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Narcolepsy is a rare sleep disorder characterized by excessive daytime sleepiness (EDS), cataplexy, sleep hallucination and sleep paralysis, and the vast majority of patients are accompanied by anxiety. There are neuronal interconnections and projections between the neural circuits of narcolepsy and anxiety, which may share common pathophysiology mechanisms, and some medications used clinically to treat cataplexy in narcolepsy may also improve anxiety. In this study, we propose to review the epidemiologic research, pathogenesis and therapeutic advances in narcolepsy with anxiety, with the aim of increasing the diagnosis and treatment of narcolepsy with anxiety and improving the prognosis of narcolepsy with anxiety patients.

Objective To analyse the neuropsychological characteristics, sleep structure and HLA ⁃ DQB1 gene distribution of narcolepsy type 1 (NT1) patients. Methods A total of 35 patients with NT1 admitted to Tianjin Medical University General Hospital, from August 2018 to November 2022 were included, all of whom underwent neuropsychological tests, noctural polysomnography (nPSG) monitoring, Multiple Sleep Latency Test (MSLT), narcolepsy evaluation and HLA ⁃ DQB1 gene detection. Results Neuropsychological tests showed the scores of Montreal Cognitive Assessment (MoCA; t = 3.159, P = 0.004) and the correct number of immediate recall of the first (t = 3.028, P = 0.004) and the second (t = 2.054, P = 0.044) of Hopkins Verbal Learning Test⁃Revised (HVLT⁃R) were lower of the NT1 group than those of the control group. Trail Making Test (TMT) ⁃B completion time (t = ⁃ 2.126, P = 0.019), Hamilton Anxiety Scale (HAMA) score (Z = ⁃ 5.109, P = 0.000), Hamilton Depression Scale⁃17 (HAMD⁃17) score (Z = ⁃ 3.204, P = 0.001), Epworth Sleepiness Scale (ESS) socre (t = ⁃ 13.609, P = 0.000) and Pittsburgh Sleep Quality Index (PSQI) score (Z = ⁃ 3.004, P = 0.003) were higher of the NT1 group than those of the control group. nPSG showed the total recording time (Z = ⁃ 4.491, P = 0.000), the proportion of non⁃rapid eye movement (NREM) 3 (Z = ⁃ 2.647, P = 0.008), the proportion of rapid eye movement (REM; t = 2.908, P = 0.005) and periodic limb movements of sleep index (PLMSI; Z = ⁃ 3.501, P = 0.000) were higher of the NT1 group than those of the control group. Sleep efficiency (t = ⁃ 2.489, P = 0.016), sleep latency (Z = ⁃ 4.112, P = 0.000), REM sleep latency (Z = ⁃ 4.318, P = 0.000) and the proportion of NREM2 (t = ⁃ 5.224, P = 0.000) were lower of the NT1 group than those of the control group. The clinical features of NT1 patients were excessive daytime sleepiness (97.14%, 34/35), cataplexy (97.14%, 34/35), other sleep disturbances (88.57%, 31/35), sleep hallucination (82.86%, 29/35), and sleep paralysis (68.57%, 24/35). And 34 cases (97.14%) carried the allele HLA ⁃ DQB1*0602. Conclusions Patients with NT1 have cognitive decline, mood change and sleep structure disorder, and the carrying rate of HLA⁃DQB1*0602 is high.

Objectives: To compare the characteristics of rapid eye movement(REM) density in patients with narcolepsy and idiopathic hypersomnia,and to explore the possibility of REM density may be used as a biomarker for the diagnosis of narcolepsy. Methods: Nocturnal polysomnography(nPSG),multiple sleep latency test(MSLT)data were compared and analyzed retrospectively among hospitalized patients with idiopathic hypersomnia as chief complaint(n=40). Patients with clinical diagnosis and MSLT≤8 min and SOREMPs≥2 were divided into narcolepsy group(n=25),and others were divided into idiopathic hypersomnia group(n=15).Demographic data,nPSG and MSLT data were compared in narcolepsy and idiopathic hypersomnia patients. Results: The narcolepsy group had significantly shorter sleep latency [(9.1±5,9) min vs.(3.8±3.3) min,P <0.001],more SOREMPs[(0.3±0.5) vs.(3.9±1.1),P <0.001],and more REM density compared with idiopathic hypersomnia group[(3.1±1.2) vs.(4.2±1.6),P <0.05]. Conclusions: It suggests that rapid eye movement density is increased in patients with narcolepsy,which may be used as a biomarker of auxiliary diagnosis for narcolepsy. [ABSTRACT FROM AUTHOR]

The purpose of this article is to discuss the clinical diagnosis and treatment of narcolepsy and parasomnias. In view of high probability and great harm of missed diagnosis and misdiagnosis of narcolepsy， as well as its close association with parasomnias， this study turns the research perspective to the clinical features of narcolepsy， the effects of rapid eye movement （REM） sleep， the comorbidity of narcolepsy and parasomnia （e.g. sleep paralysis， hypnagogic hallucination， and REM-sleep behavior disorder）， and the treatment of narcolepsy.

Dopamine,a neurotransmitter ubiquitous in the body fluids,blood,and urine of mammals and humans,is responsible for regulating their functions and metabolism.The dopamine system is involved in the neurobiological mechanisms of narcolepsy in animals and humans.However,researchers have drawn different or even opposite conclusions when measuring the dopamine level in the cerebrospinal fluid of narcolepsy patients.Studies have confirmed that the occurrence of narcolepsy is related to the irreversible loss of orexins.The autoimmune reaction caused by the interactions of environmental factors with genetic factors destroys the hypothalamic orexin neurons and reduces orexin secretion,thereby lowering the level of arousal.We introduce the research progress and current status of dopamine and clinical characterization of narcolepsy by reviewing more than 40 articles published from 1982 to 2023,aiming to provide a reference for studying the relationship between the dopamine level and clinical characterization of narcolepsy and searching for the biomarkers of type 2 narcolepsy.

Objective To comprehensively analyze the clinical and electroneurophysiological characteristics during the process of cataplexy by dynamic video?EEG?EMG monitoring. Methods Six narcolepsy type 1 patients with typical cataplexy were enrolled and 2 of them were diagnosed as status cataplecticus. All patients underwent polysomnography (PSG) and daytime Multiple Sleep Latency Test (MSLT) to clarify the diagnosis. Cataplexy was triggered by emotional stimulus and recorded under dynamic video-EEG-EMG monitoring. EEG characteristics during cataplexy were further compared and analyzed. Objective To comprehensively analyze the clinical and electroneurophysiological characteristics during the process of cataplexy by dynamic video-EEG-EMG monitoring. Methods Six narcolepsy type 1 patients with typical cataplexy were enrolled and 2 of them were diagnosed as status cataplecticus. All patients underwent polysomnography (PSG) and daytime Multiple Sleep Latency Test (MSLT) to clarify the diagnosis. Cataplexy was triggered by emotional stimulus and recorded under dynamic video-EEG-EMG monitoring. EEG characteristics during cataplexy were further compared and analyzed. Results Fourteen cataplectic attacks in 6 patients were recorded. According to the clinical and video- EMG characteristics, cataplectic attack was divided into 4 stages, including triggering phase (CA1), resisting phase (CA2), atonic phase (CA3) and recovering phase (CA4). EEG frequency and amplitude varied from one stage to another and hypersynchronous paroxysmal theta (HSPT) was observed in early resisting phase (CA2), which was supposed to be a distinctive EEG characteristic during the onset of cataplexy. Conclusions Generalized cataplectic ttack contain 4 stages, which indicate a complicated and dynamic process in clinical and electroneurophysiology. Moreover, it's highly possible that HSPT during resisting phase (CA2) is critical in the mechanism of cataplexy. DOI: 10.3969/j.issn.1672-6731.2017.09.006

The discovery of hypothalamus Hypocretin (Hcrt)/Orexin system and its relationship with narcolepsy is one of the major advances in sleep medicine. Hcrt/Orexin is a significant neurotransmitter in the circadian cycle of arousal. Basic research about Hcrt/Orexin system has been applied in clinical practice and the measurement of Hcrt/Orexin in the cerebrospinal fluid (CSF) is the golden criteria and classification basis for diagnosis of narcolepsy. Breakthrough advance has been made in genetic immunology of hypothalamus Hcrt/Orexin neuronal apoptosis of narcolepsy patients. Medications aimed at Hcrt/Orexin receptors are considered to be a novel target in the treatment of sleep disorders. DOI: 10.3969/j.issn.1672-6731.2017.09.004

Objective To assess circadian characteristics of spontaneous physical activity and deep body temperature in narcoleptic patients. Methods Fourteen narcoleptic patients and 14 healthy age- and sex-matched control subjects were enrolled. Nocturnal polysomnography (PSG) was recorded, followed by standard multiple sleep latency test (MSLT). Then all subjects were required to wear the actigraphy (actiwatch) at home with continuous monitoring of spontaneous physical activity for 1-2 weeks and complete the daily sleep record. All subjects' deep body temperatures were measured at 20 time points. Results In comparison with control subjects, PSG data suggested narcoleptic patients had significantly longer time in bed at night (P = 0.008), decreased sleep efficiency (P = 0.001), increased awakenings (P = 0.000), extended wake time after sleep onset (P = 0.000) and sleep onset rapid eye movement period (SOREMP, P = 0.002). MSLT data suggested decreased average sleep latency (P = 0.000) and increased SOREMPs (P = 0.000). Actigraphy data suggested increased nocturnal activity and nocturnal activity per hour (P = 0.000, for all), decreased daytime activity and daytime activity per hour (P = 0.000, for all) and increased nocturnal activity per hour/daytime activity per hour (P = 0.000, for all). The deep body temperature in both groups showed significant circadian rhythms. The differences in mesor, amplitude and peak phase of deep body temperature between 2 groups had no statistical significance (P = 0.177, 0.730, 0.488). Conclusions Narcoleptic patients are characterized by impaired circadian rhythm of sleep-wake and spontaneous physical activity. The limited effects on deep body temperature suggest the relative conservation of thermoregulation in narcolepsy. DOI: 10.3969/j.issn.1672-6731.2016.07.010

DOI: 10.3969/j.issn.1672-6731.2017.09.009

Copyright of Chinese Journal of Contemporary Neurology & Neurosurgery is the property of Chinese Journal of Contemporary Neurology & Neurosurgery and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The discovery of hypothalamus Hypocretin (Hcrt)/Orexin system and its relationship with narcolepsy is one of the major advances in sleep medicine. Hcrt/Orexin is a significant neurotransmitter in the circadian cycle of arousal. Basic research about Hcrt/Orexin system has been applied in clinical practice and the measurement of Hcrt/Orexin in the cerebrospinal fluid (CSF) is the golden criteria and classification basis for diagnosis of narcolepsy. Breakthrough advance has been made in genetic immunology of hypothalamus Hcrt/Orexin neuronal apoptosis of narcolepsy patients. Medications aimed at Hcrt/Orexin receptors are considered to be a novel target in the treatment of sleep disorders. [ABSTRACT FROM AUTHOR]

Objective To comprehensively analyze the clinical and electroneurophysiological characteristics during the process of cataplexy by dynamic video - EEG - EMG monitoring. Methods Six narcolepsy type 1 patients with typical cataplexy were enrolled and 2 of them were diagnosed as status cataplecticus. All patients underwent polysomnography (PSG) and daytime Multiple Sleep Latency Test (MSLT) to clarify the diagnosis. Cataplexy was triggered by emotional stimulus and recorded under dynamic video - EEG - EMG monitoring. EEG characteristics during cataplexy were further compared and analyzed. Results Fourteen cataplectic attacks in 6 patients were recorded. According to the clinical and video - EMG characteristics, cataplectic attack was divided into 4 stages, including triggering phase (CA1), resisting phase (CA2), atonic phase (CA3) and recovering phase (CA4). EEG frequency and amplitude varied from one stage to another and hypersynchronous paroxysmal theta (HSPT) was observed in early resisting phase (CA2), which was supposed to be a distinctive EEG characteristic during the onset of cataplexy. Conclusions Generalized cataplectic attack contain 4 stages, which indicate a complicated and dynamic process in clinical and electroneurophysiology. Moreover, it's highly possible that HSPT during resisting phase (CA2) is critical in the mechanism of cataplexy. [ABSTRACT FROM AUTHOR]

Narcolepsy is a severe neurological sleep disorder, which is caused by the large loss of Hypocretin (Hcrt)/Orexin producing neurons in the hypothalamus in narcolepsy with cataplexy patients. It is likely the result of an autoimmune process that causes selective destruction of Hcrt neurons in the hypothalamus. It has been known that narcolepsy is associated with the human leukocyte antigens (HLA)-DR2 and HLA-DQB1*0602. Recent epidemic data have shown a robust incidence of disease onset associated with pandemic H1N1-infection, H1N1 vaccination and streptococcus pyogenes infection. A genome-wide association variants in P2RY11 are associated with narcolepsy. Narcolepsy is associated with polymorphisms of the genes encoding T cell receptor-α (TCR-α) and tumor necrosis factor-α (TNF-α). Several cases report successful management of cataplexy with intravenous immunoglobulins at narcolepsy onset, but the effects are not persistent. However, there is no significant evidence of narcolepsy specific antibodies or T cell reactions found, and the relations with cytokines and central nervous system inflammatory cells have not been elaborated well. It is still a big challenge for further study.

Objective To evaluate the therapeutic efficacy and side effects of various treatment for sleep disorders in order to provide the best therapeutic regimen for the evidence-based treatment of sleep disorders. Methods Sleep disorder, insomnia, restless legs syndrome or RLS, obstructive sleep apnea or OSA, narcolepsy, REM behaviour disorder or RBD, treatment or therapy were used as retrieval words. Cochrane Library, MEDLINE, ScienceDirect were used for retrieval, and manual searching was also used. Related clinical guidelines, systematic reviews, randomized controlled clinical trials, retrospective case analysis, case-observation studies and reviews were collected and evaluated by Jadad Scale. Results Forty related articles were selected as following: 6 clinical guidelines, 12 systematic reviews, 5 randomized controlled trials, 2 retrospective case analysis, 1 case-observation study and 14 reviews. Thirty-three were of high quality, while 7 were of low quality with score. According to the evaluation of therapeutic efficacy and side effects of various therapies, it is suggested as following: 1) insomnia is the most common in sleep disorders; the treatment methods of insomnia mainly include drug therapy and cognitive behavioral treatment (CBT); the two kinds of therapy have their own advantages and disadvantages, and the combination therapy of drugs and CBT is the best treatment plan. 2) The first-line treatment of primary RLS is dopamine agonists and anti-seizure drugs; however, the treatment of secondary RLS is mainly etiologic treatment. 3) The main treatments of OSAS are nasal continuous positive airway pressure (nCPAP), oral orthotics and surgery, and nCPAP is the first-line treatments. 4) The medication of narcolepsy is mainly modafinil, hydroxy butyric acid sodium and antidepressants, and the specific choosing should accord to clinical classifications. 5) The main treatments of RBD include general treatments such as avoiding triggers, insuring the safety of sleep environment, and drug therapy such as benzodiazepines and melatonin. Conclusion Evidence-based medicine can provide the best clinical evidence on sleep disorders' treatment.

Copyright of Chinese Journal of Contemporary Neurology & Neurosurgery is the property of Chinese Journal of Contemporary Neurology & Neurosurgery and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

This study aims to explore the clinical effect of venlafaxine sustained-release capsules combined with methylphenidate hydrochloride tablets on narcolepsy. Thirty-eight cases of narcoleptic patients were randomly divided into venlafaxine combined with methylphenidate hydrochloride treatment group (observation group, N = 19) and methylphenidate hydrochloride and clomipramine treatment group (control group, N = 19). After a total of 12-week treatment, clinical curative effect and adverse drug reactions were observed in 2 groups of patients. The results showed that effective rate of the treatment for excessive daytime sleepiness (EDS) in observation group was higher than that of the control group (15/19 vs 8/19, P = 0.044), and effective rate of the treatment for cataplexy in observation group was higher than that of the control group (13/19 vs 6/19, P = 0.048). The rate of adverse drug reactions in observation group was lower than that in the control group (χ2 = 8.889, P = 0.003). It was indicated that venlafaxine combined with methylphenidate had good curative effect on narcolepsy with EDS and cataplexy symptoms. doi：10.3969/j.issn.1672-6731.2013.11.010

We present the case of an 18-year-old male patient who had narcolepsy type 1 and comorbid schizophrenia. The patient's first symptom was mainly excessive daytime sleepiness, which was followed by psychotic symptoms, including hallucinations, delusions, and abnormal speech and behaviors. After admission to the hospital, the patient underwent a number of ancillary tests. Multiple sleep latency test (MSLT) showed that the mean sleep latency was 2 min and the hypocretin-1 was found to be 90.56 pg/mL. The patient was diagnosed with: 1) schizophrenia; 2) narcolepsy. After receiving antipsychotic drugs and behavioral therapy, the patient's hallucinations and abnormal speech and behaviors disappeared, and delusions and excessive daytime sleepiness decreased significantly. The symptoms and manifestations of narcolepsy type 1 overlaps with those of schizophrenia, which may lead to misdiagnosis and underdiagnosis in clinical practice. The case is presented with a view to providing a reference for the clinical diagnosis and treatment of narcolepsy type 1 with comorbid schizophrenia., (Copyright© by Editorial Board of Journal of Sichuan University (Medical Sciences).)

Objective To evaluate the therapeutic efficacy and side effects of various treatment for sleep disorders in order to provide the best therapeutic regimen for the evidence-based treatment of sleep disorders. Methods Sleep disorder, insomnia, restless legs syndrome or RLS, obstructive sleep apnea or OSA, narcolepsy, REM behaviour disorder or RBD, treatment or therapy were used as retrieval words. Cochrane Library, MEDLINE, ScienceDirect were used for retrieval, and manual searching was also used. Related clinical guidelines, systematic reviews, randomized controlled clinical trials, retrospective case analysis, case - observation studies and reviews were collected and evaluated by Jadad Scale. Results Forty related articles were selected as following: 6 clinical guidelines, 12 systematic reviews, 5 randomized controlled trials, 2 retrospective case analysis, 1 case-observation study and 14 reviews. Thirty-three were of high quality, while 7 were of low quality with score. According to the evaluation of therapeutic efficacy and side effects of various therapies, it is suggested as following: 1) insomnia is the most common in sleep disorders; the treatment methods of insomnia mainly include drug therapy and cognitive behavioral treatment (CBT); the two kinds of therapy have their own advantages and disadvantages, and the combination therapy of drugs and CBT is the best treatment plan. 2) The first-line treatment of primary RLS is dopamine agonists and anti - seizure drugs; however, the treatment of secondary RLS is mainly etiologic treatment. 3) The main treatments of OSAS are nasal continuous positive airway pressure (nCPAP), oral orthotics and surgery, and nCPAP is the first - line treatments. 4) The medication of narcolepsy is mainly modafinil, hydroxy butyric acid sodium and antidepressants, and the specific choosing should accord to clinical classifications. 5) The main treatments of RBD include general treatments such as avoiding triggers, insuring the safety of sleep environment, and drug therapy such as benzodiazepines and melatonin. Conclusion Evidence - based medicine can provide the best clinical evidence on sleep disorders' treatment. [ABSTRACT FROM AUTHOR]

Narcolepsy is a severe neurological sleep disorder, which is caused by the large loss of Hypocretin (Hcrt)/Orexin producing neurons in the hypothalamus in narcolepsy with cataplexy patients. It is likely the result of an autoimmune process that causes selective destruction of Hcrt neurons in the hypothalamus. It has been known that narcolepsy is associated with the human leukocyte antigens (HLA)- DR2 and HLA - DQB1"0602. Recent epidemic data have shown a robust incidence of disease onset associated with pandemic H1N1 - infection, H1N1 vaccination and streptococcus pyogenes infection. A genome-wide association variants in P2RY11 are associated with narcolepsy. Narcolepsy is associated with polymorphisms of the genes encoding T cell receptor-α (TCR -α) and tumor necrosis factor-α (TNF-α). Several cases report successful management of cataplexy with intravenous immunoglobulins at narcolepsy onset, but the effects are not persistent. However, there is no significant evidence of narcolepsy specific antibodies or T cell reactions found, and the relations with cytokines and central nervous system inflammatory cells have not been elaborated well. It is still a big challenge for further study. [ABSTRACT FROM AUTHOR]

This study aims to explore the clinical effect of venlafaxine sustained?release capsules combined with methylphenidate hydrochloride tablets on narcolepsy. Thirty - eight cases of narcoleptic patients were randomly divided into venlafaxine combined with methylphenidate hydrochloride treatment group (observation group, N = 19) and methylphenidate hydrochloride and clomipramine treatment group (control group, N = 19). After a total of 12 - week treatment, clinical curative effect and adverse drug reactions were observed in 2 groups of patients. The results showed that effective rate of the treatment for excessive daytime sleepiness (EDS) in observation group was higher than that of the control group (15/19 vs 8/19, P = 0.044), and effective rate of the treatment for cataplexy in observation group was higher than that of the control group (13/19 vs 6/19, P = 0.048). The rate of adverse drug reactions in observation group was lower than that in the control group (X2 = 8.889, P = 0.003). It was indicated that venlafaxine combined with methylphenidate had good curative effect on narcolepsy with EDS and cataplexy symptoms. [ABSTRACT FROM AUTHOR]

Narcolepsy is the most common cause of excessive daytime sleepiness (EDS) following obstructive sleep apnea. Its treatment aims to reduce EDS and cataplexy, improve nighttime sleep disturbance, sleep paralysis and sleep-related hallucinations. Pitolisant (a histamine H3 receptor antagonist) and solriamfetol (a norepinephrine reuptake inhibitor) have recently been approved effective for narcolepsy in the United States and the European Union. Pitolisant has proved to be effective for both EDS and cataplexy. Besides being effective on EDS, solriamfetol seems to have advantages in abuse potential and withdrawal syndrome. As potential treatments for EDS and cataplexy associated with narcolepsy, several new drugs are being developed and tested. These new drugs include new hydroxybutyrate preparations (controlled release sodium hydroxybutyrate FT218, low sodium hydroxybutyrate JZP-258), selective norepinephrine reuptake inhibitor (AXS-12), and modafinil combined with astroglial junction protein inhibitor (THN102). This paper reviews the recently approved drugs and potential treatments for narcolepsy.

Objective: To discusses the predictive value of typical cataplexy+ HLA-DQB1*0602 positive to hypocretin-1 (HCRT-1) reduction in cerebrospinal fluid (CSF) in patients with narcolepsy. Methods: A total of 165 narcoleptic patients, who were diagnosed at the Sleep Center of Peking University People's Hospital and Peking University International Hospital from March 2003 to March 2017, were recruited. The CSF HCRT-1 level and DQB1*0602 were measured in all the subjects. The narcoleptic patients were divided into two groups: typical cataplexy+ DQB1*0602 positive were CH group, and others who were not typical cataplexy and DQB1*0602 positive simultaneously were NCH group. The HCRT-1 level in CSF was declared to have a serious reduction when HCRT-1≤110 ng/L. According to this standard, the CH group and NCH group were subdivided into sub-groups and the data was analyzed to investigate the predictive value of typical cataplexy+ HLA-DQB1*0602 positive to HCRT-1 reduction. Results: There were 142 patients in CH group, including 137 patients with HCRT-1 reduction and 5 patients without. There were 23 patients in NCH group, including 15 patients with HCRT-1 reduction and 8 patients without. The positive predictive value of typical cataplexy+ DQB1*0602 positive for the reduction of HCRT-1 in CSF was 96.5%. Typical cataplexy+ DQB1*0602 positive had a good consistency with the HCRT-1 reduction in CSF (χ(2)=26.7, P <0.001). Conclusion: Typical cataplexy+ DQB1*0602 positive has a good predictive value to the HCRT-1 reduction in CSF in patients with narcolepsy.

Objective: To analyze the clinical features of Kleine-Levin syndrome (KLS) patients. Methods: Clinical data of 44 patients with KLS of the Sleep Center of Peking University People's Hospital from January 2002 to July 2013 were systematically reviewed. The predisposing factors and clinical presentations were summarized, and compared with the data from a Western KLS study with a large subjects number. Nocturnal polysomnography (PSG) and multiple sleep latency test (MSLT) were conducted during relapse and remission period, respectively. HLA-DQB1*0602 gene were screened and analyzed. Results: Among the 44 patients, 28(63.6%) were men and 16(36.4%) were women, with a mean age of (18.3±8.9) years old. Most patients developed the symptoms during adolescence. Infection or fever was the most common trigger for episode. The main clinical presentations were 44(100.0%) hypersomnia, 31(70.5%) forgetfulness, 26(59.1%) decreased appetites, 24(54.5%) juvenile behavior, 18(40.9%) depression, etc. Compared with the Western study, it showed that our patients had decreased instead of increased appetite. The PSG testing did not have remarkable findings. MSLT showed mean sleep latency was significantly shorter during relapse than during remission [(10.4±5.4) vs (15.3±3.4) min, P =0.009]. HLA-DQB1*0602 was positive in 12 of 40(30.0%) patients, which was similar to the data in the Chinese population. Conclusions: KLS has various clinical characteristics. The presentation of appetite may be different between Chinese and western KLS patients.