Introduction Narcolepsy type 1 (NT1) is a chronic neurological disorder characterised by excessive daytime sleepiness, cataplexy and disturbed nocturnal sleep. Current pharmacological treatments often have limited efficacy, significant side effects or potential for abuse. Transcutaneous auricular vagus nerve stimulation (taVNS) is a non-invasive neuromodulation technique that has shown promise in regulating sleep–wake cycles and neural network activity. Preliminary evidence from our prior study on Transcutaneous Auricular Vagus Nerve Stimulation in patients with NT1 (TARGET-NT1 study) suggests potential benefits of taVNS for NT1. The TARGET-2 trial aims to evaluate the efficacy and safety of bilateral taVNS compared with unilateral taVNS and sham stimulation in patients with NT1.Methods and analysis TARGET-2 is a randomised double-blind trial. A total of 153 participants with NT1 will be recruited from four centres in China. Participants will be randomly allocated (1:1:1) to receive either (1) bilateral taVNS (active stimulation on both ears), (2) unilateral taVNS (active stimulation on the left ear only) or (3) sham taVNS (minimal intensity stimulation on both ears). Participants and outcome assessors are blinded. The intervention will be administered twice daily for 30 min per session over 12 weeks. The primary outcome is the change in the Epworth Sleepiness Scale (ESS) score from baseline to the 12-week endpoint. Secondary outcomes are changes in the narcolepsy severity scale score, Pittsburgh Sleep Quality Index, fatigue severity scale score, 14-item Hamilton Anxiety Rating Scale score and 17-item Hamilton Depression Rating Scale score, as well as responder rates (proportion with ≥25% ESS reduction) from baseline to week 12. Safety will be assessed by monitoring adverse events.Ethics and dissemination Ethical approval has been granted by the Medical Ethical Committee of Xijing Hospital (Approval Number: XJLL-KY-20252403). Written informed consent will be obtained from all participants before initiating any study-specific procedures. The results will be submitted for publication in a peer-reviewed journal. The anticipated completion date for reporting of the study results is 31 March 2027.Trial registration Chinese Clinical Trial Registry, ChiCTR2500110531 (registered on 15 October 2025).

This study aims to provide physicians with insights into the clinical manifestations and outcomes of children and young adolescents experiencing sleep terrors following SARS-CoV-2 infection. We enrolled patients who developed new onset sleep terrors after SARS-CoV-2infection fromDecember2022to April 2023 in the Xijing hospital, Xi’an, China. We enrolled six patients who experienced sleep terrors following SARS-CoV-2 infection. Out of these patients, five were children and only one was an adolescent, with a mean age of 9 years. Neuroimaging results were negative for all cases. Sleep terrors occurred during both the active course of COVID-19 illness and the recovery period in all patients. Symptoms included crying or screaming in terror, hyperactivity, inappropriate behavior and periods of mental confusion during sleep. These episodes typically occurred 40 min to 1 h after falling asleep. EEG monitoring confirmed two patients’ episodes occurred during non-rapid eye movement (NREM) stage 3 sleep. The duration of sleep terrors ranged from 3mines to30 mines, with each patient experiencing 3–4 to 30–40 instances. Initially, the frequency of episodes was highest at 3–4 times per night, gradually decreasing to once a night, then once a week, until complete disappearance. No medical intervention was required. Clinical follow-up ranged from 6 to 12 months, with spontaneous remission occurring within 1 week to 2 months for different patients. SARS-CoV-2 infection may precipitate acute sleep terrors in children and adolescents. The course of these sleep terrors is generally benign, with all patients achieving spontaneous complete remission over time.

Aim To investigate the prevalence and clinical features of kidney injury in patients with autoimmune encephalitis (AE). Methods Kidney injury was suspected in kidney-involving group due to persistent abnormal in urinary protein and serum albumin. Data on demographics and clinical features were compared between kidney-involving group and kidney-sparing group (patients without kidney injury) using Wilcoxon rank-sum test or chi-square test. Renal biopsy was conducted to identify the type of kidney injury. Results Approximate 30 % (32 of 108) patients with AE were suspicious of kidney injury. Nine patients further tested 24 h urine total protein, and seven of them had an elevated urine protein higher than 150 mg. The predominantly patterns of kidney injury were elevated urine protein, decreased serum albumin and normal kidney function. Compared to kidney-sparing group, the spectrum of AE antibodies in kidney-involving group was different, manifested as less anti-N-methyl-d-aspartate receptor antibodies (50 % vs. 72.4 %, p = 0.025) and more anti-contactin-associated protein like 2 antibodies (18.8 % vs. 1.3 %, p = 0.003). Definite pathological changes indicative of IgA nephropathy and membranous nephropathy in renal biopsy of two cases provided evidence of autoimmune attacks. Discussion Kidney injury occurred in considerable proportion of patients with AE. An in-depth screening for nephropathy could be essential for AE.

Adults with restless sleep disorder (RSD) have never been studied clinically and polysomnographically. This study aimed to describe the clinical manifestation, duration, and distribution of sleep-related movements in adult patients with restless sleep disorder. Patients who had performed VPSG from Jan 2021 to Jan 2022 and met the diagnosis criteria of RSD were enrolled in the study. Patients’ bed partners were also interviewed or telephoned in identifying this disorder. Scoring of movements during sleep was according to the diagnosis criteria of RSD and scoring of large muscle group movements during sleep proposed by the International RLS Study Group in 2020 and 2021, respectively. The clinical manifestation, the distribution of sleep stage as well as the types and duration of the movements were carefully recorded and analyzed. We included ten patients in the study with a mean age of 27.6 years (range 22–38). There was a male prevalence in adults with RSD. The study highlighted the findings from video-polysomnography, which indicated frequent sleep-related movements occurring throughout the Night. These movements were most prominent during N1 and N2 sleep stage, followed by REM sleep, while fewer movements were observed during N3 sleep. Adults with RSD experienced significant daytime functioning impairments, including non-refreshing sleep, daytime fatigue/sleepiness, and mood disturbance. Two of the patients in the study were diagnosed with anxiety and depression, further underscoring the impact of RSD on mental health. Adult patients also suffer from severe RSD, and the RSD that originates in childhood tends to persist into adulthood. In these cases, longer duration of the disease and poor sleep quality may be associated with an increased risk of developing psychiatric comorbidities. Our cases represent an objectively documented type of RSD in younger adult patients.

Background:Sleep-related facial mandibular myoclonus (SRFMM) remains rare in clinical practice. The aim of this study was to provide a comprehensive understanding of the electroclinical manner, therapeutic regimen, and prognosis of SRFMM.Methods:Twenty-three patients who were diagnosed with SRFMM by clinical manifestation, video-electroencephalography (EEG) and electromyography over bilateral masseter and temporalis muscles were enrolled. Clinical and electrophysiological evaluation as well as follow-up information were recorded and analyzed.Results:The cohort involved 4 infants and 19 adults with a mean onset age of 43.5 years for SRFMM, among whom 19 were male. Twenty-one patients complained of tongue injuries and disturbed night-time sleep. SRFMM in 4 patients were ascribed to oral aripiprazole, brainstem ischemia and brain trauma. In 62 SRFMM episodes, 93.5% occurred in NREM sleep and 6.5% in REM sleep, and all events were associated with EEG arousals. In 13 patients with or without clonazepam, the motor events gradually disappeared, and the rest turned to be sporadic.Conclusion:SRFMM is a characteristic parasomnia manifested by tongue biting and accompanying facial mandibular myoclonus, leading to disrupted sleep. Besides adults, infants can also experience SRFMM with spontaneous remission. Most patients respond well to clonazepam, eventually with favorable prognosis.

Background: Excessive daytime sleepiness (EDS) forms a prevalent symptom of obstructive sleep apnea (OSA) and narcolepsy type 1 (NT1), while the latter might always be overlooked. Machine learning (ML) models can enable the early detection of these conditions, which has never been applied for diagnosis of NT1. Objective: The study aimed to develop ML prediction models to help non-sleep specialist clinicians identify high probability of comorbid NT1 in patients with OSA early. Methods: Totally, clinical features of 246 patients with OSA in three sleep centers were collected and analyzed for the development of nine ML models. LASSO regression was used for feature selection. Various metrics such as the area under the receiver operating curve (AUC), calibration curve, and decision curve analysis (DCA) were employed to evaluate and compare the performance of these ML models. Model interpretability was demonstrated by Shapley Additive explanations (SHAP). Results: Based on the analysis of AUC, DCA, and calibration curves, the Gradient Boosting Machine (GBM) model demonstrated superior performance compared to other machine learning (ML) models. The top five features used in the GBM model, ranked by feature importance, were age of onset, total limb movements index, sleep latency, non-REM (Rapid Eye Movement) sleep stage 2 and severity of OSA. Conclusion: The study yielded a simple and feasible screening ML-based model for the early identification of NT1 in patients with OSA, which warrants further verification in more extensive clinical practices.

Epileptic spasms followed by a tonic component have been frequently observed in patients with late-onset spasms (LOS). However, there is a lack of comprehensive analysis and summary of clinical data related to tonic spasms (TS), including seizures, video-electroencephalogram (V-EEG), synchronous electromyography (EMG) and follow-up data. To investigate the characteristics of TS, we prospectively collected the clinical data, including 24-h V-EEG and synchronous EMG data of 32 enrolled patients who suffered from epileptic spasms followed by a TS onset at least once during the 24-h V-EEG in the epilepsy center of Xijing Hospital between June 2015 and July 2020. The patients were prescribed anti-seizure medications (ASMs) and followed up for 2–7 years. The average age of epilepsy onset was 48.06 ± 16.07 months (range: 25 to 88 months). Among the enrolled patients, 22 patients presented with mild intellectual deficits. During the 24-h video-EEG monitoring, an average of 6.94 TS events (range: 3 to 21) were recorded, and these TS seizures often occurred in clusters. In addition to TS, 26 patients experienced generalized tonic-clonic seizures (GTCS), atypical absence seizures, myoclonic seizure, and epileptic spasms. None of the 32 patients with TS displayed hypsarrhythmia during the 24-h video-EEG recording. A total of 28 patients showed normal EEG backgrounds. Interictal epileptic discharges, including slow waves (SW), spike/sharp slow waves (SSW), and spikes, often displayed multifocally. Notably, two patients achieved seizure freedom for more than 2 years through monotherapy with oxcarbazepine (OXC), which was associated with normalization of the EEG. It is difficult to classify the patients with TS as any existing epileptic syndromes, which were distinct from West syndrome or Lennox-Gastaut syndrome. TS might be an underreported seizure type and further studies are needed to gain a more comprehensive understanding of the electro-clinical features and appropriate choice of ASMs for treating tonic spasms.

OBJECTIVES:To explore the efficacy of ofatumumab in new onset narcolepsy type 1 following SARS-CoV-2 infection. METHODS:We present a 9-year-old girl who experienced new onset narcolepsy type 1 following SARS-CoV-2 infection. Polysomnography (PSG) followed by a daytime multiple sleep latency test (MSLT) was under taken after admission. A lumbar puncture was performed to evaluate the CSF orexin-A level. We assessed the CSF hypocretin-1 concentration utilizing the RIA kit from Phoenix Pharmaceuticals Inc. HLA typing was performed. Furthermore, we treated the patient with subcutaneous injections of ofatumumab, and followed her for nearly six-month. The CSF orexin-A level, CD19+ and total B cell population were measured before and after treatment. RESULTS:The girl had experienced SARS-CoV-2 infection 4 months before presentation. After that, she started to experience excessive daytime sleepiness and cataplexy. She also began to experience nightmares and violent behaviors during her nocturnal sleep, which were not present before her SARS-CoV-2 infection. At the same time, she developed obesity and exhibited psychiatric symptoms such as agitation, anxiety, and aggression. MSLT showed a mean sleep latency of 2.7 min, and 5 times sleep onset REM periods. The CSF orexin-A level was pathologically low at 34.06 pg/mL, and she tested positive for HLA-DQB1*06:02. Consequently, a diagnosis of narcolepsy type 1 was confirmed. Before and after treatment with subcutaneous injections of ofatumumab, the CD19+ and total B cell population before treatment and after 1 months showed a significant reduction from 11% and 296 cells per microliter to 0.56% and 11 cells per microliter, respectively. Within a week following ofatumumab therapy, there was a marked improvement in both excessive daytime sleepiness and cataplexy. Notably, her cataplexy was almost entirely resolved following ofatumumab therapy. Her condition remained stable throughout the 9-month follow-up period. She could normally attend school. INTERPRETATION:The efficacy of ofatumumab in this case provides additional support for an autoimmune etiology in narcolepsy with cataplexy, highlighting the potential involvement of B-cells in its pathophysiology. This understanding will aid in the development of specific immunotherapeutic strategies for early implementation upon disease onset.

BackgroundMajor depression disorder (MDD) forms a common psychiatric comorbidity among patients with narcolepsy type 1 (NT1), yet its impact on patients with NT1 is often overlooked by neurologists. Currently, there is a lack of effective methods for accurately predicting MDD in patients with NT1.ObjectiveThis study utilized machine learning (ML) algorithms to identify critical variables and developed the prediction model for predicting MDD in patients with NT1.MethodsThe study included 267 NT1 patients from four sleep centers. The diagnosis of comorbid MDD was based on Diagnostic and Statistical Manual of Mental Disorders fifth edition (DSM-5). ML models, including six full models and six compact models, were developed using a training set. The performance of these models was compared in the testing set, and the optimal model was evaluated in the testing set. Various evaluation metrics, such as Area under the receiver operating curve (AUC), precision-recall (PR) curve and calibration curve were employed to assess and compare the performance of the ML models. Model interpretability was demonstrated using SHAP.ResultIn the testing set, the logistic regression (LG) model demonstrated superior performance compared to other ML models based on evaluation metrics such as AUC, PR curve, and calibration curve. The top eight features used in the LG model, ranked by feature importance, included social impact scale (SIS) score, narcolepsy severity scale (NSS) score, total sleep time, body mass index (BMI), education years, age of onset, sleep efficiency, sleep latency.ConclusionThe study yielded a straightforward and practical ML model for the early identification of MDD in patients with NT1. A web-based tool for clinical applications was developed, which deserves further verification in diverse clinical settings.

Background and objectiveSudden unexpected death in epilepsy (SUDEP) has been regarded as a leading cause of premature death in patients with epilepsy (PWE). Although patients, relatives and caregivers have the right to be informed of SUDEP, neurologists prefer not to release the facts for fear of associated anxiety. In the study, a Chinese questionnaire survey was carried out to elucidate effect of SUDEP disclosure on anxiety in PWE and variables determining the anxiety of patients and provided suggestions for SUDEP disclosure.MethodsA survey study in China was conducted. We recruited 305 PWE from 3 tertiary epilepsy centers who attended outpatient clinic from December 2021 to February 2022. Two hundred and thirty-two PWE completed the screening evaluation, survey and Hamilton anxiety rating scale (HAMA) twice with 171 PWE completing third HAMA at follow-up. HAMA scores at baseline, T1, T2 were compared using analysis of variance and dependent samples t-test. The variables related to anxiety were screened out by univariate analysis and used for multivariate logistic regression.ResultWe found 127 (54.7%) among the 232 participants experienced anxiety after SUDEP disclosure. HAMA scores at T1 were significantly higher than at baseline and T2, while there was no statistical difference between baseline and T2. Medical insurance, seizure severity, and whether the PWE supported SUDEP being disclosed to their relatives and caregivers only were associated with the occurrence of anxiety.ConclusionSUDEP disclosures may cause short-term acute anxiety, but have no long-term effects in PWE. Acute anxiety caused by SUDEP disclosure may be more common in PWE with NCMI and severe seizures. Meanwhile, compared with indirect SUDEP disclosure to their relatives and caregivers, direct SUDEP disclosure to PWE reduces the risk of anxiety. Recommendations are provided to avoid anxiety caused by SUDEP disclosure.

Objective:To study the case of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) which mimic migraine attacks with visual aura, to analyze the clinical features, and to recognize the nature of headache.Methods:The clinical features, image data and video electroencephalogram (EEG) of a suspected patient with MELAS were analyzed. Genomic DNA of mitochondria was extracted from blood and the next generation sequencing was performed to explore the mutation of genes about MELAS.Results:The patient was adolescent-onset, and presented with migraine-like attacks with visual aura, epileptic seizures, stroke-like episodes and hyperlactemia. Brain images suggested basal ganglia calcification, reversible left occipital cortex infarction and abnormal lactic acid peaks in both occipital cortex. Video EEG suggested abnormal adolescent EEG. Mitochondrial DNA sequencing showed that MT-TL1 gene had m. 3243A>G pathogenic mutation.Conclusions:There are a variety of clinical manifestations in MELAS, and migraine-like attacks with visual aura as initial symptoms may be manifestations of occipital lobe epilepsy. Clinicians should avoid confusing the diagnosis of migraine with visual aura, occipital epilepsy and MELAS, in order to make rational clinical decisions.

Study Objectives: To evaluate clinical and electrophysiological features of sleep-related head jerk (SRHJ) and electromyographic activity of superficial neck muscles during head jerk. Methods: Totally, 850 cases with video-polysomnography recording were collected, among which 50 presented with SRHJ. In these 50 patients, 15 underwent electromyography (EMG) check on bilateral sternocleidomastoid (SCM) and trapezius muscles as well as chin, while 35 had only chin EMG check. Further, the sensitivity and specificity of the both EMGs were calculated and compared. Results: Six among the 50 SRHJ patients had a primary complaint of involuntary head jerks associated with impaired sleep. Approximately 76.1% of head jerks occurred during REM sleep with the median head jerk index of 5.9/h, 64.5% of which were associated with electroencephalogram arousals and 66.4% with body movements. One patient showed SRHJ predominantly in NREM sleep but also in wakefulness. Surface EMG of SCM/ trapezius muscles showed a sensitivity of 92% and a specificity of 97.8%, whereas chin EMG had a sensitivity of 14.5% and a specificity of 98.8%. Conclusion: SRHJ was associated with electroencephalogram arousals and might interfere with sleep. Surface EMG of SCM/trapezius muscles exhibited a good accuracy in the revelation of SRHJ.

目的 探讨癫痫合并颈肌阵挛的临床和神经电生理特点.方法 以"癫痫"和"颈肌阵挛"为关键词,在西京医院神经内科脑电监测中心数据库进行检索,检索时间为2018年1月至2020年1月.分析检索到的癫痫合并颈肌阵挛患者的临床和神经电生理特点,并随访.结果 4例符合癫痫合并颈肌阵挛的患者均为男性,均为遗传性全面性癫痫.3例为单纯强直阵挛发作癫痫,1例为眼睑肌阵挛伴失神癫痫,首次癫痫发作年龄9～27岁.2例患者在确诊癫痫及使用丙戊酸钠后出现颈肌阵挛,其中1例患者停用丙戊酸钠后颈肌阵挛消失.24 h V-EEG监测期间共记录到254次颈肌阵挛,92.5％肌阵挛发生于快动眼睡眠期,余多见于非快动眼睡眠1期.结论 遗传性全面性癫痫也许更易发生颈肌阵挛,对于遗传性全面性癫痫合并颈肌阵挛的患者,视频脑电监测对于二者鉴别至关重要,二者共存的电临床特点及其机制需进一步研究.

Sleep disorders are common diseases of nervous system, while sleep disorders in autoimmune encephalitis have received little attention. Sleep involves in the complex function of nerve centers and brain networks. Some autoimmune encephalitis with specific antibodies has sleep disorders as the core symptoms, or sleep patterns change as disease evolution. Sleep disorders often occur in autoimmune encephalitis and usually persist beyond the acute stage of disease,which seriously affect the recovery and life quality of patients. The study of sleep disorders in autoimmune encephalitis is rarely recognized and studied. The characteristics and possible mechanisms of sleep disorders in autoimmune encephalitis were reviewed here to improve the recognition and attention of clinicians.

Objective:To describe the electroclinical features of the coexistence of epilepsy and narcolepsy.Methods:The electroencephalography database was searched using the terms “epilepsy” and “narcolepsy” over a four-year period from January 2016 to December 2019 in the Xijing Hospital. The clinical and electrophysiological characteristics of patients with coexistence of epilepsy and narcolepsy were studied.Results:Five patients with comorbidity for epilepsy and narcolepsy were found, of which three patients were female, two patients were male. The age at epilepsy onset and narcolepsy onset was 2-12 years and 8-17 years, respectively. There were two patients with juvenile myoclonic epilepsy, one with sleep-related hypermoter epilepsy, one with epilepsy with retardation of brain development, one with symptomatic epilepsy with cognitive decline. All the patients had narcolepsy with cataplexy, which followed the onset of epilepsy by three months to eight years. All the patients accepted 24 h video electroencephalography monitoring and multiple sleep latency test. Interictal epileptic discharges were found, mean sleep latency was<8 min, and two or more sleep onset rapid eye movement periods were recorded. Duloxetine hydrochloride can effectively improve the drowsiness and catalepsy symptoms of narcolepsy, and seizures did not worsen in patients using duloxetine hydrochloride.Conclusions:Both generalized and focal epilepsy can occur in narcolepsy with cataplexy. Duloxetine hydrochloride may be safe and effective in treating narcolepsy in patients with epilepsy.

Objective:To discuss the clinical and electrophysiological characteristics of neck myoclonus during sleep.Methods:The clinical and electrophysiological characteristics of 31 patients in the Electroencephalography Monitoring Center of Xijing Hospital from January 2020 to August 2020 were studied retrospectively. All the patients received video-polysomnography and video-electroencephalography.Results:There were 22 males (71%) and nine females (29%) in the 31 patients. The mean age of the patients at the time of inclusion in the study was 27.8 years. Neck myoclonus was most common in patients with narcolepsy ( n=8), followed by epilepsy ( n=4), obstructive sleep apnea syndrome ( n=4), anxiety and depression ( n=3), snoring ( n=3), etc. A total of 555 motor events were considered and analyzed, 89.5% (497/555) of which occurred during rapid eye movement (REM) sleep. The mean neck myoclonus index in REM sleep (5.8) was significantly higher than that in non-rapid eye movement sleep (0.2). Totally 48.3% (268/555) of neck myoclonus were accompanied by an arousal, 0.7% (4/555) by a full awakening, and 2.7% (15/555) by limb movements. Conclusions:Neck myoclonus is common during REM sleep, which can occur in patients with sleep disorders and epilepsy. Physiological or pathological significance of neck myoclonus has to be investigated in further studies.

*These authors contributed equally to this work Study Objectives: To evaluate clinical and electrophysiological features of sleep-related head jerk (SRHJ) and electromyographic activity of superficial neck muscles during head jerk. Methods: Totally, 850 cases with video-polysomnography recording were collected, among which 50 presented with SRHJ. In these 50 patients, 15 underwent electromyography (EMG) check on bilateral sternocleidomastoid (SCM) and trapezius muscles as well as chin, while 35 had only chin EMG check. Further, the sensitivity and specificity of the both EMGs were calculated and compared. Results: Six among the 50 SRHJ patients had a primary complaint of involuntary head jerks associated with impaired sleep. Approximately 76.1% of head jerks occurred during REM sleep with the median head jerk index of 5.9/h, 64.5% of which were associated with electroencephalogram arousals and 66.4% with body movements. One patient showed SRHJ predominantly in NREM sleep but also in wakefulness. Surface EMG of SCM/ trapezius muscles showed a sensitivity of 92% and a specificity of 97.8%, whereas chin EMG had a sensitivity of 14.5% and a specificity of 98.8%. Conclusion: SRHJ was associated with electroencephalogram arousals and might interfere with sleep. Surface EMG of SCM/trapezius muscles exhibited a good accuracy in the revelation of SRHJ.

• Cataplexy can be the only clinical manifestation in the early stage of NPC disease. • The electrophysiological feature of cataplexy is different between NPC and idiopathic narcolepsy. • High amplitude delta pattern correlates of cataplexy indicated the involvement of cortico-thalamo-cortical feedback loops. • Electrophysiological monitoring is of great significance to better understand the pathophysiology of cataplexy.

目的:分析基于特发性和继发性快速眼动睡眠行为障碍的睡眠结构.方法:择10例特发性快速眼动睡眠行为障碍(RBD)、10例继发性RBD患者为研究对象,分析两组患者的睡眠结构.结果:据统计,相比继发性RBD患者,特发性RBD患者夜间活动于语言及肢体动作较多,两组差异显著(P<0.05).结论:特发性RBD患者其夜间行为异常较多,睡眠结构无显著改变,而继发性RBD患者存在显著睡眠结构紊乱.