Li Xuanting, Yuan Junliang, Liu Lei, Hu Wenli
Department of Neurology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, 100020, China.
Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, China.
BMC Neurol. 2019 Feb 7;19(1):19. doi: 10.1186/s12883-019-1251-4.
Anti leucine-rich glioma inactivated 1 (LGI1) encephalitis is a rare autoimmune encephalitis (AE), characterized by acute or subacute cognitive impairment, faciobrachial dystonic seizures, psychiatric disturbances and hyponatremia. Antibody-LGI 1 autoimmune encephalitis (anti-LGI1 AE) has increasingly been recognized as a primary autoimmune disorder with favorable prognosis and response to treatment.
Herein, we reported a male patient presenting as rapidly progressive dementia and hyponatremia. He had antibodies targeting LGI1 both in the cerebrospinal fluid and serum, which demonstrated the diagnosis of typical anti-LGI1 AE. The scores of Mini-Mental State Examination and Montreal Cognitive Assessment were 19/30 and 15/30, respectively. Cranial magnetic resonance images indicated hyperintensities in bilateral hippocampus. The findings of brain arterial spin labeling and Fluorine-18-fluorodeoxyglucose positron emission tomography showed no abnormal perfusion/metabolism. After the combined treatment of intravenous immunoglobulin and glucocorticoid, the patient's clinical symptoms improved obviously.
This case raises the awareness that a rapid progressive dementia with predominant memory deficits could be induced by immunoreactions against LGI1. The better recognition will be great importance for the early diagnosis, essential treatment, even a better prognosis.
抗富含亮氨酸胶质瘤失活1（LGI1）脑炎是一种罕见的自身免疫性脑炎（AE），其特征为急性或亚急性认知障碍、面臂肌张力障碍性癫痫发作、精神障碍和低钠血症。抗体-LGI 1自身免疫性脑炎（抗LGI1 AE）越来越被认为是一种预后良好且对治疗有反应的原发性自身免疫性疾病。
在此，我们报告了一名以快速进展性痴呆和低钠血症为表现的男性患者。他的脑脊液和血清中均存在靶向LGI1的抗体，这证实了典型抗LGI1 AE的诊断。简易精神状态检查表和蒙特利尔认知评估量表的得分分别为19/30和15/30。头颅磁共振成像显示双侧海马区高信号。脑动脉自旋标记和氟代脱氧葡萄糖正电子发射断层扫描结果显示无异常灌注/代谢。经过静脉注射免疫球蛋白和糖皮质激素联合治疗后，患者的临床症状明显改善。
该病例提高了人们对针对LGI1的免疫反应可诱发以记忆缺陷为主的快速进展性痴呆的认识。更好地认识这一点对于早期诊断、必要的治疗乃至更好的预后都非常重要。
