Department of Neurology, Tongji Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Neurology, Department of Pneumology, No. 9 Hospital of Wuhan City, Wuhan, China.
Eur Neurol. 2022;85(6):437-445. doi: 10.1159/000524974. Epub 2022 Jul 27.
Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is clinically heterogeneous, especially at presentation, and though it is sometimes found in association with tumor, this is by no means the rule.
Clinical data for 10 patients with anti-LGI1 encephalitis were collected including one case with teratoma and nine cases without and compared for clinical characteristics. Microscopic pathological examination and immunohistochemical assay of the LGI1 antibody were performed on teratoma tissue obtained by laparoscopic oophorocystectomy.
In our teratoma-associated anti-LGI1 encephalitis case, teratoma pathology was characterized by mostly thyroid tissue and immunohistochemical assay confirmed positive nuclear staining of LGI1 in some tumor cells. The anti-LGl1 patient with teratoma was similar to the non-teratoma cases in many ways: age at onset (average 47.3 in non-teratoma cases); percent presenting with rapidly progressive dementia (67% of non-teratoma cases) and psychiatric symptoms (33%); hyponatremia (78%); normal cerebrospinal fluid results except for positive LGI1 antibody (78%); bilateral hippocampal hyperintensity on magnetic resonance imaging (44%); diffuse slow waves on electroencephalography (33%); good response to immunotherapy (67%); and mild residual cognitive deficit (22%). Her chronic anxiety and presentation with status epilepticus were the biggest differences compared with the non-teratoma cases.
In our series, anti-LGI1 encephalitis included common clinical features in our series: rapidly progressive dementia, faciobrachial dystonic seizures, behavioral disorders, hyponatremia, hippocampal hyperintensity on magnetic resonance imaging, and residual cognitive deficit. We observed some differences (chronic anxiety and status epilepticus) in our case with teratoma, but a larger accumulation of cases is needed to improve our knowledge base.
抗亮氨酸丰富胶质瘤失活 1 型（LGI1）脑炎临床表现具有异质性，尤其是在首发时，虽然它有时与肿瘤有关，但这绝不是普遍现象。
收集了 10 例抗 LGI1 脑炎患者的临床资料，其中 1 例伴畸胎瘤，9 例无畸胎瘤，并对其临床特征进行了比较。对腹腔镜卵巢囊肿切除术获得的畸胎瘤组织进行了 LGI1 抗体的组织病理学检查和免疫组化检测。
在我们的畸胎瘤相关抗 LGI1 脑炎病例中，畸胎瘤的病理特征主要为甲状腺组织，免疫组化检测证实部分肿瘤细胞的 LGI1 核染色阳性。该例抗 LGl1 脑炎伴畸胎瘤患者在许多方面与非畸胎瘤病例相似：发病年龄（非畸胎瘤病例平均 47.3 岁）；快速进展性痴呆的发生率（67%）和精神症状（33%）；低钠血症（78%）；脑脊液除 LGI1 抗体阳性外均正常（78%）；磁共振成像双侧海马高信号（44%）；脑电图弥漫性慢波（33%）；免疫治疗反应良好（67%）；轻度认知缺陷残留（22%）。与非畸胎瘤病例相比，她的慢性焦虑和癫痫持续状态是最大的区别。
在我们的系列中，抗 LGI1 脑炎包括常见的临床特征：快速进展性痴呆、面臂肌张力障碍性发作、行为障碍、低钠血症、磁共振成像双侧海马高信号、认知缺陷残留。我们观察到畸胎瘤病例存在一些差异（慢性焦虑和癫痫持续状态），但需要积累更多的病例来提高我们的知识库。
