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Autoimmune cortical encephalitis in two children with anti-myelin oligodendrocyte glycoprotein (MOG) antibody
Journal of Neurology ( IF 6 ) Pub Date : 2020-10-14 , DOI: 10.1007/s00415-020-10260-4
D Doig 1 , C McNamara 2 , L Mewasingh 3 , S Beri 3 , B Jones 2 , C Kachramanoglou 2 , W Jan 2
Affiliation  

Purpose

Anti-myelin oligodendrocyte glycoprotein antibodies (anti-MOG), directed against a component of the myelin sheath, are sometimes detected in the blood or cerebrospinal fluid (CSF) of patients with acute demyelinating conditions. Cortical encephalitic presentations in anti-MOG-antibody-positive patients are recognized but rare, and few pediatric cases have been described.

Methods

We describe clinical, biochemical, and MRI findings in two children presenting with generalized seizures due to cortical encephalitis, and review potential underlying immunological processes.

Results

In both patients, anti-MOG antibodies were detected. Both underwent MRI scans which demonstrated bilateral cortical swelling and T2/fluid-attenuated inversion recovery (FLAIR) hyperintensity with corresponding regions of reduced diffusion.

Conclusion

Early detection of anti-MOG antibodies in patients with a similar presentation and imaging features would enable rapid institution of appropriate treatment, and potentially reduce the need for invasive diagnostic procedures such as brain biopsy.



中文翻译:

两个患抗髓磷脂少突胶质细胞糖蛋白(MOG)抗体的儿童的自身免疫性皮质脑炎

目的

有时在患有急性脱髓鞘疾病的患者的血液或脑脊液(CSF)中检测到针对髓鞘鞘成分的抗髓磷脂少突胶质细胞糖蛋白抗体(anti-MOG)。认识到抗MOG抗体阳性患者的皮质脑病表现,但很少见,已经描述了很少的儿科病例。

方法

我们描述了由于皮层脑炎而导致全身性癫痫发作的两名儿童的临床,生化和MRI表现,并回顾了潜在的潜在免疫学过程。

结果

在这两名患者中,均检测到抗MOG抗体。两者均进行了MRI扫描,结果显示双侧皮质肿胀和T2 /流体衰减倒置恢复(FLAIR)高强度,并伴有扩散减少的相应区域。

结论

在具有相似表现和影像特征的患者中早期检测到抗MOG抗体将能够迅速进行适当的治疗,并有可能减少对侵入性诊断程序(例如脑活检)的需求。

更新日期:2020-10-14
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