Grumpy face during adult sleep: A clue to negative emotion during sleep?

Negative facial expressions and frowns have been studied (albeit more rarely than smiles) in fetus' and neonate' sleep, but they have not been investigated during adult sleep. Video polysomnography (including corrugator muscle electromyography and face-focussed video) was used to study negative facial expressions in sleeping adults, including healthy subjects and patients with/out parasomnia. Frowns were observed during sleep in 89/91 (97.8%) adults during normal (29 healthy subjects) and abnormal sleep (29 patients without parasomnia, 15 patients with disorders of arousal and 18 patients with rapid eye movement [REM] sleep behaviour disorder [RBD]). In healthy subjects, the following events occurred in decreasing frequency: isometric corrugator activations, brief frowns, and then prolonged frowns and raised eyebrows (both similarly rare). Frowns predominated in REM sleep, and had a lower frequency in non-REM sleep. In healthy subjects, frowns were elementary and not associated with other face movements to the point of composing negative expressions. In contrast, frowns were occasionally associated with overt negative facial expressions in REM sleep in patients with RBD and a young patient with night terrors. They included mostly painful expressions and rarely sadness and anger in connection with apparently negative behaviours (shouts, painful moaning, and speeches). Frowns persist during normal sleep (mostly in REM sleep) in adults, but overt negative facial expressions are restricted to patients with parasomnia. Whether elementary frowns translate a negative dream emotion should be determined, but overt negative facial expressions during RBD could be used as a direct access to dream emotions.

Narcolepsy and psychosis: A systematic review.

OBJECTIVE: Narcolepsy is a rare sleep disorder in which psychotic-like symptoms can present diagnostic and therapeutic challenges. We aimed to review the association between, and medical management of, narcolepsy and psychosis in children and adults. METHODS: We reviewed the full text of 100 papers from 187 identified by a PubMed search on narcolepsy plus any of these keywords: psychosis, schizophrenia, delusion, side effects, safety, and bipolar disorder. RESULTS: Three relevant groups are described. (i) In typical narcolepsy, psychotic-like symptoms include predominantly visual hallucinations at the sleep-wake transition (experienced as "not real") and dissociation because of intrusion of rapid eye movement (REM) sleep phenomena into wakefulness. (ii) Atypical patients ("the psychotic form of narcolepsy") experience more severe and vivid, apparently REM-related hallucinations or dream/reality confusions, which patients may rationalize in a delusion-like way. (iii) Some patients have a comorbid schizophrenia spectrum disorder with psychotic symptoms unrelated to sleep. Psychostimulants used to treat narcolepsy may trigger psychotic symptoms in all three groups. We analyzed 58 published cases from groups 2 and 3 (n = 17 and 41). Features that were reported significantly more frequently in atypical patients include visual and multimodal hallucinations, sexual and mystical delusions, and false memories. Dual diagnosis patients had more disorganized symptoms and earlier onset of narcolepsy. CONCLUSION: Epidemiological studies tentatively suggest a possible association between narcolepsy and schizophrenia only for very early-onset cases, which could be related to the partially overlapping neurodevelopmental changes observed in these disorders. We propose a clinical algorithm for the management of cases with psychotic-like or psychotic features.

Smiling asleep: A study of happy emotional expressions during adult sleep.

Human foetuses and newborns smile first during sleep, before they smile while awake and interacting with caregivers. Whether smiling persists during adult sleep, and expresses inner joy, is yet unknown. Smiles were looked for during night-time video-polysomnography combined with electromyography of the zygomatic and orbicularis oculi muscles in 100 controls, 22 patients with sleepwalking and 52 patients with rapid eye movement (REM) sleep behaviour disorder. Autonomous reactions (heart rate and level of vasoconstriction) and the presence of rapid eye movements were examined during smiles and laughs. On visual examination of the face video clips synchronous with zygomatic contraction, 8% of controls smiled while asleep (7% in REM sleep and 1% in non-REM sleep). Some patients with sleepwalking also smiled and laughed during N2 sleep and N3 parasomnia. Half of the patients with REM sleep behaviour disorder smiled and one-third laughed, mostly during REM sleep. The 173 happy faces included mild smiles (24.8%), open-mouth smiles (29.5%) and laughs (45.7%). More than half of the smiles were the Duchenne (genuine) type, including an active closure of the eyelids. Approximately half of the smiles and laughs were temporally associated with rapid eye movements. There was no increased heart rate variability during smiles and laughs. Two scenic behaviours including smiles and laughs suggested that the happy facial expression was associated with a happy dreaming scenario. Smiling and laughing occasionally persist during adult sleep. There are several lines of evidence suggesting that these happy emotional expressions reflect a true inner mirth.

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.

"I know when to stop": Sentinels and counter-strategies against drowsy driving in narcolepsy and idiopathic hypersomnia.

STUDY OBJECTIVES: To collect prodromal symptoms experienced by participants with narcolepsy and idiopathic hypersomnia (considered "hypersomnolence experts") prior to drowsy driving and counter-strategies used to maintain alertness. METHODS: Systematic, face-to-face interview (using a semi-structured questionnaire), including clinical measures, frequency of car accidents/near misses, and symptoms experienced before impending drowsy driving episodes and counter-strategies. RESULTS: Among 61 participants (32 with narcolepsy, 29 with idiopathic hypersomnia; 56 drivers), 61% of drivers had at least one lifetime accident/near miss. They had a higher sleepiness score (14 ± 4 vs. 11 ± 5, P<0.04) than those without an accident/near miss, but no other differences in demographics, driving experience, medical conditions, symptoms, sleep tests, and treatment. All but three participants experienced prodromal symptoms of drowsy driving, which included postural and motor changes (86.9%: axial hypotonia - e.g., eyelid droop, stereotyped movements), cognitive impairment (53.3%: automatic steering, difficulty concentrating/shifting, dissociation, mind wandering, dreaming), sensory (65%: paresthesia, pain, stiffness, heaviness, blunted perceptions such as a flat dashboard with loss of 3D, illusions and hallucinations), and autonomic symptoms (10%, altered heart/breath rate, penile erection). Counterstrategies included self-stimulation from external sources (pain, cold air, music, drinks, driving with bare feet), motor changes (upright posture, movements), and surprise (sudden braking). CONCLUSIONS: Drowsy driving symptoms can result from "local" NREM, entry in N1 sleep, and hybrid wake/REM sleep states. These rich qualitative insights from participants with narcolepsy and idiopathic hypersomnia, as well as sophisticated counter-strategies, can be gathered to reduce the crash risk in this population, but also in inexperienced healthy drivers.

Latency to N3 interruption in arousal disorders.

STUDY OBJECTIVES: To help expert witnesses in criminal cases using the "sleepwalking defense," we studied the time of first and last interruptions from stage N3 in patients with arousal disorders, including sexsomnia, as well as their determinants. METHODS: The epochs of lights off, sleep onset, first N3 interruption (with and without behaviors), and last N3 interruption were determined by videopolysomnography on two consecutive nights in 163 adults with disorders of arousal, including 46 with and 117 without sexsomnia. RESULTS: The first N3 interruption (independently of concomitant behavior) occurred as early as 8 minutes after sleep onset and within 100 minutes of falling asleep in 95% of cases. The first motor arousal from N3 occurred as early as 25 minutes after lights off time, a timing more variable between participants (between 30 and 60 minutes after lights off time in 25% of participants and within 60 minutes of falling asleep in 50%). These latencies did not differ between the groups with and without sexsomnia. No correlation was found between these latencies and the young age, sex, or clinical severity. The latency of motor arousals was shorter when they were associated with a fast-wave EEG profile and were not preceded by another type of N3 arousal. CONCLUSIONS: The first motor arousal may occur early in the night in patients with arousal disorders, with or without sexsomnia, suggesting that abnormal behaviors occurring as early as 25 minutes after lights off time in clinical and criminal cases can be a parasomnia manifestation.

The dynamics of emotional behaviors in rapid eye movement sleep.

Dream's emotions could exert a major role in desensitizing negative emotions. Studying emotional dynamics (how emotions fluctuate across time) during rapid eye movement (REM) sleep could provide some insight into this function. However, studies so far have been limited to dream reports. To bypass this limit, REM sleep behavior disorder (RBD), in which participants enact their dreams, enables direct access to overt emotional dream behaviors (such as facial expressions and speeches). In total, 17 participants with RBD, and 39.7 h of REM sleep video were analyzed. The frequency of emotional behaviors did not differ between REM sleep episodes of early and late night. Within individual REM sleep episodes, emotional behaviors exhibited a biphasic temporal course, including an increased frequency for the first 10 min, followed by a progressive decrease. The negative emotional behaviors occurred earlier (mean time: 11.3 ± 10 min) than positive (14.4 ± 10.7 min) and neutral behaviors (16.4 ± 11.8 min). Emotional behaviors of opposing (negative and positive) valences were observed in 31% (N = 14) of episodes containing at least one emotional behavior, and were separated by a median time of 4.2 [1.1-10.9] min. The biphasic temporal course of behaviors in REM sleep could include the generation reactivation of emotional content during the ascending phase, followed by processing and extinction during the descending phase. The earlier occurrence time of negative emotional behavior suggests that negative emotions may need to be processed first. The rapid succession of emotions of opposite valence could prevent prolonged periods of negative emotions and eventually nightmares.

Hypersomnia and narcolepsy in 42 adult patients with craniopharyngioma.

STUDY OBJECTIVES: To evaluate sleep, sleepiness, and excessive need for sleep in patients with craniopharyngioma (a suprasellar tumor which can affect sleep-wake systems). METHODS: A retrospective study of all adult patients living with craniopharyngioma referred to the sleep clinic, who received a sleep interview, nocturnal polysomnography, multiple sleep latency tests (MSLT), and 18-h bed rest polysomnography. Their sleep measurements were compared with those of age- and sex-matched healthy controls. RESULTS: Of 54 patients screened with craniopharyngioma, 42 were analyzed, 80% of whom complained of excessive daytime sleepiness. Sleep testing revealed that 6 (14.3%) of them had secondary narcolepsy (including one with cataplexy), and 11 (26.2%) had central hypersomnia associated with a medical disorder. Compared with controls, patients were more frequently obese, had a shorter mean sleep latency on MSLT, and slept longer on the first night. There was a nonsignificant trend for patients with (vs. without) narcolepsy and hypersomnia to be younger, to have a higher body mass index, to be more likely to have received radiation therapy, and to have more severe damage to the hypothalamus after surgery. Treatment with stimulants (modafinil, pitolisant, and methylphenidate) was beneficial in 9/10 patients. CONCLUSIONS: Nearly half of the patients with craniopharyngioma and sleep disorders have a central disorder of hypersomnolence (narcolepsy and hypersomnia), which should be investigated and lead to considerations beyond sleep apnea syndrome in these obese patients.

Behavioral and brain responses to verbal stimuli reveal transient periods of cognitive integration of the external world during sleep.

Sleep has long been considered as a state of behavioral disconnection from the environment, without reactivity to external stimuli. Here we questioned this 'sleep disconnection' dogma by directly investigating behavioral responsiveness in 49 napping participants (27 with narcolepsy and 22 healthy volunteers) engaged in a lexical decision task. Participants were instructed to frown or smile depending on the stimulus type. We found accurate behavioral responses, visible via contractions of the corrugator or zygomatic muscles, in most sleep stages in both groups (except slow-wave sleep in healthy volunteers). Across sleep stages, responses occurred more frequently when stimuli were presented during high cognitive states than during low cognitive states, as indexed by prestimulus electroencephalography. Our findings suggest that transient windows of reactivity to external stimuli exist during bona fide sleep, even in healthy individuals. Such windows of reactivity could pave the way for real-time communication with sleepers to probe sleep-related mental and cognitive processes.

Movements and behaviors during spontaneous arousals in healthy young adults: an intermediary stage between wakefulness and sleep?

BACKGROUND: Arousals are common, sudden and transient elevations of the vigilance level during normal sleep, but arousal-associated behaviors have not yet been studied. OBJECTIVE: We aimed to describe the duration as well as motor and autonomic patterns associated with arousals across sleep stages in normal subjects. METHODS: The spontaneous arousals of 25 healthy young adults were randomly analyzed on polysomnography with body- and face-oriented video cameras. The duration of the heart rate response as well as the frequency, amplitude, speed, body segment and semiology of associated movements were measured. RESULTS: Among 624 arousals (258 in N2, 140 in N3 and 226 in REM sleep), REM sleep arousals had the shortest duration, and N3 arousals were associated with greater heart rate acceleration. Movements and behaviors (mostly involving the head and neck, then the upper limbs, with rare eyes opening and no turning in bed) were frequent during arousals (69.4% during N2 sleep, 89.3% during N3 and 93.8% during REM sleep). Arousals more frequently included ample, prolonged and whole-body movements during N3 sleep and fast movements and facial expressions during REM sleep. During N2 arousals, chewing was the most prevalent behavior. Some movements resembled orientation and comfort behaviors (flexing/rotating the neck and trunk, scratching, pulling the sheets, rubbing the nose, yawning, smiling, frowning and speaking), whereas others resembled sleep-associated automatisms (swallowing, chewing). CONCLUSION: In contrast with previous assumptions, most arousals are associated with movements. The type of movements suggests that arousal is an intermediary state between wakefulness and sleep.

Eye movement patterns correlate with overt emotional behaviours in rapid eye movement sleep.

Growing evidence suggests that sleep plays a key role in regulating emotions. Rapid eye movements (REMs) in REM sleep could be associated with dreams emotions, but supporting evidence is indirect. To highlight this association, we studied the REM sleep during video-polysomnography of 20 subjects with REM sleep behaviour disorder (RBD), a model of enacted dreams offering direct access to the emotional content of the sleeper (face expression, speeches, behaviour). Video and the electro-oculography recordings were divided into 3 s time intervals and classified as non-behavioural, or behavioural (neutral, positive or negative emotions), and as containing no eye movements (EMs), slow eye movements (SEMs) or REMs (isolated or bursts). Compared to the absence of EMs, neutral behaviours successively increased in the presence of SEMs (odd ratio, OR = 1.4), then isolated REMs (OR = 2.8) and then REM bursts (OR = 4.6). Positive behaviours increased with SEMs (OR = 2.8) but did not increase further with isolated REMs (OR = 2.8) and REM bursts (OR = 3). Negative behaviours were absent with SEMs, increased with isolated REMs (OR = 2.6) and further with REM bursts (OR = 10.1). These results support an association between REMs and SEMs, and dream emotions.

Sleeping through a pandemic: impact of COVID-19-related restrictions on narcolepsy and idiopathic hypersomnia.

STUDY OBJECTIVES: To assess the impact of coronavirus disease 2019 (COVID-19)-related restrictions on narcolepsy type 1 (NT2), narcolepsy type 2 (NT2), and idiopathic hypersomnia (IH). METHODS: Participants with NT1, NT2, and IH followed in a university hospital completed an online 78-question survey assessing demographic, clinical, and occupational features of the population during the first COVID-19-related lockdown. RESULTS: A total of 219 of 851 (25.7%) respondents of the survey reported a mean increase of 1.2 ± 1.9 hours (P < .001) in night sleep time and a mean decrease of 1.0 ± 3.4 points (P < .001) on the Epworth Sleepiness Scale during lockdown. Bedtime was delayed by 46.1% of participants and wakeup time was delayed by 59.6%, driven primarily by participants with IH. Teleworkers (but not in-person workers) reported a mean increase of 0.9 ± 1.2 hours in night sleep (P < .001) and a mean decrease in sleepiness score of 1.6 ± 3.1 (P < .001). Cataplexy improved in 54.1% of participants with NT1. Sleepiness correlated with psychological wellness (r = .3, P < .001). As many as 42.5% enjoyed the lockdown, thanks to reallocation of time usually spent commuting toward longer sleep time, hobbies, and family time, and appreciated a freer napping schedule. Conversely, 13.2% disliked the lockdown, feeling isolation and psychological distress. CONCLUSIONS: Extended sleep time, circadian delay (in patients with IH), and teleworking resulted in decreased symptoms of central hypersomnias. These findings suggest that people with IH, NT1, and NT2 may benefit from a decrease in social and professional constraints on sleep-wake habits, and support advocacy efforts aimed at facilitating workplace and schedule accommodations for this population. CITATION: Nigam M, Hippolyte A, Dodet P, et al. Sleeping through a pandemic: impact of COVID-19-related restrictions on narcolepsy and idiopathic hypersomnia. J Clin Sleep Med. 2022;18(1):255-263.

Comorbid parasomnias in narcolepsy and idiopathic hypersomnia: more REM than NREM parasomnias.

STUDY OBJECTIVES: To assess the frequency, determinants, and clinical impact of clinical rapid eye movement (REM) and non-REM (NREM) parasomnias in adult patients with narcolepsy type 1 (NT1), narcolepsy type 2 (NT2), and idiopathic hypersomnia compared with healthy controls. METHODS: Familial and past and current personal parasomnias were assessed by questionnaire and medical interviews in 710 patients (220 NT1, 199 NT2, and 221 idiopathic hypersomnia) and 595 healthy controls. RESULTS: Except for sleep-related eating disorder, current NREM parasomnias were rare in all patient groups and controls. Sleep-related eating disorder was more frequent in NT1 patients (7.9% vs 1.8% in NT2 patients, 2.1% in patients with idiopathic hypersomnia, and 1% in controls) and associated with disrupted nighttime sleep (odds ratio = 3.9) and nocturnal eating in full awareness (odds ratio = 6.9) but not with sex. Clinical REM sleep behavior disorder was more frequent in NT1 patients (41.4%, half being violent) than in NT2 patients (13.2%) and affected men more often than women (odds ratio = 2.4). It was associated with disrupted nighttime sleep, depressive symptoms, and antidepressant use. Frequent (> 1/week) nightmares were reported by 39% of patients with NT1, 29% with NT2, and 27.8% with idiopathic hypersomnia (vs 8.3% in controls) and were associated with depressive symptoms in narcolepsy. No parasomnia (except sleep-related hallucinations) worsened daytime sleepiness. CONCLUSIONS: In patients with central disorders of hypersomnolence, comorbid NREM parasomnias (except for sleep-related eating disorder) are rare and do not worsen sleepiness. In contrast, REM parasomnias are prevalent (especially in NT1) and are associated with male sex, disrupted nighttime sleep, depressive symptoms, and antidepressant use. CITATION: Leu-Semenescu S, Maranci J-B, Lopez R, et al. Comorbid parasomnias in narcolepsy and idiopathic hypersomnia: more REM than NREM parasomnias. J Clin Sleep Med. 2022;18(5):1355-1364.

Functional brain imaging using 18F-fluorodeoxyglucose positron emission tomography/computerized tomography in 138 patients with Kleine-Levin syndrome: an early marker?

Kleine-Levin syndrome is a rare disorder characterized by relapsing-remitting episodes of severe hypersomnia, cognitive impairment, apathy, derealization and behavioural disturbances. Between episodes, most patients experience normal sleep, mood and behaviour, but they may have some residual abnormalities in brain functional imaging; however, the frequency, localization and significance of abnormal imaging are unknown, as brain functional imaging have been scarce and heterogenous [including scintigraphy 18F-fluorodeoxyglucose positron emission tomography/computerized tomography (FDG-PET/CT) and functional MRI during resting state and cognitive effort] and based on case reports or on group analysis in small groups. Using visual individual analysis of 18F-fluorodeoxyglucose positron emission tomography/computerized tomography at the time of Kleine-Levin syndrome diagnosis, we examined the frequency, localization and clinical determinants of hypo- and hypermetabolism in a cross-sectional study. Among 179 patients with Kleine-Levin syndrome who underwent 18F-fluorodeoxyglucose positron emission tomography/computerized tomography, the visual analysis was restricted to the 138 untreated patients studied during asymptomatic periods. As many as 70% of patients had hypometabolism, mostly affecting the posterior associative cortex and the hippocampus. Hypometabolism was associated with younger age, recent (<3 years) disease course and a higher number of episodes during the preceding year. The hypometabolism was more extensive (from the left temporo-occipital junction to the entire homolateral and then the bilateral posterior associative cortex) at the beginning of the disorder. In contrast, there was hypermetabolism in the prefrontal dorsolateral cortex in half of the patients (almost all having concomitant hypometabolism in the posterior areas), which was also associated with younger age and shorter disease course. The cognitive performances (including episodic memory) were similar in patients with versus without hippocampus hypometabolism. In conclusion, hypometabolism is frequently observed upon individual visual analysis of 18F-fluorodeoxyglucose positron emission tomography/computerized tomography during asymptomatic Kleine-Levin syndrome periods; it is mostly affecting the posterior associative cortex and the hippocampus and is mostly in young patients with recent-onset disease. Hypometabolism provides a trait marker during the first years of Kleine-Levin syndrome, which could help clinicians during the diagnosis process.

Are sleep paralysis and false awakenings different from REM sleep and from lucid REM sleep? A spectral EEG analysis.

STUDY OBJECTIVES: To determine the polysomnography characteristics during sleep paralysis, false awakenings, and lucid dreaming (which are states intermediate to rapid eye movement [REM] sleep and wake but exceptionally observed in sleep laboratory). METHODS: In 5 participants, we captured 5 episodes of sleep paralysis (2 time marked with the ocular left-right-left-right code normally used to signal lucid dreaming, 1 time marked by an external noise, and 2 retrospectively reported) and 2 episodes of false awakening. The sleep coding (using 3-second mini-epochs) and spectral electroencephalography analysis were compared during these episodes and normal REM sleep as well as wakefulness in the same 4 of 5 participants and vs lucid REM sleep in 4 other patients with narcolepsy. RESULTS: During episodes of sleep paralysis, 70.8% of mini-epochs contained theta electroencephalography rhythm (vs 89.7% in REM sleep and 21.2% in wakefulness), 93.8% contained chin muscle atonia (vs 89.7% in REM sleep and 33.3% in wakefulness), and 6.9% contained rapid eye movements (vs 11.9% in REM sleep and 8.1% in wakefulness). The electroencephalography spectrum during sleep paralysis was intermediate between wakefulness and REM sleep in the alpha, theta, and delta frequencies, whereas the beta frequencies were not different between sleep paralysis and normal REM sleep. The power spectrum during false awakening followed the same profile as in sleep paralysis. CONCLUSIONS: The predominant theta electroencephalography rhythm during sleep paralysis and false awakenings (with rare and lower alpha rhythm) suggests that the brain during sleep paralysis is not in an awake but in a dreaming state.

Real-time dialogue between experimenters and dreamers during REM sleep.

Dreams take us to a different reality, a hallucinatory world that feels as real as any waking experience. These often-bizarre episodes are emblematic of human sleep but have yet to be adequately explained. Retrospective dream reports are subject to distortion and forgetting, presenting a fundamental challenge for neuroscientific studies of dreaming. Here we show that individuals who are asleep and in the midst of a lucid dream (aware of the fact that they are currently dreaming) can perceive questions from an experimenter and provide answers using electrophysiological signals. We implemented our procedures for two-way communication during polysomnographically verified rapid-eye-movement (REM) sleep in 36 individuals. Some had minimal prior experience with lucid dreaming, others were frequent lucid dreamers, and one was a patient with narcolepsy who had frequent lucid dreams. During REM sleep, these individuals exhibited various capabilities, including performing veridical perceptual analysis of novel information, maintaining information in working memory, computing simple answers, and expressing volitional replies. Their responses included distinctive eye movements and selective facial muscle contractions, constituting correctly answered questions on 29 occasions across 6 of the individuals tested. These repeated observations of interactive dreaming, documented by four independent laboratory groups, demonstrate that phenomenological and cognitive characteristics of dreaming can be interrogated in real time. This relatively unexplored communication channel can enable a variety of practical applications and a new strategy for the empirical exploration of dreams.

Sleep in ADCY5-Related Dyskinesia: Prolonged Awakenings Caused by Abnormal Movements.

STUDY OBJECTIVES: ADCY5 mutations cause early-onset hyperkinetic movement disorders comprising diurnal and nocturnal paroxysmal dyskinesia, and patient-reported sleep fragmentation. We aimed to characterize all movements occurring during sleep and in the transition from sleep to awakening, to ascertain if there is a primary sleep disorder, or if the sleep disturbance is rather a consequence of the dyskinesia. METHODS: Using video polysomnography, we evaluated the nocturnal motor events and abnormal movements in 7 patients with ADCY5-related dyskinesia and compared their sleep measures with those of 14 age- and sex-matched healthy controls. RESULTS: We observed an increased occurrence of abnormal movements during wake periods compared to sleep in patients with ADCY5-related dyskinesia. While asleep, abnormal movements occurred more frequently during stage N2 and REM sleep, in contrast with stage N3 sleep. Abnormal movements were also more frequent during morning awakenings compared to wake periods before falling asleep. The pattern of the nocturnal abnormal movements mirrored those observed during waking hours. Compared to controls, patients with ADCY5-related dyskinesia had lower sleep efficiencies due to prolonged awakenings secondary to the abnormal movements, but no other differences in sleep measures. Notably, sleep onset latency was short and devoid of violent abnormal movements. CONCLUSIONS: In this series of patients with ADCY5-related dyskinesia, nocturnal paroxysmal dyskinesia were not associated with drowsiness or delayed sleep onset, but emerged during nighttime awakenings with subsequent delayed sleep, whereas sleep architecture was normal.

Sexsomnia: A Specialized Non-REM Parasomnia?

Introduction: To describe patients with sexsomnia and to contrast their clinical and sleep measures with those of healthy controls and sleepwalkers. Aims and Methods: Subjects referred for sexsomnia and for sleepwalking/night terror were interviewed, completed the Paris Arousal Disorder Severity Scale (PADSS), and were monitored 1-2 nights with video-polysomnography. Results: Seventeen patients (70.6% male, aged 17-76 years) had sexsomnia, with amnestic fondling of the bed partner (n = 11), complete sexual intercourse (n = 8), masturbation (n = 8), and spontaneous orgasm (n = 1). The sexual behaviors were more direct during sleep than during wakefulness (n = 12), leading to 6 sexual assaults, including intra-conjugal rape (n = 3), assault of a family member (n = 2), rape of a friend (n = 1), and forensic consequences (n = 2). In 47% of sexsomnia patients, there was a history or current occurrences of sleepwalking/night terrors. Patients with sexsomnia had more N3 awakenings than healthy matched controls and the same amount as regular sleepwalkers. Half of them presented evidence of cortico-cortical dissociation, including concomitant slow (mostly frontal) and rapid (mostly temporal and occipital) electroencephalography (EEG) rhythms, with concomitant N3 penile erection in 1 case. Of 89 sleepwalkers, 10% had previous episodes of amnestic sexual behaviors, with a higher PADSS-A score and a trend of a higher total PADSS score than the 80 sleepwalkers without sexsomnia. Conclusion: In this single-center series, we confirmed the male predominance of sexsomnias and its potential for severe clinical and forensic consequences. The results suggest a continuum of regular sleepwalking, sleepwalking with occasional sexsomnia, and quasi-exclusive sexsomnia.