Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, China.
Department of Orthopedics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, China.
BMC Neurol. 2021 Nov 27;21(1):461. doi: 10.1186/s12883-021-02460-w.
Autoimmune encephalitis (AE) with multiple auto-antibodies is of great clinical significance because its complex clinical manifestations and atypical imaging increase the difficulty of diagnosis, differential diagnosis and treatment, which may aggravate the disease, increase the recurrence rate and mortality. The coexistence of anti-Leucinie-rich Glioma Inactivated 1 (LGI1) and anti-γ-aminobutyric acid-beta-receptor 1 (GABAR1) has not been published before.
We herein present the case of a 60-year-old man with slow response, behavioral changes, psychosis and sleep disorders. Laboratory test included serum hyponatremia, positive serum LGI1 and GABAR1 antibodies using transfected cell-based assays. Electroencephalogram exhibited moderate diffusion abnormality. The patient responded well to steroid impulse treatment and sodium supplement therapy, and did not recur during the follow-up.
Here we report the first AE characterized by positive LGI1 and GABAR1 antibodies, as well as summarizing AE with multiple auto-antibodies reported so far, hopefully to provide experience for clinical practice.
伴多种自身抗体的自身免疫性脑炎(AE)具有重要的临床意义,因为其复杂的临床表现和非典型的影像学增加了诊断、鉴别诊断和治疗的难度,可能会加重病情、增加复发率和死亡率。抗亮氨酸丰富胶质瘤失活 1 型(LGI1)和抗γ-氨基丁酸-β-受体 1(GABAR1)共存以前尚未有报道。
我们在此介绍了一例 60 岁男性患者,其表现为反应迟钝、行为改变、精神病和睡眠障碍。实验室检查包括血清低钠血症、转染细胞检测的阳性血清 LGI1 和 GABAR1 抗体。脑电图显示中度弥散异常。患者对类固醇冲击治疗和钠补充治疗反应良好,随访期间未复发。
我们在此报告首例以 LGI1 和 GABAR1 抗体阳性为特征的 AE,并总结了迄今为止报道的伴有多种自身抗体的 AE,希望为临床实践提供经验。