Differentiating Oneiric Stupor in Agrypnia Excitata From Dreaming Disorders

Oneiric Stupor (OS) in Agrypnia Excitata represents a peculiar condition characterized by the recurrence of stereotyped gestures such as mimicking daily-life activities associated with the reporting of a dream mentation consisting in a single oneiric scene. It arises in the context of a completely disorganized sleep structure lacking any physiological cyclic organization, thus, going beyond the concept of abnormal dream. However, a proper differential diagnosis of OS, in the complex world of the “disorders of dreaming” can become quite challenging. The aim of this review is to provide useful clinical and videopolygraphic data on OS to differentiate it from other dreaming disorders. Each entity will be clinically evaluated among the areas of dream mentation and abnormal sleep behaviors and its polygraphic features will be analyzed and distinguished from OS

Definition and diagnostic criteria of sleep-related hypermotor epilepsy

The syndrome known as nocturnal frontal lobe epilepsy is recognized worldwide and has been studied in a wide range of clinical and scientific settings (epilepsy, sleep medicine, neurosurgery, pediatric neurology, epidemiology, genetics). Though uncommon, it is of considerable interest to practicing neurologists because of complexity in differential diagnosis from more common, benign sleep disorders such as parasomnias, or other disorders like psychogenic nonepileptic seizures. Moreover, misdiagnosis can have substantial adverse consequences on patients' lives. At present, there is no consensus definition of this disorder and disagreement persists about its core electroclinical features and the spectrum of etiologies involved. To improve the definition of the disorder and establish diagnostic criteria with levels of certainty, a consensus conference using formal recommended methodology was held in Bologna in September 2014. It was recommended that the name be changed to sleep-related hypermotor epilepsy (SHE), reflecting evidence that the attacks are associated with sleep rather than time of day, the seizures may arise from extrafrontal sites, and the motor aspects of the seizures are characteristic. The etiology may be genetic or due to structural pathology, but in most cases remains unknown. Diagnostic criteria were developed with 3 levels of certainty: witnessed (possible) SHE, video-documented (clinical) SHE, and video-EEG-documented (confirmed) SHE. The main research gaps involve epidemiology, pathophysiology, treatment, and prognosis

Definition and diagnostic criteria of sleep-related hypermotor epilepsy.

The syndrome known as nocturnal frontal lobe epilepsy is recognized worldwide and has been studied in a wide range of clinical and scientific settings (epilepsy, sleep medicine, neurosurgery, pediatric neurology, epidemiology, genetics). Though uncommon, it is of considerable interest to practicing neurologists because of complexity in differential diagnosis from more common, benign sleep disorders such as parasomnias, or other disorders like psychogenic nonepileptic seizures. Moreover, misdiagnosis can have substantial adverse consequences on patients' lives. At present, there is no consensus definition of this disorder and disagreement persists about its core electroclinical features and the spectrum of etiologies involved. To improve the definition of the disorder and establish diagnostic criteria with levels of certainty, a consensus conference using formal recommended methodology was held in Bologna in September 2014. It was recommended that the name be changed to sleep-related hypermotor epilepsy (SHE), reflecting evidence that the attacks are associated with sleep rather than time of day, the seizures may arise from extrafrontal sites, and the motor aspects of the seizures are characteristic. The etiology may be genetic or due to structural pathology, but in most cases remains unknown. Diagnostic criteria were developed with 3 levels of certainty: witnessed (possible) SHE, video-documented (clinical) SHE, and video-EEG-documented (confirmed) SHE. The main research gaps involve epidemiology, pathophysiology, treatment, and prognosis

MECP2 duplication syndrome: The electroclinical features of a case with long-term evolution

MECP2 duplication syndrome (MDS) is a rare and severe neurodevelopmental disorder frequently associated with epilepsy. Different seizure types and electroencephalographic (EEG) patterns were described in patients with MDS, although it lacks a specific phenotype. We report on an adult patient with long-term epilepsy showing an evolution of the EEG pattern that progressively changed into burst suppression (BS) during sleep. As BS has not been previously reported in MDS, this report expands the neurophysiological phenotype of MDS and further suggest the possible occurrence of a longitudinal spectrum of seizure types and EEG patterns in MDS

Violent and Complex Behaviors and Non-Restorative Sleep Are the Main Features of Disorders of Arousal in Adulthood: Real Picture or a More Severe Phenotype?

Disorders of arousal (DoA) are NREM parasomnias characterized by motor and emotional behaviors emerging from incomplete arousals from deep sleep. DoA are largely present in pediatric populations, a period during which they are labeled as self-limited manifestations. However, an extensive literature has shown that DoA can persist in adulthood, with different characteristics from childhood DoA. Adult DoA patients usually report excessive daily sleepiness, sleep-related violence during DoA episodes or potentially harmful behaviors, which are rare in childhood. The semeiological features of DoA episodes in adulthood may complicate differential diagnoses with other motor manifestations during sleep, in particular sleep-related hypermotor epilepsy. However, it cannot be excluded that adults with DoA attending sleep centers constitute a more severe phenotype, thus not being representative of adult DoA in the general population. Video-polysomnographic studies of DoA document a spectrum of motor patterns of different complexities, the simplest of which may often go unnoticed. Despite the different complexities of the episodes, neurophysiologic studies showed the co-existence of deep sleep and wakefulness during DoA episodes or even before their onset. These aspects make DoA an ideal model to investigate the mechanisms regulating local sleep, sleep arousal and cognitive functions including spatial and temporal orientation, attention or memory

Clinical features and pathophysiology of disorders of arousal in adults: A window into the sleeping brain

Introduction: Disorders of Arousal (DoA) are NREM parasomnias that have been typically regarded as self-limited childhood manifestations. It is now clear that DoA can persist in adults, often presenting with distinctive characteristics. So far, few studies have described the clinical course and characteristics of DoA in adulthood, therefore a large part of their semiology is ignored. The aim of this study is to describe the clinical manifestations of DoA in an adult population and to provide a pathophysiological interpretation of their features. Methods: We screened our database for all 1,600 adult ( 6515 years) patients with sleep-related motor behaviors between 1995 and 2016. We identified 45 patients with typical DoA episodes, of whom a complete history, neurological examination and diagnostic video-polysomnography (VPSG) were available. All patients provided a detailed description of their episodes (with particular regards to semiology, frequency, and association with stressful life events) in different life periods. VPSG recordings were reviewed and DoA episodes were identified and assigned to three different categories according to their complexity. Results: Our population was composed of 45 adult patients ranging between 15 and 76 years. Sleepwalking was reported by 86% of patients, possibly associated with complex interactions with the environment and violent behaviors in 53% of cases; distressing mental contents were reported by 64%. Recall of the episodes was reported in 77% of patients. Non-restorative sleep was reported in 46% of patients. Stress was a potential episode trigger in 80% of patients. VPSG recordings documented 334 DoA episodes. According to our classification of motor patterns, 282 episodes (84%) were Simple Arousal Movements (SAMs), 34 (10%) Rapid Arousal Movements (RAMs) and 18 (5%) Complex Arousal Movements (CAMs). Discussion: Our study confirms that DoA in adulthood present with distinctive characteristics, such as non-restorative sleep, violence and complex, or bizarre behaviors. Alternative classifications of DoA based on motor patterns could be useful to characterize DoA episodes in adults, as different motor patterns often coexist in the same individual and minor episodes are more common but generally underreported by patients. Prospective studies are needed for a definitive characterization of DoA in adulthood throughout the life course

Nightmare disorder and REM sleep behavior disorder in inflammatory arthritis: Possibility beyond neurodegeneration.

OBJECTIVES:To investigate the prevalence of REM sleep behavior disorder (RBD) in patients with inflammatory arthritis (IA) to ascertain if RBD could be an internal red flag signaling a fluctuating state of inflammation based on the theory of "protoconsciousness". MATERIALS & METHODS:One hundred and three patients with a confirmed diagnosis of IA were consecutively recruited. The patients underwent general (IA activity, functional status, laboratory tests) and neurological evaluations. A neurologist investigated RBD and REM sleep parasomnias in a semi-structured interview. Sleep quality was assessed with the Pittsburgh Sleep Quality Index, while the risk of obstructive sleep apnea syndrome (OSAS) was evaluated with the Berlin questionnaire. Beck Depression Inventory II and State-Trait Anxiety Inventory investigated depression and anxiety. RESULTS:Patients had a mean age of 53.7 ± 14.6 years, 65% were women; 57.3% were in a clinically active phase of IA. Two women fulfilled ICSD-3 criteria for RBD appearing 11 years after and 20 years before IA onset respectively. 31 patients scored positive for nightmare disorder (ND), 8 for recurrent isolated sleep paralysis. 65 (63.1%) patients reported poor sleep quality and 25 (24.3%) resulted at high risk for OSAS. 32 (31.0%) patients scored positively for depression or anxiety. CONCLUSIONS:The prevalence of RBD in patients with IA did not differ from that in the general population, whereas ND presented a 2-fold increased prevalence. Whether RBD can be considered a red flag signaling an internal danger remains an open question, while ND may be a new player in this intriguing relation

Gender medicine and sleep disorders: from basic science to clinical research

Several pivotal differences in sleep and sleep disorders are recognized between women and men. This is not only due to changes in hormonal balance during women's reproductive life, such as in pregnancy and menopause. Women are more likely to report insomnia and non-specific symptoms of apneas, such as fatigue or mood disturbance, compared to men. Thus, it is important for clinicians and researchers to take sex and gender differences into account when addressing sleep disorders in order to acknowledge the biology unique to women. We present a narrative review that delves into the primary sleep disorders, starting from basic science, to explore the impact of gender differences on sleep and the current status of research on women's sleep health

Sleep profile in a population of community-dwelling nonagenarians: data from the Mugello study

Background: Very few studies have investigated sleep characteristics in the oldest-old individuals (aged ≥85 years) and data collected often rely on self-reported information. This study had three aims: (i) to objectively assess, using a wearable device, the sleep characteristics of a large community of oldest-old subjects; (ii) to assess differences in sleep parameters between self-reported ‘good sleepers’ and ‘bad sleepers’; (iii) to assess whether there was a relationship between sleep parameters and cognitive status in this community-dwelling population. Methods: There were 178 subjects (74.2% women, median age 92 years) included in the ‘Mugello study’, who wore an armband 24 h/day for at least two consecutive nights to estimate sleep parameters. The perceived sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI), the cognitive status through the Mini-Mental State Examination. Continuous variables were compared between men/women, and good/bad sleepers with the independent t-test or Mann–Whitney U-test, according to data distribution. Chi-square test was used for categorical/dichotomous variables. An ordinal logistic regression model was used to study the possible association between sleep parameters and cognitive function. Results: Participants spent in bed nearly 9 h, with a total sleep time of 7 h, a sleep onset latency of 17 min, and a sleep efficiency of 83%. Sleep onset latency was significantly associated with different cognitive levels when age and education level were considered. No significant difference in sleep parameters estimated using the SenseWear armband were found between poor (n = 136, 76.4%) and good sleepers (n = 42, 23.6%), identified according to the PSQI. Conclusions: In this study, actigraphic measurements revealed that subjects with a cognitive decline were more prone to increased sleep onset latency. Sleep quality assessed using the PSQI was not coherent with actigraphic measurements in this sample, supporting the need for objective measures when investigating sleep quality in the oldest-old population

Functional connectivity and metabolic brain alterations in sleepwalkers

Objective: Disorders of arousal (DoA) are characterized by an intermediate state between wakefulness and deep sleep, leading to incomplete awakenings from NREM sleep. Multimodal studies have shown subtle neurophysiologic alterations even during wakefulness in DoA. The aim of this study was to explore the brain functional connectivity in DoA and the metabolic profile of the anterior and posterior cingulate cortex, given its pivotal role in cognitive and emotional processing. Methods: Fifteen consecutive patients with DoA (9 males, mean age 26.3 ± 7.7) and 15 age- and sex-matched healthy controls (8 males, mean age 25.8 ± 3.6) were enrolled. All participants underwent a protocol including sleep and psychological evaluation scales and multimodal brain MRI with resting-state functional MRI and 1H-MR spectroscopy. Results: The independent component analysis disclosed an altered resting-state functional connectivity (FC) in the patients' sensory motor network, with a higher connectivity strength in opercular cortex, precuneus, occipital pole, and lingual gyrus. The seed-based analysis revealed a decreased FC between posterior cingulate cortex (PCC) and several cerebral areas. Finally, spectroscopic imaging revealed a reduced content of glutamine in the PCC (p < 0.001). Interpretation: The increased connectivity in the sensory-motor network of DoA patients could constitute a “facilitatory medium” enhancing motor circuit activation, while the connectivity and metabolic alterations of PCC might represent a trait functional feature, contributing to a dysfunctional arousal process and the difficulty to reach a complete awareness during DoA episodes. In addition, these alterations at rest might be related to daytime impairment reported by patients, requiring new strategies for DoA management