Cornacchini Sara, Farina Antonio, Contento Margherita, Berti Valentina, Biggi Martina, Barilaro Alessandro, Massacesi Luca, Damato Valentina, Rosati Eleonora
Department of Neurosciences Drugs and Child Health, University of Florence, Florence, Italy.
Department of Neurology 2, Careggi University Hospital, Florence, Italy.
Front Neurol. 2022 Aug 16;13:949240. doi: 10.3389/fneur.2022.949240. eCollection 2022.
The use of CD20-depleting monoclonal antibodies has shown to improve the long-term outcome of patients with anti-leucine-rich glioma-inactivated protein 1 antibodies (anti-LGI1-Abs) encephalitis after first-line immunotherapy, but currently predictive markers of treatment response and disease activity are lacking.
A 75-year-old man presented cognitive impairment and faciobrachial dystonic seizures (FBDS), with mild abnormalities at electroencephalography (EEG), normal brain magnetic resonance and cerebrospinal fluid (CSF) analysis. Anti-LGI1-Abs were detected in serum and CSF, and corticosteroids and intravenous immunoglobulins were administered. Despite partial cognitive improvement, 18F-fluoridesoxyglucose-positron emission tomography (18F-FDG-PET) showed the persistence of temporo-mesial hypermetabolism, and FBDS were still detected by long-term monitoring video EEG (LTMV EEG). Rituximab was therefore administered with FBDS disappearance, further cognitive improvement, and resolution of 18F-FDG-PET temporo-mesial hypermetabolism.
Our experience supports the use of 18F-FDG-PET and LTMVEEG as useful tools to measure disease activity, evaluate treatment response and guide therapeutic decisions in the long-term management of anti-LGI1-antibody encephalitis.
使用抗 CD20 单克隆抗体已显示可改善一线免疫治疗后抗富含亮氨酸胶质瘤失活蛋白 1 抗体(抗 LGI1 抗体)脑炎患者的长期预后,但目前缺乏治疗反应和疾病活动的预测标志物。
一名 75 岁男性出现认知障碍和面臂肌张力障碍性癫痫发作(FBDS),脑电图(EEG)有轻度异常,脑磁共振成像和脑脊液(CSF)分析正常。在血清和脑脊液中检测到抗 LGI1 抗体,并给予了皮质类固醇和静脉注射免疫球蛋白。尽管认知有部分改善,但 18F - 氟脱氧葡萄糖 - 正电子发射断层扫描(18F - FDG - PET)显示颞叶内侧高代谢持续存在,长期监测视频脑电图(LTMV EEG)仍检测到 FBDS。因此给予利妥昔单抗治疗,FBDS 消失,认知进一步改善,18F - FDG - PET 颞叶内侧高代谢消退。
我们的经验支持将 18F - FDG - PET 和 LTMV EEG 作为测量疾病活动、评估治疗反应以及指导抗 LGI1 抗体脑炎长期管理中治疗决策的有用工具。