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To investigate clinical and video-polysomnography (VPSG) findings of hallucinatory experiences in patients suffering from disorders of arousal (DOA) in the absence of other pathologies.The authors retrospectively reviewed the records of 370 adults with DOA. Thirty (8.1%) patients concomitantly reported complex nocturnal visual hallucinations. VPSG recordings were scrutinized, and motor behavioral and electroencephalogram (EEG) patterns were classified according to previous descriptions of DOA.Thirty DOA patients reported seeing images of objects, people, and animals; either distorted, static, or mobile. The images disappeared with increased illumination in 80% of patients, and 23.3% reported preceding dream imagery. In addition to the classical DOA patterns on VPSG, a distinct pattern of behavioral and EEG manifestation associated with complex hallucinatory episodes was identified in 16 (53.3%) DOA patients. This consisted of low-voltage mixed-frequency EEG activity before eye opening that persisted while patients were observed staring or visually tracking before the onset of motor behavior.A novel, distinct behavioral and EEG pattern in patients with DOA and history of reported complex nocturnal visual hallucinations was identified. This may represent a unique phenotype of dissociation between sleep states that merits further investigation.

Fatal insomnia (FI) is a rare prion disease severely affecting sleep architecture. Breathing during sleep has not been systematically assessed. Our aim was to characterize the sleep architecture, respiratory patterns, and neuropathologic findings in FI.Eleven consecutive FI patients (ten familial, one sporadic) were examined with video-polysomnography (vPSG) between 2002 and 2017. Wake/sleep stages and respiration were evaluated using a modified scoring system. Postmortem neuropathology was assessed in seven patients.Median age at onset was 48 years and survival after vPSG was 1 year. All patients had different combinations of breathing disturbances including increased respiratory rate variability (RRV; n = 7), stridor (n = 9), central sleep apnea (CSA) (n = 5), hiccup (n = 6), catathrenia (n = 7), and other expiratory sounds (n = 10). RRV in NREM sleep correlated with ambiguous and solitary nuclei degeneration (r = 0.9, p = 0.008) and reduced survival (r = -0.7, p = 0.037). Two new stages, Subwake1 and Subwake2, present in all patients, were characterized. NREM sleep (conventional or undifferentiated) was identifiable in ten patients but reduced in duration in eight. REM sleep occurred in short segments in nine patients, and their reduced duration correlated with medullary raphe nuclei degeneration (r = -0.9, p = 0.005). Seven patients had REM without atonia. Three vPSG patterns were identified: agitated, with aperiodic, manipulative, and finalistic movements (n = 4); quiet-apneic, with CSA (n = 4); and quiet-non-apneic (n = 3).FI patients show frequent breathing alterations, associated with respiratory nuclei damage, and, in addition to NREM sleep distortion, have severe impairment of REM sleep, related with raphe nuclei degeneration. Brainstem impairment is crucial in FI.

Parasomnias are undesirable physical events or experiences associated with sleep. Episodes are usually classified according the stage of sleep during or from which they occur. Non-rapid eye movement (NREM) parasomnias occur from deep NREM sleep and often involve complex behaviours that may appear purposeful but are not conscious or under deliberate control. There are a number of different types/presentations of NREM parasomnias. These may range from common manifestations such as sleeptalking, sleepwalking or night terrors, to the rarer sleep-related eating disorder or sexsomnia. Clinical features can facilitate the differential diagnosis, but polysomnography may be needed, particularly to assess potential concomitant sleep disorders that might represent precipitating factors. Medical treatment is often not indicated, but clonazepam or antidepressants may be used in more severe cases. Safety aspects, treating concomitant sleep disorders and avoiding trigger factors are key aspects of management.

Excessive daytime sleepiness (EDS) is a public health issue. However, it remains largely undervalued, scarcely diagnosed, and poorly supported. Variations in the definition of EDS and limitations in clinical assessment lead to difficulties in its epidemiological study, but the relevance of this symptom from a socioeconomic perspective is inarguable. EDS might be a consequence of several behavioural issues leading to insufficient or disrupted sleep, as well as a consequence of sleep disorders including sleep apnoea syndrome, circadian disorders, central hypersomnolence disorders (narcolepsy and idiopathic hypersomnia), other medical or psychiatric conditions, or medications. Furthermore, EDS can have implications for health as it is thought to act as a risk factor for other conditions, such as cardiovascular and neurodegenerative disorders. Because of the heterogeneous causes of EDS and the complexity of its pathophysiology, management will largely depend on the cause, with the final aim of making treatment specific to the individual using precision medicine and personalised medicine.

Background: The COVID-19 pandemic has led to significant changes in daily routines and lifestyle worldwide and mental health issues have emerged as a consequence. We aimed to assess the presence of sleep disturbances during the lockdown in the general population. Methods: Cross-sectional, online survey-based study on adults living through the COVID-19 pandemic. The questionnaire included demographics and specific questions assessing the impact of the pandemic/ lockdown on sleep, daytime functioning and mental health in the general population. Identification of sleep pattern changes and specific sleep-related symptoms was the primary outcome, and secondary outcomes involved identifying sleep disturbances for predefined cohorts (participants reporting impact on mental health, self-isolation, keyworker status, suspected COVID-19 or ongoing COVID-19 symptoms). Results: In total, 843 participants were included in the analysis. The majority were female (67.4%), middle aged [52 years (40–63 years)], white (92.2%) and overweight to obese [BMI 29.4 kg/m2 (24.1–35.5 kg/m2)]; 69.4% reported a change in their sleep pattern, less than half (44.7%) had refreshing sleep, and 45.6% were sleepier than before the lockdown; 33.9% had to self-isolate, 65.2% reported an impact on their mental health and 25.9% were drinking more alcohol during the lockdown. More frequently reported observations specific to sleep were ‘disrupted sleep’ (42.3%), ‘falling asleep unintentionally’ (35.2%), ‘difficulties falling’/‘staying asleep’ (30.9% and 30.8%, respectively) and ‘later bedtimes’ (30.0%). Respondents with suspected COVID-19 had more nightmares and abnormal sleep rhythms. An impact on mental health was strongly associated with sleep-related alterations. Conclusions: Sleep disturbances have affected a substantial proportion of the general population during the COVID-19 pandemic lockdown. These are significantly associated with a self-assessed impact on mental health, but may also be related to suspected COVID-19 status, changes in habits and self-isolation.

Vagus nerve stimulation (VNS) is a neuromodulatory therapeutic option for drug-resistant epilepsy. In randomised controlled trials, VNS implantation has resulted in over 50% reduction in seizure frequency in 26%–40% of patients within 1 year. Long-term uncontrolled studies suggest better responses to VNS over time; however, the assessment of other potential predictive factors has led to contradictory results. Although initially designed for managing focal seizures, its use has been extended to other forms of drug-resistant epilepsy. In this review, we discuss the evidence supporting the use of VNS, its impact on seizure frequency and quality of life, and common adverse effects of this therapy. We also include practical guidance for the approach to and the management of patients with VNS in situ.

BackgroundIdiopathic/isolated REM sleep behaviour disorder (iRBD) is characterised by vivid dreams with ‘acting-out’ behaviours due to REM sleep loss of atonia. It is associated with a risk of Parkinson’s disease (PD) and other synucleinopathies. There is uncertainty about what should be disclosed to iRBD patients in terms of future risk1.MethodsA survey was administered to patients with iRBD and participating in the PREDICT-PD study. They were invited to complete a 9-question survey exploring the information they would like to receive about the potential future implications of having iRBD.ResultsWe interviewed 31 patients (28 males, 70yo, SD 8.7y) with iRBD (mean 8.7-year disease duration, SD 6.4). A third had not received any information about the link of iRBD with other conditions by health- care professionals. More than half (61%) had searched for that information online. Most patients (87%) wanted to receive prognostic information to help them make informed decisions about their future. The preferred time for this was when a diagnosis of iRBD was made rather than when parkinsonism starts (61% vs 7%). Most wanted this information to come from their iRBD specialist (93%).ConclusionsPatients with iRBD mostly wished to receive information regarding the potential future impli- cations of having iRBD when the diagnosis of iRBD is made. Taking account of patients’ preferences is relevant to decide what, when and how to disclose prognostic information to patients with iRBD.

Sleep disorders, such as sleep-disordered breathing (SDB), insomnia or restless legs syndrome (RLS), are common in the general population and after stroke. In some cases, sleep disturbances are pre-existing, but can also appear de novo as a direct consequence of brain damage or due to stroke-related complications. Furthermore, some sleep conditions may act as a risk factor of stroke. This review explores the available evidence of the two-way relationship between sleep and stroke. Cardiovascular physiological changes during sleep are described, as well as the evidence on the relationship between stroke and sleep duration, SDB, RLS, insomnia, excessive daytime sleepiness (EDS), and circadian rhythm alterations. Potential changes on sleep architecture, and the links that may exist between sleep and functional outcomes after stroke are also discussed. Importantly, sleep-related disturbances may be associated with worse stroke recovery outcomes and increased cerebrovascular morbidity. It is therefore relevant that the bidirectional association between stroke and sleep is taken into consideration by clinicians taking care of these patients. Future research may focus on this mutual relationship for a better understanding of the impact of stroke on sleep, the importance of sleep in stroke incidence and recovery, and have further evidence on treatment strategies that may improve functional outcome after stroke.

Unpleasant dreamlike mentation can occur during non-rapid eye movement parasomnias, leading to associated panic attacks. The mentations are rarely remembered and are likely underreported. However, they may lead to significant personal distress and, if not addressed, may contribute to poorer clinical outcomes. Cotard le délire de negation are very rare nihilistic delusions, historically described with psychotic disorders. Their association with a variety of neurologic disorders, including migraine and cluster-headache, has also been reported. Here we present three cases of Cotard parasomnia during which distinct states of consciousness defined by nihilistic ideation occurred. Patients described believing they are dead or dying, while unable to perceive or experience their bodies in whole, or in part, as their own. A source analysis of the electroencephalographic fingerprint of these mentations suggests right-hemispheric hypoactivity subsequent to confusional arousals. Mechanistically, an aberrant activation of two major intrinsic brain networks of wakefulness, the salience network and the default mode network, is argued. CITATION: Gnoni V, Higgins S, Nesbitt AD, et al. Cotard parasomnia: le délire de negation that occur during the sleep-wake dissociation? J Clin Sleep Med. 2020;16(6):971–976.

The central hypersomnias encompass a range of conditions causing persisting or intermittent excessive daytime sleepiness (EDS). These conditions therefore present not infrequently in general clinical sleep practice, and remain in the differential for patients presenting with sleepiness. Our understanding of the pathophysiology, diagnosis and management of these conditions has progressed significantly over recent years, and in this article we review this group of disorders, focussing in particular on recent changes in classification and diagnosis, pathophysiological advances, and novel treatment options.

The correct spelling of the author should be listed as Laura Perez-Carbonell, MD as shown above.

The aim of this review is to discuss and summarize the main therapeutic strategies for the management of excessive daytime sleepiness (EDS) in patients with narcolepsy. An overview of novel therapies and potential future options are covered as well. First line treatments for EDS in narcolepsy patients include modafinil/armodafinil and sodium oxybate. Other options with a stimulant effect, such as methylphenidate and amphetamines are considered if the former do not control the symptoms. More recently, pitolisant (H3 receptor inverse agonist) was approved by the European Medicines Agency, and solriamfetol (dopamine and norepinephrine reuptake inhibitor) by the Food and Drug Administration, for the treatment of EDS in adult narcolepsy patients. Sodium oxybate was recently approved for EDS management in paediatric patients from the age of seven. Further studies involving the paediatric population are warranted to have solid evidence in the management of children with narcolepsy. Ongoing research of new molecules is based on several mechanisms of action (histamine antagonists/inverse agonists, GABA receptor modulators), and potential future strategies involve immunologic treatment and hypocretin-based therapies. Additionally to the wakefulness-promoting agents and stimulants classically used, other pharmacologic options have been recently approved for the treatment of EDS in Europe (pitolisant) and the US (solriamfetol). Emerging treatments are under development; newly developed wakefulness-promoting agents act via different mechanisms of action, whereas other forms of therapy are focused in the underlying hypocretin deficiency that characterizes narcolepsy type 1.

Limited treatment options are available for patients with central hypersomnia, and many patients continue to have significant symptoms despite use of established therapies. Pitolisant is a novel wakefulness-promoting agent which has been demonstrated to reduce sleepiness in treatment naive patients with narcolepsy. Little is known about the utility of Pitolisant therapy in patients whose symptoms are refractory to other available medications. We assessed outcomes in hypersomnia patients who were resistant to or intolerant of conventional stimulant therapy. Patients with objectively confirmed central hypersomnia, who had persistent EDS despite use of ≥2 wakefulness promoting agents, were commenced on Pitolisant via a pharmacist-led medication management clinic. We prospectively recorded the Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), and quality of life scores (EQ-5D visual analogue scale) at initiation and after 12 weeks of Pitolisant therapy. For the purposes of this analysis we defined responders as those with a reduction in ESS of ≥2. A total of 38 patients were given Pitolisant (55% female; 66% NT1; age 41.2±13.5 years). 6 stopped treatment due to adverse effects (50% insomnia; 33% apparent allergic reaction). Among those who completed 12 weeks of therapy, ESS reduced from 18.4±4.1 to 15.5±5.1 (p=0.001), PSQI reduced from 10.5±3.4 to 8.4±4.5 (p=0.006), and EQ-5D improved from 52±22 to 63±19 (p=0.004). Of the cohort, 17 (45%) were defined as responders – no significant differences in demographic factors, underlying diagnosis, or baseline symptom burden were observed between responders and non-responders. In a cohort of patients with difficult to treat central hypersomnia, Pitolisant therapy led to a reduction in daytime sleepiness and improved quality of life scores in a significant proportion of patients. Pitolisant may be a useful adjuvant therapy in some patients with treatment refractory hypersomnia.

Introduction Excessive daytime sleepiness (EDS) is disabling and it’s control is usually contingent on ongoing pharmacological therapy including: modafinil, methylphenidate, dexamfetamine, pitolisant, and sodium oxybate. Irrespective of the medication used, all pharmacological options must be taken to have an effect and while assessment of treatment adherence is standard clinical practice in many chronic conditions, in sleep medicine, evidence regarding adherence to prescribed medications is strikingly limited. The aim of this study was to assess degree and predictors of adherence to prescribed treatment in patients with narcolepsy attending a tertiary Sleep Disorders Centre. Methods We examined adherence to treatment in consecutive adult patients with a final diagnosis of narcolepsy by comparing prescription collection rates with prescribed therapy over a one-year period. Three levels of adherence were defined depending on the medication supplied in the last year in proportion to the total prescribed: poor (≤50%), intermediate (51–79%), and good (≥80%) adherence. Patients with adherence Results Demographic and clinical characteristics 162 patients were identified, from which 123 subjects with accurate information regarding current treatment regimen and adherence were included (see table 1). Good adherence was seen in 52.8% of patients, whilst 12.2% were intermediately and 35% poorly adherent. No difference was seen in proportion of good adherence between patients with refractory vs non-refractory symptoms (41.5% vs 58.5%; p=0.68). The proportion of refractory patients with suboptimal adherence was 44.9%. Patients with NT1 were less likely than those with NT2 to have suboptimal adherence (40.4% vs 75%; p=0.002). Discussion Our findings suggest that poor adherence is observed in a high proportion of patients with narcolepsy, and that a diagnosis of NT2 seems to be associated with non-adherence to prescribed treatment. Adherence to treatment should be routinely assessed in narcolepsy, particularly prior to initiating any step-up in therapy.

Idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD) is characterized by unpleasant dreams, dream-enacting behaviors, loss of REM sleep atonia, lack of waking motor and cognitive complaints, and the absence of a detectable underlying condition. iRBD typically occurs in people aged 50 years or older, mainly in men, with an estimated prevalence of 0.3–1%. Content of dreams is usually of frightening situations where patients are attacked or chased, and they often have to defend themselves with aggressive behaviors. The most common RBD behaviors are limb and body jerks, but elaborated and aggressive behaviors are also typical, with consequent sleep-related injuries. Despite the ongoing motor-behavioral activity during REM sleep with RBD, it is surprising that patients usually report a good night’s sleep and feel rested upon arising in the morning. Since about 50% of patients are completely unaware of their sleep behaviors, interviews with bed partners are essential to obtain a full clinical description. Clonazepam and melatonin typically have a beneficial effect in treating both RBD nightmares and vigorous behaviors. iRBD patients show markers of neurodegeneration (hyposmia, depression, constipation, substantia nigra hyperechogenicity, abnormal executive, and visuospatial functions) and the neuropathological substrate of Parkinson disease (deposits of abnormal synuclein in the peripheral autonomous nervous system, microglial activation in substantia nigra, and decreased dopamine activity in the striatum). In the majority of cases, there is a progression from iRBD to Parkinson disease, dementia with Lewy bodies, mild cognitive impairment, and multiple system atrophy. iRBD constitutes an excellent target population to test neuroprotective agents to halt or delay the occurrence of parkinsonism and dementia.

Sleep-related movement and behaviour disorders may have an impact on sleep quality and lead to daytime symptoms. These groups of conditions include diseases such as restless legs syndrome, periodic leg movements, and REM and NREM parasomnias. The knowledge of their clinical features and management is of utmost importance for the neurologist and sleep specialist. Frequently, these patients are referred to such specialists and it is relevant to know that certain sleep disorders may be associated with other neurological conditions.