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髓鞘少突胶质细胞糖蛋白抗体相关疾病中的大脑皮层脑炎。

Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

机构信息

Department of Neurology, Mayo Clinic, Scottsdale, AZ, USA.

Department of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA.

出版信息

Ann Neurol. 2023 Feb;93(2):297-302. doi: 10.1002/ana.26549. Epub 2022 Dec 2.

Abstract

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.

摘要

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.

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ac7/10107670/6f409e5e9d76/ANA-93-297-g002.jpg

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