Narcolepsy（NP） is a rare central sleep disorder， and it is categorized into narcolepsy type1 （NT1） and narcolepsy type2（NT2）.The main clinical symptoms of NP include daytime lethargy， cataplexy induced by emotions， and sleep paralysis， with a peak onset during adolescence （with an age of 8‒12 years）. At present， the pathogenesis of NP remains unclear and is associated with various factors such as human leucocyte antigen （HLA）， infection， and epigenetic silencing， and the selective loss of hypocretin neurons in the hypothalamus is the key pathophysiological mechanism of NP. Pharmacological intervention is currently the main treatment method， and diagnostic delay can be as long as more than 10 years， which not only affects the social activities， academic performance， and work of patients， but also leads to mental health issues such as anxiety and depression. This article reviews the latest research findings in the epidemiology， pathogenesis， and treatment of NT1 and clarifies the deficiencies and controversies in current research， so as to provide new ideas and directions for subsequent studies.
Narcolepsy ; Cataplexy

Objective The efficacy and safety of pitolisant in the treatment of adult narcolepsy have been confirmed in clinical trials abroad， but there is a lack of data on the application of pitolisant in Chinese patients. The aim of this study is to investigate the efficacy and safety of pitolisant in the treatment of adult narcolepsy in China.Methods A total of 30 subjects were enrolled in this study and were given individualized titration once a day for 8 weeks. Epworth Somnolence Scale （EES） and daily cataplexy rate （DCR） were used as the primary outcome measures，and Clinical Global Impression-Severity scale（CGI-S） and Clinical Global Impression of Change Scale （CGI-C） were used as the secondary outcome measures；safety indicators included adverse event （AE） records and laboratory examination.Results The results showed that there were significant improvements in ESS score and number of cataplexy attacks after medication. At the end of treatment，ESS score was reduced by（7.63±4.79）（P<0.001）. For all subjects， the average daily number of cataplexy attacks was 0.83 at baseline，which was reduced significantly to （0.39±0.82）after 3 weeks of pitolisant treatment and（0.38±0.79）after 5 weeks of pitolisant treatment（P<0.05）. After the treatment ended， the median CGI-S score of excessive daytime sleepiness （EDS） improved from "severe" at baseline to "mild"（P<0.001）， and the CGI-S score of cataplexy improved from "mild" at baseline to "basically normal"（P<0.001）. Most AEs were mild and did not receive any drug treatment， and there were no significant changes in other laboratory markers used to monitor the general condition of the subjects before and after treatment. Conclusion Pitolisant has good efficacy and safety in the treatment of EDS and cataplexy in Chinese adults with narcolepsy.
Narcolepsy ; Histamine ; Cataplexy

No abstract available.
Narcolepsy*

No abstract available.
Narcolepsy*

Narcolepsy is a serious, lifelong, disabling disorder characterized by a tetrad of symptoms including excessive daytime sleepiness, cataplexy, hypnagogic hallucination, and sleep paralysis. We experienced a 14 year old boy with a narcolepsy diagnosed by typical clinical symptoms above mentioned and multiple sleep latency test. A brief review of the related literatures was also made.
Adolescent ; Cataplexy ; Hallucinations ; Humans ; Male ; Narcolepsy* ; Sleep Paralysis

Humans ; Cataplexy/drug therapy* ; Sleep, REM ; Dopamine ; Narcolepsy/drug therapy*

Objective Narcolepsy type 1 （NT1） is known to be associated with low levels of hypocretin-1 （Hcrt-1） in cerebrospinal fluid （CSF）. The standard method for Hcrt-1 measurement is radioimmunoassay （RIA） with imported reagents， but this antibody-dependent method is limited to radiation safety-certified lab， gradual radioactivity degradation， and slow turn-around time. The purpose of this study is to explore a non-radioactive， faster， and antibody independent domestic method in China for Hcrt-1 detection. Methods Repeated testing of cerebrospinal fluid from 14 clinically diagnosed NT1 patients and 10 non-narcolepsy patients was performed using liquid chromatography-tandem mass spectrometry （LC-MS/MS）technology，including the establishment and optimization of fundamental methodological procedures. The main steps involved the addition of non-radioactive isotope-labeled internal standards to the cerebrospinal fluid， followed by solid-phase extraction， mass spectrometry signal acquisition， and quantitative analysis. The results were then compared with the corresponding radioimmunoassay（RIA） findings. Results The LC-MS/MS method showed faster speed， and good linearity across a wider range of synthesized standard（5~2 500 pg/ml）， and good repeatability. Although this absolute-quantitation-based LC-MS/MS method and RIA method have different reading values in Hcrt-1 quantitation， they both can segregate NT1 group from non-NT1 group well. Conclusion Although larger cohorts are needed to set up a standard method in China，LC-MS/MS method is proved to be an easier， safer， faster， and possibly more accurate method for Hcrt-1 quantitation and detection for NT1 diagnosis.
Narcolepsy ; Radioimmunoassay

Narcolepsy with cataplexy (NC) is associated with hypocretin deficiency, and is thought to be an autoimmunity condition. The mean age at onset is estimated to be in the early 20s. Recent papers have addressed the response to immunotherapies in NC, with challenging results. We report a case of late-onset NC in a patient who did not benefit from early intravenous high-dose immunoglobulin (IVIg) therapy. This is the first reported attempt at using IVIg to treat an NC patient in Korea.
Autoimmunity ; Cataplexy ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Immunotherapy ; Intracellular Signaling Peptides and Proteins ; Korea ; Narcolepsy ; Neuropeptides ; Orexins

Childhood narcolepsy is one of the underdiagnosed diseases even the first symptoms often appear in childhood. Making diagnosis through history taking is not always easy because the symptoms of childhood narcolepsy are different from those of adulthood. Diagnostic laboratory tests such as sleep studies, tests for human leukocyte antigens, cerebrospinal fluid hypocretin measurement should be considered when the child has excessive daytime sleepiness without cataplexy. Treatment approach should be start as early as possible to avoid secondary academic, emotional difficulties. Both pharmacological and non-pharmacological management, and close cooperation between parents and school teachers should be maintained. In the near future, childhood narcolepsy can be a key to understand the pathogenesis of narcolepsy.
Cataplexy ; Child ; HLA Antigens ; Humans ; Intracellular Signaling Peptides and Proteins ; Narcolepsy ; Neuropeptides ; Parents ; Orexins

A 5-year-old, castrated male, Maltese was presented with history of acute flaccid paralysis. The dog was presented with sudden loss of muscle tone and involuntary movements of hind limbs. Neurologic examination revealed reduced postural reaction in the bilateral hind limbs. MRI of brain showed moderate hydrocephalus, but other examination results were normal. Based on the characteristic episodes and examination results, canine cataplexy was suspected. Treatment was initiated with clomipramine as cataplexy control. Clinical signs resolved with 3-month medication. This case demonstrates therapeutic diagnosis of cataplexy. To the author's knowledge, this is the first report of cataplexy treating with clomipramine.
Animals ; Brain ; Cataplexy ; Clomipramine ; Dogs ; Dyskinesias ; Extremities ; Humans ; Hydrocephalus ; Male ; Muscles ; Narcolepsy ; Neurologic Examination ; Paralysis