Anti-leucine-rich glioma inactivated protein-1 (LGI-1) encephalitis is a rare autoimmune disease with a varied neuropsychiatric clinical manifestation and have potential for reversal with immunotherapy. Early identification and treatment are therefore of paramount importance. We present the case of a 43-year-old man admitted for acute psychosis with facio-brachial dystonic seizures not responding to anti-epileptic drugs (AEDs). His symptoms started approximately 5 months prior admission to the hospital and associated with progressive cognitive impairment (particularly verbal and visio-spatial domains) followed by altered behavior, irrelevant talk, and disturbance in daily activities of living and facio-brachial dystonic seizures. There was history of allergic reaction to valproate, carbamazepine and levetiracetam, and serum sample tested strongly positive for LGI-1 (leucine rich glioma inactivated-1) antibodies. He was treated with injection methylprednisolone (1 gm intravenous) brief pulse therapy for 7 days and then underwent four sessions of plasma exchange followed by oral steroids and immunosuppressant therapy. Treatment was successful and patient returned to his previous baseline functioning but patient develops steroid induced hypomania and diabetes mellitus in the due course of time. Patient was discharged after successful resolution of symptoms. Patient did not come up for follow-up. He was contacted telephonically and we get to know that he relapsed after 2 weeks and consulted at higher center where he died while on treatment. Prognosis of anti-LGI-1 encephalitis is usually good but our patient died.

中文翻译：

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一例抗 LGI-1 脑炎表现为急性精神病

富含抗亮氨酸的胶质瘤灭活蛋白-1 (LGI-1) 脑炎是一种罕见的自身免疫性疾病，具有多种神经精神临床表现，并且有可能通过免疫治疗逆转。因此，早期识别和治疗至关重要。我们介绍了一名 43 岁男性因面臂肌张力障碍癫痫而入院的案例，该患者对抗癫痫药物 (AED) 无反应。他的症状在入院前大约 5 个月开始出现，并伴有进行性认知障碍（特别是语言和视觉空间领域），随后是行为改变、不相关的谈话、日常生活活动障碍和面臂肌张力障碍性癫痫发作。对丙戊酸盐、卡马西平和左乙拉西坦有过敏反应史，和血清样本的 LGI-1（富含亮氨酸的胶质瘤灭活-1）抗体呈强阳性。他接受了注射甲泼尼龙（1 gm 静脉注射）短暂脉冲治疗 7 天，然后接受了四次血浆置换，随后是口服类固醇和免疫抑制剂治疗。治疗成功，患者恢复到以前的基线功能，但患者在适当的时间发展为类固醇诱发的轻躁狂和糖尿病。患者症状成功解决后出院。病人没有来随访。我们通过电话联系了他，我们知道他在 2 周后复发，并在更高的中心就诊，他在治疗期间死亡。抗 LGI-1 脑炎的预后通常良好，但我们的患者死亡。