CNS免疫病文献复习系列之二：MOG抗体阳性的单侧皮质脑炎

MOG antibody-positive,benign, unilateral,cerebral cortical encephalitis with epilepsy

表现为癫痫的良性、单侧、MOG抗体阳性的皮质脑炎

本文对Tohoku大学医院2008年-2014年的24例病因不明，激素反应良好，MOG抗体阳性的皮层脑炎患者进行了回顾性分析。研究发现：这些患者的中位数年龄是37岁（27-39岁），主要的症状是癫痫发作，伴或不伴异常行为或意识障碍。所有的患者颅脑磁共振表现为Flair上偏侧皮层高信号，肿胀，SPECT提示灌注增高。脑脊液中可见中等程度白细胞增多，蛋白轻度增高。但MBP未见增高。脑炎相关的自身免疫抗体如AQP4-IgG,NMDA受体抗体、VGKC抗体均阴性。所有患者均接受抗癫痫药物治疗，在大剂量激素冲击治疗后均完全恢复，磁共振上偏侧皮层病灶消失。没有患者复发。

结论：这样表现的脑炎可能为自身免疫性，但其表现和MOG抗体介导的中枢神经系统脱髓鞘、 Rasmussen脑炎和其它已知免疫介导的脑炎均不同。

OrbitalMRI findings in cases 1 and 2  Hyperintensitieson axial short T1 inversion recovery (A, C) and gadolinium enhancement (B, D)were seen in the right optic nerves in cases 1 (A, B) and 2 (C, D), suggestingunilateral optic neuritis.

BrainMRI findings in case 1  Whenhospitalized with epileptic seizure, the right hemispheric cortical region incase 1 was fluid-attenuated inversion recovery hyperintense (A) (arrowheads) and partiallygadolinium enhanced. Meanwhile, hyperintensities in the cortical region were less evidenton diffusion-weighted (B), apparent diffusion coefficient (C), T2-weighted (D),T1-weighted (E), and gadolinium enhancement on T1-weighted (F) MRI.

·BrainMRI and SPECT findings in cases 1–4   Onadmission, fluid-attenuated inversionrecovery (FLAIR) hyperintensity was seen in the unilateral cerebralcortex in cases 1 (A–C), 2 (F–H), 3 (K–N), and 4 (P–R) (arrowheads). BrainSPECT showed hyperperfusion in those cerebral cortical regions withFLAIR hyperintensity in cases 1 (D), 2 (I), and 4 (S).However, the FLAIR hyperintensities in the cortical regions disappearedafter more than 2 years (E, J, O, T).

原文献：

OgawaR, NakashimaI, et al.MOG antibody-positive, benign,unilateral, cerebral cortical encephalitis with epilepsy.Neurol Neuroimmunol Neuroinflamm. 2017;4(2):e322

相关文章请参阅：

1.CNS脱髓鞘病变文献复习系列之一：CLIPPERS？anti-MOG+ NMOSD？ 

2.万人大讨论：一例NMOSD的讨论及引发的思考

3 颅内撒“胡椒盐”征——CLIPPERS 综合征

4. 黎明，不会遥远……

5. 1例硬脑膜动静脉瘘的误诊分析及经验总结

6. 中枢神经系统免疫病的“四个诊断和两个评估”

7.误诊为脑肿瘤的中枢神经系统脱髓鞘假瘤1例

更多文章，请自行翻阅公众号历史文章

如果您对此话题感兴趣，欢迎留言讨论、指导！