文摘
摘要目的:描述的临床特征和抗体曲目thymoma-associated自身免疫性脑炎。
背景:胸腺瘤是最常见的肿瘤之一,与自身免疫性脑炎;的频率神经表面抗体(NS-ab)是未知的。
设计/方法:回顾性评估50胸腺瘤患者和疑似自身免疫性脑炎研究在医院诊所,大学的巴塞罗那,2011年1月至2020年9月。NS-ab和onconeuronal抗体测定大鼠大脑免疫组织化学,内部细胞化验,和商业免疫印迹分析。
结果:NS-ab被发现在31/50(62%)的病人。与19 NS-ab消极的病人相比,那些NS-ab更有可能有边缘脑炎(20% vs 5%), Morvan综合症(14% vs 5%),癫痫发作(55% vs 25%),多病灶的cortico-subcortical天赋MRI异常(43% vs 25%)和临床复发(27% vs 5%) (p < 0 05)。其他临床特征没有显著不同:neuromyotonia (7% vs 10%), stiff-person综合征/烫发(9% vs 5%),或肌无力(36% vs 42%)。GABAaR抗体是最普遍的(12例)。其他NS-ab包括:4 LGI-1;2 AMPAR;1 CASPR2;1 NMDAR;迦得和并发2 GlyR (1);1 AQP4; 2 LGI1 + CASPR2; 1 GABAaR + LGI1; 1 GABAaR + AMPAR; and 1 CASPR2 + AMPAR + GABAbR; in the remaining 3 patients the identity of the antigens was unknown. Antibodies against onconeuronal antigens (2 CRMP5, 1 Hu, 1 Ma2) were only identified in the group of patients with NS-ab. Age, sex, tumor characteristics, inflammatory CSF, EEG findings, treatments, and outcome were similar in NS-ab positive and negative patients (not shown).
结论:在胸腺瘤和自身免疫性脑炎患者中,62%有NS-ab。最普遍的抗体GABAaR,通常与癫痫发作有关,多病灶的天赋MRI异常。多个并发NS-Ab或onconeuronal抗体发生在抗体阳性病例的26%。
披露:3月Guasp没有披露。马丁斯博士没有披露。乔恩·兰达没有披露。莉迪亚萨瓦特没有披露。格劳博士没有披露。Dalmau博士已经收到个人薪酬在10000 - 49999美元的范围作为一个编辑,副主编,或为美国神经病学学会编辑顾问委员会成员。首页立即家人Dalmau博士已收到个人薪酬在5000 - 9999美元的范围作为一个编辑,副主编,或编辑顾问委员会成员Springer自然。立即家人Dalmau博士已收到个人薪酬在5000 - 9999美元的范围作为拉姆杜根&穆雷LLP)的专家证人。Dalmau博士的机构收到圣人疗法的研究支持。Dalmau博士的机构已经接到爱德蒙·j·莎拉基础研究支持。 The institution of Dr. Dalmau has received research support from La Caixa Foundation. The institution of Dr. Dalmau has received research support from Spanish Ministry of Health (ISCIII). The institution of Dr. Dalmau has received research support from Department of Health, Government of Catalunya. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. An immediate family member of Dr. Dalmau has received publishing royalties from a publication relating to health care.
