PT -期刊文章非盟-约翰·布鲁克斯AU -皮埃尔Bourque AU -哈罗德·阿特金斯AU -格雷戈里天盟卡冲TI -自体造血干细胞移植的病例报告相关Anti-IgLON5脑炎患者(2726)DP - 2020年4月14日TA -神经病学PG - 2726 VI - 94 IP - 15补充4099 - //www.ez-admanager.com/content/94/15_Supplement/2726.short 4100 - //www.ez-admanager.com/content/94/15_Supp首页lement/2726.full所以Neurology2020 4月14日;94 AB -目的:NABackground:抗体针对IgLON5粘附分子已被证明通常伴随睡眠障碍的综合征包括阻塞性睡眠障碍性呼吸模式和运动障碍,可以模仿进行性核上的麻痹。神经异常等现象,超兴奋性和神经精神障碍普遍出现在受影响的人口。设计/方法:NAResults: 47岁的绅士与进步的夜间呼吸困难、吞咽困难、自发性收缩、痉挛和温和的帕金森病提出了评估和治疗在渥太华医院在渥太华,加拿大安大略省。他被诊断出患有anti-IgLON5脑炎与抗体血清(Weislab、瑞典)中找到。测试65迦得、VGKC CASPR2, LGI1, NMDAR,甘氨酸抗体-就像肯尼迪疾病基因检测。病人的临床状态恶化,尽管治疗类固醇,丙种球蛋白、血浆置换、咪唑硫嘌呤和利妥昔单抗,最终他接受自体造血干细胞移植(AHSCT)。他容忍过程几乎没有并发症包括轻度transaminitis,两集的肺炎不需要住院治疗,和粘膜炎导致暂时性的减少口服摄入。程序后,他似乎与一般的主观改善临床稳定包括那些关于他的睡眠。几个症状持续要求持续减轻治疗尤其是对于胃肠道症状和肌肉痉挛。结论:知识anti-IgLON5光谱的相关疾病正在进化。 Although some patients have been shown to stabilize with conventional therapy, a tendency toward treatment resistance and associated brain tau accumulation driving an inexorable neurodegenerative process suggest that potent and early therapy may be paramount to patient survival. The presented case exemplifies that bone marrow ablation with AHSCT may be a viable option to halt and even reverse the progression of life-threatening symptoms in IgLON5 associated encephalitis. To our knowledge this is the first patient with IgLON5 associated disease for whom AHSCT was provided.Disclosure: Dr. Brooks has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Actelion. Dr. Brooks has received research support from Biogen Idec Canada, EMD Serono, Sanofi Genzyme, Novartis Pharmaceuticals Canada Inc., Hoffmann-La Roche, and Teva Canada Innovation. Dr. Bourque has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Akcea Pharmaceuticals. Dr. Atkins has nothing to disclose. Dr. Day has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with DynaMed%20(EBSCO%20Health), Parabon Nanolabs. Dr. Day holds stock and/or stock options in ANI Pharmaceuticals (a generic pharmaceutical company).Dr. Rush has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Roche, EMD Serono, Sanofi Genzyme, Biogen Idec, Teva, Novartis.