Moon Jiyeon, Kim Hyeyun
Department of Neurology and Sleep Medicine Research Center, International St. Mary's Hospital, Catholic Kwandong University College of Medicine, Incheon, Korea.
Encephalitis. 2022 Oct;2(4):116-120. doi: 10.47936/encephalitis.2022.00080. Epub 2022 Oct 6.
Leucine-rich glioma-inactivated 1 (LGI-1) antibody encephalitis is a type of limbic encephalitis characterized by faciobrachial dystonic seizure and short-term memory loss as initial clinical symptoms. We present a case initially misdiagnosed as schizophrenia and finally diagnosed as LGI-1 antibody encephalitis. A 41-year-old female presented to the neurology clinic with a 4-month history of anxiety and disoriented speech and a new onset headache. Her explanation of symptoms was unclear, and she was unable to answer questions properly. Her brain magnetic resonance imaging (MRI) showed no specific lesions. After 6 months, depersonalization, place disorientation and memory impairment were noted. Her symptoms continue to progress, experiencing visual/auditory hallucinations. She was diagnosed with schizophrenia and admitted to a closed psychiatric ward. In the hospital, she showed mild fever, and her memory loss worsened faster than her psychiatric symptoms, unlike in schizophrenia. Follow-up MRI scans showed a diffusely enlarged right hippocampus with a 2.5 × 1.3-cm mass lesion. Electroencephalogram showed rhythmic theta activities/interictal spikes in the right frontal lobe, for which she was treated with an antiepileptic drug. Cerebrospinal fluid analysis results showed pleocytosis. Based on this, autoimmune encephalitis was diagnosed, and steroid pulse treatment and immunoglobulin treatment were performed. Positivity for LGI-1 antibody was reported and finally led to diagnosis of LGI-1 antibody encephalitis. Clinical symptoms gradually improved, and the lesion had shrunk considerably on MRI performed 6 months after immunoglobulin treatment. She reports persistent amnesia for 6 months but has returned to her daily life under follow-up observation.
富含亮氨酸胶质瘤失活蛋白1(LGI-1)抗体脑炎是一种边缘叶脑炎,其初始临床症状为面臂肌张力障碍性癫痫发作和短期记忆丧失。我们报告一例最初被误诊为精神分裂症,最终被诊断为LGI-1抗体脑炎的病例。一名41岁女性因焦虑、言语定向障碍4个月及新发头痛就诊于神经内科门诊。她对症状的解释不清楚,无法正确回答问题。她的脑部磁共振成像(MRI)未显示特异性病变。6个月后,出现人格解体、地点定向障碍和记忆障碍。她的症状持续进展,出现视幻觉/听幻觉。她被诊断为精神分裂症并入住封闭式精神科病房。在医院里,她有低热,与精神分裂症不同,她的记忆丧失比精神症状恶化得更快。后续MRI扫描显示右侧海马弥漫性增大,有一个2.5×1.3厘米的肿块病变。脑电图显示右侧额叶有节律性θ活动/发作间期棘波,为此她接受了抗癫痫药物治疗。脑脊液分析结果显示有细胞数增多。基于此,诊断为自身免疫性脑炎,并进行了类固醇冲击治疗和免疫球蛋白治疗。报告显示LGI-1抗体呈阳性,最终确诊为LGI-1抗体脑炎。临床症状逐渐改善,免疫球蛋白治疗6个月后进行的MRI显示病变已明显缩小。她自述持续失忆6个月,但在随访观察下已恢复日常生活。