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.
