Ikeguchi Ryotaro, Kanda Natsuki, Kobayashi Masaki, Masui Kenta, Nitta Masayuki, Misu Tatsuro, Muragaki Yoshihiro, Kawamata Takakazu, Shibata Noriyuki, Kitagawa Kazuo, Shimizu Yuko
Department of Neurology, Tokyo Women's Medical University, Tokyo, Japan.
Department of Pathology, Tokyo Women's Medical University, Tokyo, Japan.
Mult Scler J Exp Transl Clin. 2024 Dec 5;10(4):20552173241301011. doi: 10.1177/20552173241301011. eCollection 2024 Oct-Dec.
Few studies have examined B cells among patients with anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), including brain pathology.
To describe cases of tumefactive MOGAD with B-cell dominant central nervous system (CNS) infiltration.
In this study, we reviewed three cases with clinical and brain histopathological features with tumefactive MOGAD.
Forty-nine cases of tumefactive brain lesions (TBL) between January 2003 and December 2023 were included; of these, seven had MOGAD. Three underwent a brain biopsy. B-cell dominant CNS infiltration was observed in two cases. In two cases with B-cell dominant CNS infiltration, symptoms included fever, headache, nausea, somnolence, and focal neurological deficits. Cerebrospinal fluid examination revealed both mild pleocytosis and negative oligoclonal IgG bands. Magnetic resonance imaging of the brain revealed large abnormal lesions extending from the basal ganglia to the parietotemporal lobe in both cases. These cases showed a good response to steroids; however, one case relapsed. Brain pathology showed demyelination and perivascular lymphocytic infiltration. One showed small vessel vasculitis. Deposition of the activated complement component was absent or rarely observed. Loss of MOG was observed in two cases.
MOGAD could exhibit B-cell dominant CNS infiltration and small vessel vasculitis. MOGAD should be considered in differential diagnosis of TBL.
很少有研究在抗髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）患者中研究B细胞，包括脑病理学。
描述具有B细胞为主的中枢神经系统（CNS）浸润的瘤样MOGAD病例。
在本研究中，我们回顾了3例具有瘤样MOGAD临床和脑组织病理学特征的病例。
纳入2003年1月至2023年12月期间的49例瘤样脑病变（TBL）；其中7例患有MOGAD。3例接受了脑活检。2例观察到B细胞为主的CNS浸润。在2例B细胞为主的CNS浸润病例中，症状包括发热、头痛、恶心、嗜睡和局灶性神经功能缺损。脑脊液检查显示轻度细胞增多和寡克隆IgG带阴性。脑部磁共振成像显示2例均有从基底节延伸至顶颞叶的大片异常病变。这些病例对类固醇治疗反应良好；然而,1例复发。脑病理学显示脱髓鞘和血管周围淋巴细胞浸润。1例显示小血管血管炎。未观察到或很少观察到活化补体成分的沉积。2例观察到MOG缺失。
MOGAD可表现为B细胞为主的CNS浸润和小血管血管炎。在TBL的鉴别诊断中应考虑MOGAD。
