TY -的T1 -摩根富林明anti-IgLON5疾病的临床表现,神经学乔-神经病学SP - 1736 LP - 1743 - 10.1212首页 / WNL。0000000000003887六世- 88 - 18盟卡莱斯Gaig AU -弗朗西斯克格劳盟Yarko Compta盟Birgit Hogl AU -路易斯Bataller盟诺伯特•Bruggemann AU -卡洛琳Giordana盟-安娜Heidbreder盟Katya Kotschet AU - Jan Lewerenz盟Stefan大人物AU -玛丽亚·j·马蒂盟特蕾莎修女Montojo AU -耶稣Perez-Perez盟Inmaculada门Simabukuro盟盟-卡斯帕塞茨盟Mateus - Nieves的泰雷兹盟Klaus-Peter扫读非盟-亚历克斯Iranzo盟瓜达卢佩Ercilla盟——莉迪亚萨瓦特AU -琼Santamaria盟约瑟Dalmau Y1 - 2017/05/02 UR - //www.ez-admanager.com/content/88/18/1736.abstract N2 -目的:报告表示,主要症状,人类白细胞抗原(HLA)协会和免疫球蛋白G(免疫球蛋白)子类anti-IgLON5疾病:与异态睡眠障碍,睡眠呼吸暂停,IgLON5抗体。首页方法:这是22名患者的回顾性临床分析。免疫球蛋白子类决心使用报告技术。结果:病人的平均年龄是64岁(范围46 - 83)。症状,导致最初的咨询包括睡眠问题(8例;36%)、步态异常(8;36%),球功能障碍(3;14%)、舞蹈病(2;9%),认知能力下降(1;5%)。诊断的障碍的时候,4综合症被确定:(1)与深眠状态和睡眠睡眠障碍8例(36%)患者的呼吸困难; (2) a bulbar syndrome including dysphagia, sialorrhea, stridor, or acute respiratory insufficiency in 6 (27%); (3) a syndrome resembling progressive supranuclear palsy (PSP-like) in 5 (23%); and (4) cognitive decline with or without chorea in 3 (14%). All patients eventually developed parasomnia, sleep apnea, insomnia, or excessive daytime sleepiness. HLA-DRB1*10:01 and HLA-DQB1*05:01 were positive in 13/15 (87%) patients; the DRB1*10:01 allele was 36 times more prevalent than in the general population. Among 16 patients with paired serum and CSF samples, 14 had IgLON5 antibodies in both, and 2 only in serum (both had a PSP-like syndrome). Twenty of 21 patients had IgG1 and IgG4 antibodies; the latter predominated in 16.Conclusions: Patients with IgLON5 antibodies develop a characteristic sleep disorder preceded or accompanied by bulbar symptoms, gait abnormalities, oculomotor problems, and, less frequently, cognitive decline. IgG4 subclass antibodies predominate over IgG1; we confirm a strong association with the HLA-DRB1*10:01 allele.CBA=cell-based assay; CI=confidence interval; HLA=human leukocyte antigen; IgG=immunoglobulin G; MSA=multiple system atrophy; NREM=non-REM; OSA=obstructive sleep apnea; PSG=polysomnography; PSP=progressive supranuclear palsy ER -