval and the probability of statistical significance. RESULTS: 224 videos (102 women; 68 children, 116 adults, and 40 elderly people) were scored. Elderly people had significantly (P < 0.012) less odds of ambulation in sleep likely consistent with somnambulism compared to adults and children. Adult females performed complex manual activities during sleepwalking more often, than males (P < 0.012). Elderly males had 40-fold odds compared to adults and children, to perform aggressive movements and 70-fold odds of complex movements in bed, compared to adults. Elderly people presented emotional behaviors less frequently than adults (P < 0.004), and females showed them twice as often as males. Adults sleep-talked full sentences more often than children and elderly people (P < 0.001). CONCLUSION: Our results support the existence of age- and sex-specific parasomnia phenotypes, denoting possible safety measures. The remarkably low odds of sleepwalking in the elderly highlight the possibility of different pathomechanisms in higher age groups compared to children.

Insomnia and parasomnia induced by validated smoking cessation pharmacotherapies and electronic cigarettes: a network meta-analysis.

We aim to assess the relationship between validated smoking cessation pharmacotherapies and electronic cigarettes (e-cigarettes) and insomnia and parasomnia using a systematic review and a network meta-analysis. A systematic search was performed until August 2022 in the following databases: PUBMED, COCHRANE, CLINICALTRIAL. Randomized controlled studies against placebo or validated therapeutic smoking cessation methods and e-cigarettes in adult smokers without unstable or psychiatric comorbidity were included. The primary outcome was the presence of "insomnia" and "parasomnia." A total of 1261 studies were selected. Thirty-seven studies were included in the quantitative analysis (34 for insomnia and 23 for parasomnia). The reported interventions were varenicline (23 studies), nicotine replacement therapy (NRT, 10 studies), bupropion (15 studies). No studies on e-cigarettes were included. Bayesian analyses found that insomnia and parasomnia are more frequent with smoking cessation therapies than placebo except for bupropion. Insomnia was less frequent with nicotine substitutes but more frequent with bupropion than the over pharmacotherapies. Parasomnia are less frequent with bupropion but more frequent with varenicline than the over pharmacotherapies. Validated smoking cessation pharmacotherapies can induce sleep disturbances with different degrees of frequency. Our network meta-analysis shows a more favorable profile of nicotine substitutes for insomnia and bupropion for parasomnia. It seems essential to systematize the assessment of sleep disturbances in the initiation of smoking cessation treatment. This could help professionals to personalize the choice of treatment according to sleep parameters of each patient. Considering co-addictions, broadening the populations studied and standardizing the measurement are additional avenues for future research.

Strange episodes during sleep - epilepsy or parasomnia? / Underlige episoder under søvn ­ epilepsi eller parasomni?

In some forms of epilepsy, the seizures occur almost exclusively during sleep. This is particularly the case with hypermotor frontal lobe seizures. Clinically it can be difficult to distinguish such seizures from parasomnias and psychogenic non-epileptic seizures. This clinical review article aims to highlight the importance of making the correct diagnosis, as these conditions require completely different treatment.

Exploding Head Syndrome: A Systematic Scoping Review.

Exploding head syndrome (EHS) has historically been viewed as a disorder predominantly affecting older people and being more common in females. Through a comprehensive review of data since 2005, this scoping review provides updated evidence from 4082 participants reporting EHS across a variety of study designs on: how EHS presents; key information on comorbidity and correlates of EHS; how EHS is experienced in terms of symptoms and beliefs; causal theories arising from the research reviewed; and evidence-based information on how research has reported on the management of EHS. Since 2005, EHS has attracted increasing research interest; however, there are significant gaps in the research that are hindering a better understanding of EHS that might be helpful for clinicians.

The parasomnia defense in sleep-related homicide: A systematic review and a critical analysis of the medical literature.

This review critically analyzes the forensic application of the Parasomnia Defense in homicidal incidents, drawing from medical literature on disorders of arousal (DOA) and rapid-eye-movement sleep behavior disorder (RBD). A systematic search of PubMed, Scopus, Embase, and Cochrane databases was conducted until October 16, 2022. We screened English-language articles in peer-reviewed journals discussing murders committed during sleep with a Parasomnia Defense. We followed PRISMA guidelines, extracting event details, diagnosis methods, factors influencing the acts, perpetrator behavior, timing, motives, concealment, mental experiences, victim demographics, and court verdicts. Three sleep experts evaluated each case. We selected ten homicides, four attempted homicides, and one homicide/attempted homicide that met inclusion/exclusion criteria. Most cases were suspected DOA as unanimously confirmed by experts. RBD cases were absent. Among aggressors, a minority reported dream-like experiences. Victims were primarily female family members killed in or near the bed by hands and/or with sharp objects. Objective sleep data and important crime scene details were often missing. Verdicts were ununiform. Homicides during DOA episodes, though rare, are documented, validating the Parasomnia Defense's use in forensics. RBD-related fatal aggression seems very uncommon. However, cases often lack diagnostic clarity. We propose updated guidelines to enhance future reporting and understanding of such incidents.

The Parasomnias.

Parasomnias usually present in childhood and resolve spontaneously. The diagnosis of non-rapid eye movement-related parasomnias is mainly based on clinical descriptors and can be challenging. Rapid eye movement-related parasomnias may index an underlying psychiatric disorder. Even if benign, parasomnias can affect quality of life. Pediatricians and child psychiatrists should be familiarized with these sleep disorders and suggest adequate sleep hygiene, avoidance of sleep deprivation, and regular bedtimes even on weekends as the first step in management of these disorders. Clinicians should pursue the opportunity for tailoring treatments and consider referral to a sleep expert when indicated.

The Clinical Spectrum of the Parasomnias.

Parasomnias are defined as abnormal movements or behaviors that occur in sleep or during arousals from sleep. Parasomnias vary in frequency from episodic events that arise from incomplete sleep state transition. The framework by which parasomnias are categorized and diagnosed is based on the International Classification of Sleep Disorders-Third Edition, Text Revision (ICSD-3-TR), published by the American Academy of Sleep Medicine. The recent Third Edition, Text Revision (ICSD-3-TR) of the ICSD provides an expert consensus of the diagnostic requirements for sleep disorders, including parasomnias, based on an extensive review of the current literature.

Sleep-Related Dissociative Disorders.

In sleep-related dissociative disorders, phenomena of the psychiatrically defined dissociative disorders emerge during the sleep period. They occur during sustained wakefulness, either in the transition to sleep or following an awakening from sleep. Behaviors during episodes vary widely, and can result in injury to self or others. Daytime dissociative episodes and a background of trauma are almost always present; there is typically major co-existing psychopathology. Diagnosis is based on both clinical history and polysomnography; differential diagnosis primarily involves other parasomnias and nocturnal seizures. Information available about treatment is limited; in a few reported cases, psychological interventions have proven effective.

Parasomnias During the COVID-19 Pandemic.

COVID-19 had a massive impact on sleep, resulting in overall increase of sleep disturbances. During lockdown many factors contributed to sleep disturbances, in particular changes in sleep-wake habits and stress. This article will describe the frequency and features of the principal parasomnias and the impact of the pandemic and the government restriction measures on sleep. Among different pathophysiological hypotheses, we will discuss the role of stress, considered as an expression of the allostatic load. Finally, during the pandemic, parasomnias were mainly investigated by questionnaires, with controversial results; video-polysomnographic studies are crucial to obtain a definitive diagnosis, even in critical conditions.

Educational Resources to Support Patients with Parasomnias.

This article serves to help reduce patient burden in searching for credible information about parasomnias-abnormal behaviors during sleep-including sleepwalking, night terrors, and rapid eye movement sleep behavior disorder. It exhibits a compiled list of accessible online resources about parasomnias as well as detailed descriptions about each resource. By increasing patient accessibility to clinically validated resources, patients are more empowered to take an active role in managing their conditions, collaborating with their health-care practitioners in clinical management, enrolling in registries, and joining newsletters sponsored by these resources.

Understanding Sexual Parasomnias: A Review of the Current Literature on Their Nature, Diagnosis, Impacts, and Management.

Sexual behavior during sleep, known as sexual parasomnias, has captured the interest of researchers and clinicians. These parasomnias involve various sexual activities that occur unconsciously during sleep. Although relatively rare, they can profoundly affect well-being and relationships and can carry legal consequences. Understanding their nature, prevalence, and causes is crucial for advancing knowledge in this field. This article revisits the topic of sexsomnia, presenting new data and discussing cases published from 2007 to 2023. By analyzing these cases, we aim to enhance recognition, diagnosis, and management of sexsomnia, reducing stigma and providing better support for affected individuals.

Somnambulism.

Somnambulism, also called sleepwalking, classified as a non-rapid eye movement sleep parasomnia, encompasses a range of abnormal paroxysmal behaviors, leading to sleepwalking in dissociated sleep in an altered state of consciousness with impaired judgment and configuring a kind of hierarchical continuum with confusional arousal and night terror. Despite being generally regarded as a benign condition, its potential severity entails social, personal, and even forensic consequences. This comprehensive review provides an overview on the current state of knowledge, elucidating the phenomenon of somnambulism and encompassing its clinical manifestations and diagnostic approaches.

Forensic Implications of the Parasomnias.

Although many sleep-related behaviors are benign, others can result in physical or sexual aggression toward bed partners or others. Individuals who engage in sleep-related violence (SRV) and sexual behavior in sleep (SBS) may face legal sanctions for their behavior. Attorneys or legal decision-makers may call on an expert to evaluate a defendant and opine about the veracity of an alleged parasomnia diagnosis, the criminal responsibility of the defendant, and his risk of violence to others. This article reviews the phenomena of SRV and SBS and guides evaluators in the forensic considerations relevant to parasomnias.

Sleep Terrors.

Sleep terrors, categorized under disorders of arousal, more prevalent in pediatric population, generally are self-limited but sometimes can persist or occur in adulthood. These are primed by factors enhancing homeostatic drive on backdrop of developmental predisposition and are precipitated by factors increasing sleep fragmentation resulting in dissociated state of sleep with some cerebral regions showing abnormal slow wave activity and others fast activity. This phenotypically evolves into abrupt partial arousal with individual arousing from N3 or N2 sleep with behaviors representing intense fear such as crying with autonomic hyperactivity. There is no recollection of the event, and lack of vivid dream mentation although fragmented imagery may be noted. Behavioral management is of prime importance including addressing precipitating factors, family reassurance, safety measures, and scheduled awakenings. Pharmacologic agents such as clonazepam and antidepressants are used infrequently in case of disruptive episodes.

Sleep-Related Eating Disorder.

Sleep-related eating disorder is a non-rapid-eye movement parasomnia typified by recurrent episodes of eating/drinking following arousals, with associated partial/complete amnesia. Adverse health consequences and quality of life impairments are common. The condition can be idiopathic but most often accompanies unrecognized/untreated comorbid sleep disorders and/or is induced by psychoactive medications. As such, management consists of addressing comorbidities and removing potentially offending medications. While a thorough clinical history is often sufficient, additional sleep testing may help identify coexisting sleep disorders and/or other phenomena that may cause arousals. Limited data suggest benefit from topiramate and other medications in idiopathic or otherwise refractory cases.