Department of Neurology, Shenzhen Children's Hospital, Shenzhen 518038, Guangdong, China.
Department of Neurology, Shenzhen Children's Hospital, Shenzhen 518038, Guangdong, China.
Mult Scler Relat Disord. 2022 Dec;68:104126. doi: 10.1016/j.msard.2022.104126. Epub 2022 Aug 23.
Myelin oligodendrocyte glycoprotein (MOG) antibodies mediate inflammatory demyelinating diseases of the central nervous system. This study aimed to understand the clinical characteristics of MOG antibody-associated aseptic meningitis (MOGAM).
Here, we report the cases of two children with MOGAM. A systematic literature review was conducted and included patients who had MOGAM only, without neurological parenchymal lesions. The clinical characteristics that may have affected the outcome were statistically analyzed.
We reviewed 12 cases of MOGAM; male: female = 9: 3. Prolonged fever lasting over 7 days (11/12) was the most frequent symptom, followed by headache (10/12), vomiting (5/12), and seizures (4/12). None of the patients had focal neurological manifestations or parenchymal lesions on imaging. Cerebrospinal fluid (CSF) leukocytosis was observed in all patients (12/12), and blood leukocytosis and elevated CSF pressure was observed in all patients who had corresponding results (9/9 and 4/4, respectively). Seizures occurrence was lower than that of MOG antibody-associated cortical encephalitis. Seven cases progressed to other MOG antibody-associated diseases (MOGADs) in the later phase of MOGAM. Patients who did not progress to other MOGADs had a shorter disease duration from onset to the initiation of intravenous methylprednisolone than those who did. All the patients achieved full recovery after steroid treatment. One patient had relapses.
MOGAM without inflammatory demyelination is a rare but distinct phenotype of MOGAD, with fewer clinical manifestations mimicking bacterial or viral meningitis/encephalomeningitis. Delayed diagnosis and treatment may induce the progression to other severe MOGADs. Early recognition of this unique autoimmune aseptic meningitis may contribute to early diagnosis, treatment, and better outcomes.
髓鞘少突胶质细胞糖蛋白（MOG）抗体介导中枢神经系统的炎症性脱髓鞘疾病。本研究旨在了解MOG 抗体相关性无菌性脑膜炎（MOGAM）的临床特征。
在此，我们报告了两例 MOGAM 患儿的病例。进行了系统的文献复习，并纳入了仅有 MOGAM 而无神经实质病变的患者。对可能影响结局的临床特征进行了统计学分析。
我们回顾了 12 例 MOGAM 患者；男：女=9:3。最常见的症状是持续超过 7 天的高热（11/12），其次是头痛（10/12）、呕吐（5/12）和癫痫发作（4/12）。所有患者的影像学均无局灶性神经表现或实质病变。所有患者的脑脊液（CSF）白细胞增多（12/12），所有有相应结果的患者均有白细胞增多和升高的 CSF 压力（9/9 和 4/4）。癫痫发作的发生率低于 MOG 抗体相关皮质脑炎。7 例在 MOGAM 后期进展为其他 MOG 抗体相关性疾病（MOGADs）。未进展为其他 MOGADs 的患者，从发病到开始静脉用甲基强的松龙的病程短于进展为其他 MOGADs 的患者。所有患者在类固醇治疗后均完全恢复。1 例患者复发。
无炎症性脱髓鞘的 MOGAM 是 MOGAD 的一种罕见但独特的表型，临床表现较少模仿细菌性或病毒性脑膜炎/脑膜脑炎。延迟诊断和治疗可能会导致其他严重 MOGAD 的进展。早期认识这种独特的自身免疫性无菌性脑膜炎可能有助于早期诊断、治疗和更好的结局。
