Department of Neurology, Mayo Clinic, Scottsdale, AZ, USA.
Department of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA.
Ann Neurol. 2023 Feb;93(2):297-302. doi: 10.1002/ana.26549. Epub 2022 Dec 2.
Cerebral cortical encephalitis (CCE) is a recently described myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) phenotype. In this observational retrospective study, we characterized 19 CCE patients (6.7% of our MOGAD cohort). Headache (n = 15, 79%), seizures (n = 13, 68%), and encephalopathy (n = 12, 63%) were frequent. Magnetic resonance imaging revealed unilateral (n = 12, 63%) or bilateral (n = 7, 37%) cortical T2 hyperintensity and leptomeningeal enhancement (n = 17, 89%). N-Methyl-D-aspartate receptor autoantibodies coexisted in 2 of 15 tested (13%). CCE pathology (n = 2) showed extensive subpial cortical demyelination (n = 2), microglial reactivity (n = 2), and inflammatory infiltrates (perivascular, n = 1; meningeal, n = 1). Most received high-dose steroids (n = 17, 89%), and all improved, but 3 had CCE relapses. This study highlights the CCE spectrum and provides insight into its pathogenesis. ANN NEUROL 2023;93:297-302.
大脑皮层脑炎(CCE)是一种新近描述的髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)表型。在这项观察性回顾性研究中,我们对 19 例 CCE 患者(我们的 MOGAD 队列的 6.7%)进行了特征描述。头痛(n=15,79%)、癫痫发作(n=13,68%)和脑病(n=12,63%)较为常见。磁共振成像显示单侧(n=12,63%)或双侧(n=7,37%)皮质 T2 高信号和软脑膜增强(n=17,89%)。在 15 例检测到的患者中,有 2 例(13%)共存 N-甲基-D-天冬氨酸受体自身抗体。2 例 CCE 病理(n=2)显示广泛的皮质下软脑膜脱髓鞘(n=2)、小胶质细胞反应(n=2)和炎症浸润(血管周围,n=1;脑膜,n=1)。大多数患者接受了大剂量类固醇治疗(n=17,89%),所有患者均有所改善,但 3 例出现 CCE 复发。本研究强调了 CCE 的谱,并深入了解其发病机制。神经病学年鉴 2023;93:297-302.