1型发作性睡病的免疫治疗进展  

Immunotherapy advances for type 1 narcolepsy

作　　者：张慧敏 詹淑琴[1] ZHANG Hui-min;ZHAN Shu-qin(Department of Neurology.Xuaneu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院神经内科,北京100053

出　　处：《中国实用内科杂志》2024年第10期810-816,共7页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金(82371489);国家重点研发计划项目(2021YFC2501400)。

摘　　要：发作性睡病是一种罕见的睡眠障碍,分为1型和2型,其中1型的特征是分泌下丘脑分泌素的神经元大量丢失。在过去的二十年里,在发作性睡病的自身免疫机制和遗传易感性研究方面取得了重大进展,特别是其与HLADQB 1*06∶02和其他非人类白细胞抗原(HLA)免疫基因的强相关性。目前治疗方法主要是对症治疗,免疫疗法也开始不断尝试旨在通过调节免疫反应并减少对下丘脑泌素神经元的攻击,达到解决病因的目的。其中静脉注射免疫球蛋白、皮质类固醇和单克隆抗体等治疗方法已显示出潜在的治疗价值,但大多数研究为个案报道,其长期有效性仍有待进一步研究。Narcolepsy,a rare sleep disorder,is divided into type 1 and type 2,with type 1 distinguished by the significant loss of hypocretin-secreting neurons in the hypothalamus.Significant advancements have been made in the past two decades in understanding the autoimmune mechanisms and genetic predispositions of narcolepsy,particularly its strong linkage with HLA-DQB1*06:02 and other non-HLA immune genes.Present treatment modalities focus primarily on symptom management,whilst trials in immunotherapy are emergently being explored aiming to modulate immune responses and diminish attacks on hypocretin-producing neurons,thereby addressing the etiology of the condition.Treatment avenues such as intravenous immunoglobulin,corticosteroids,and monoclonal antibodies have demonstrated potential therapeutic promise;however,most are derived from case reports,and their long-term efficacy remains to be further investigated in comprehensive studies.

关 键 词：1型发作性睡病 下丘脑分泌素 免疫疗法 

分 类 号：R741[医药卫生—神经病学与精神病学]