抗 LGI1 脑炎伴发 IgLON5 抗体：临床特征和人类白细胞抗原单倍型。
Anti-LGI1 Encephalitis With Co-occurring IgLON5 Antibodies: Clinical Features and Human Leukocyte Antigen Haplotypes.
机构信息
From the Neurology Department (P.S., M.L.F., E.C., L.P.-M.), University Hospital of Clermont-Ferrand; French Reference Center for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (S.M.-C., L.D.D., V.R., J.H.), Hospices Civils de Lyon; MeLiS - UCBL-CNRS UMR 5284 - INSERM U1314 (S.M.-C., L.D.D., V.R., J.H.), Université Claude Bernard Lyon 1, France; Stanford Center for Sleep Sciences and Medicine (S.M.-C.), Stanford University, Palo Alto, CA; Sleep and EEG (M.L.F.), Neurophysiology Unit, University Hospital and UMR 6602- Université Clermont Auvergne, CNRS, Institute Pascal; and UMR 1107 Inserm (L.P.-M.), Neuro-Dol, Clermont-Ferrand, France.
出版信息
Neurol Neuroimmunol Neuroinflamm. 2023 May 22;10(4). doi: 10.1212/NXI.0000000000200126. Print 2023 Jul.
OBJECTIVES
Autoimmune encephalitis (AE) with antibodies against LGI1 and IgLON5 are clinically distinctive but share some particularities such as a strong association with specific human leukocyte antigen (HLA) class II alleles.
METHODS
We clinically describe a patient with double positivity for LGI1 and IgLON5 antibodies. In addition, we conducted specific immunodepletion with the patient's serum and HLA typing and investigated the presence of serum IgLON5 antibodies in a cohort of 23 anti-LGI1 patients carrying the HLA predisposing for anti-IgLON5 encephalitis.
RESULTS
A 70-year-old woman with a history of lymphoepithelial thymoma presented with subacute cognitive impairment and seizures. Investigations included MRI and EEG showing medial temporal involvement, increased CSF protein content, and polysomnography with REM and non-REM motor activity, along with obstructive apnea. Neural antibody testing revealed both LGI1 and IgLON5 antibodies in serum and CSF, and serum immunodepletion ruled out cross-reactivity. The patient carried DRB107:01 and DQA101:01∼DQB105:01, but no other IgLON5-positive case was identified in a cohort of anti-LGI1 patients carrying DQA101∼DQB1*05. Nearly full therapeutic response was obtained after intensified immunosuppressive treatment.
DISCUSSION
We present a case of anti-LGI1 encephalitis with concomitant IgLON5 antibodies. Co-occurring IgLON5 antibodies in anti-LGI1 encephalitis are exceptional, but may appear in genetically predisposed individuals.
目的
针对 LGI1 和 IgLON5 抗体的自身免疫性脑炎（AE）在临床上具有独特性，但存在一些共同特征，如与特定人类白细胞抗原（HLA）Ⅱ类等位基因密切相关。
方法
我们临床描述了一位同时存在 LGI1 和 IgLON5 抗体双阳性的患者。此外，我们用患者的血清进行了特异性免疫耗竭、HLA 分型，并在携带易患抗 IgLON5 脑炎的 HLA 的 23 例抗 LGI1 患者队列中检测了血清 IgLON5 抗体的存在。
结果
一位 70 岁女性，有淋巴上皮胸腺瘤病史，表现为亚急性认知障碍和癫痫。检查包括 MRI 和 EEG 显示内侧颞叶受累、脑脊液蛋白含量增加、多导睡眠图显示 REM 和非 REM 运动活动以及阻塞性呼吸暂停。神经抗体检测显示血清和脑脊液中均存在 LGI1 和 IgLON5 抗体，血清免疫耗竭排除了交叉反应。患者携带 DRB107:01 和 DQA101:01∼DQB105:01，但在携带 DQA101∼DQB1*05 的抗 LGI1 患者队列中未发现其他 IgLON5 阳性病例。强化免疫抑制治疗后获得了几乎完全的治疗反应。
讨论
我们报告了一例同时存在抗 LGI1 脑炎和 IgLON5 抗体的病例。抗 LGI1 脑炎中同时存在 IgLON5 抗体较为罕见，但可能出现在遗传易感个体中。
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