Take you to know the latest trends of international guidelines for narcolepsy and new breakthrough treatment schemes.
On September 24-25, the 24th National Academic Conference on Neurology of Chinese Medical Association (NCN 202 1) was successfully held in our city.
Narcolepsy is an internationally recognized rare disease [1], but we find that most clinical experts and doctors are not familiar with it. With the deepening of international clinical research in this field, the international guidelines have been revised and updated accordingly. In 20021year, the European Society of Neurology (ean), the European Society for Sleep Research (ESRS) and the European Cooperative Group for Narcolepsy (EU-NN) jointly issued the European guidelines for narcolepsy, which provided the latest clinical recommendations for the treatment of this disease based on evidence-based medicine.
Domestic related research is also increasing year by year. During this annual meeting of neurology, many experts submitted special reports and papers on narcolepsy. Among them, Professor Wu Huijuan's "Overall Regulation of Sleep-Awakening Rhythm of Narcolepsy" and Professor Wang Zan's "Evaluation of Sleep Quality and Daytime Functional Status of Narcolepsy Patients" aroused enthusiastic response.
On September 25th, in the academic topic, Professor xuanwu hospital Wang Yuping of Capital Medical University was the chairman, and Professor Yu Huan of Huashan Hospital affiliated to Fudan University was the keynote speaker, and the 20021European Guidelines for the Treatment of Narcolepsy in Adults and Children (hereinafter referred to as "20021European Guidelines") was interpreted in detail.
hypnosia
A serious and rare sleep disorder.
Professor Yu Huan pointed out that narcolepsy is an internationally recognized rare disease [1]. Because of its extremely low incidence, hidden onset and atypical symptoms, clinicians do not know enough about it, which is easy to cause missed diagnosis and misdiagnosis [2-3]. In addition, narcolepsy * * * has a higher risk of other diseases, and the probability of being complicated with mental illness, digestive system disease and nervous system disease is 3.8 times, 2.7 times and 3.7 times that of the normal population respectively [4-5], among which about 29.4%-5 1.4% of narcolepsy patients are complicated with obstructive sleep apnea syndrome (OSA).
Narcolepsy can significantly reduce the health-related quality of life (HRQL) of patients [7-8], and at the same time, the hospitalization risk of motor vehicle accidents of narcolepsy patients is 6.725 times that of the general population [8]. Narcolepsy seriously affects the quality of life of patients and brings heavy disease burden to individuals and society. In view of this, domestic and foreign guidelines are constantly innovating to provide guidance for standardized treatment of narcolepsy.
Updating the background of narcolepsy guidelines
Guidelines for the treatment of narcolepsy were first published in Europe in 2006 and in the United States in 2007. In 20 14, the American academy of sleep medicine revised the diagnostic criteria of narcolepsy when it updated the international classification standard of sleep diseases (ICSD-3 in 20 14), and it has been used ever since. Some researchers have suggested that the current diagnostic criteria have some difficulties in clinical implementation, which leads to differences between clinical classification and pathological classification. European guideline 202 1 puts forward some suggestions on the operation of clinical diagnosis of narcolepsy.
202 1 European guidelines and key points of treatment arrangement
European guidelines for narcolepsy 202 1 points out that the treatment methods of narcolepsy mainly include non-drug treatment and drug treatment. In the aspect of non-drug treatment, the Guide recommends regular daytime siesta, which is helpful to improve the symptoms of narcolepsy patients. In terms of drug therapy, it is suggested to use a variety of drugs for different symptoms and people [9].
Figure 1: 202 1 European guidelines for narcolepsy.
■ Excessive daytime sleepiness
The guide recommends modafinil, tirolison, sodium hydroxybutyrate, Solriamfetol, methylphenidate, etc. It can be used to treat daytime sleepiness (EDS) symptoms of adult narcolepsy patients. However, it is worth noting that methylphenidate, modafinil, amphetamine and Solriamfetol have the risk of increasing heart rate and blood pressure, so it is necessary to follow up and monitor cardiovascular risk factors and provide corresponding treatment [9].
Figure 2. Suggestions on drug treatment of adult narcolepsy.
The guide recommended Tirolison (with strong recommendation and moderate evidence quality), which was mainly based on the Harmony phase study. During the treatment of 12 months, the Epwoth sleep scale (ESS) score of patients can be reduced whether tirolison is used alone or in combination with other drugs. When the newly diagnosed patients were treated with tirol alone, the ESS score decreased the most (6.51.4) [10].
■ disaster
60%-70% of narcolepsy patients have cataplexy, and the guidelines recommend the use of sodium hydroxybutyrate, SSRI-NSRI- other antidepressants, tricyclic antidepressants and Tylenol to treat adult narcolepsy cataplexy [9].
Among them, Tirolison was approved by the US Food and Drug Administration (FDA) in 2020 for cataplexy treatment of narcolepsy, and was awarded the qualification of breakthrough treatment.
■ Sleep paralysis, pre-sleep/pre-awakening hallucinations
About 50% narcolepsy patients show sleep paralysis (SP) and hallucinations before falling asleep/waking up (HH). The guideline suggests that patients with narcolepsy should be treated with sodium hydroxybutyrate, tyronisone and antidepressants before they fall asleep/wake up [9].
■ Children patients
The initial symptoms of more than 50% narcolepsy patients occurred before 18 years old. The symptoms of narcolepsy in children and adolescents are different from those in adults, and the treatment strategies of narcolepsy are also different from those in adults. The drugs recommended in the Guidelines for the Treatment of Children's Narcolepsy are: sodium hydroxybutyrate, methylphenidate, modafinil, antidepressants, tyronisone and so on. Children need to pay special attention to safety, risk-benefit ratio and other factors in the treatment process [9].
summary
At the end of the interpretation of the guide, Professor Yu Huan concluded that narcolepsy has seriously affected the quality of life of patients and caused a lot of burden to individuals, society and economy. There are many clinical manifestations of narcolepsy, and the individual differences of patients are also very obvious. Therefore, clinicians need to make personalized treatment plans for each patient. At the same time, Professor Wang Yuping also summarized this academic topic, pointing out that replacing Luo Song is a brand-new attempt in the history of drug treatment for narcolepsy, with a wide range of effects, which can not only improve the degree of wakefulness, but also resist cataplexy, improve the symptoms of patients with nocturnal sleep disorder, and provide new hope for narcolepsy patients.
Tirofisen, as an innovative drug to treat narcolepsy, can effectively improve the four symptoms of EDS, cataplexy, hallucination and sleep paralysis, and is highly safe and easily tolerated by patients. Therefore, the European guidelines for narcolepsy 202 1 recommended tiroxacin as first-line monotherapy for adult narcolepsy EDS and cataplexy, and for children narcolepsy EDS [9].
It is expected that in the near future, more innovative treatment schemes in the field of narcolepsy will enter China, enabling clinicians to use drugs within the scope corresponding to the indications, and more China patients will benefit.
References:
[1]Khatami R, Luca G, Baumann CR, etc. European narcolepsy network (EU-NN) database. J. Sleep Resolution 20 16; 25(3):356-364.
Zhang, Zhang. Research progress of narcolepsy [J]. Journal of Stroke and Neurology, 2019,36 (7): 594-596.
Xu Qinglin, Lou, et al. Progress in drug treatment of narcolepsy [J]. Journal of Zhejiang University (Medical Edition), 2020,49 (4): 419-424.