Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, University of Pavia,V.le C. Golgi, 19 - 27100 Pavia, Pavia, Italy.
Neuroimmunology Laboratory, IRCCS Mondino Foundation, Pavia, Italy.
Mult Scler Relat Disord. 2020 Jun;41:102011. doi: 10.1016/j.msard.2020.102011. Epub 2020 Feb 22.
Myelin oligodendrocyte glycoprotein (MOG) antibodies (Abs) have been associated with a heterogeneous range of acquired CNS demyelinating disorders. More recently, increasing evidence correlates the presence of such Abs with seizures, occurring in concomitance with CNS demyelinating events, or even as isolated phenomena. In this scenario, the full clinical spectrum of MOG Ab-associated seizures and the contribution of such Abs to epileptogenesis are unclear.
We report on two paradigmatic cases of MOG Ab-associated seizures, one showing isolated seizures, without evidence of encephalopathy or MRI changes, followed by a demyelinating event one month later, and the other presenting with seizures as the main manifestation of an acute disseminated encephalomyelitis (ADEM) event. To better frame this topic, we performed a literature review, identifying 49 patients with MOG Ab-associated disorders presenting seizures at any stage of their disease, and analysed the clinico-therapeutic, brain MRI, cerebrospinal fluid, and EEG features.
MOG Ab-associated seizures occurred mostly during encephalitis, including: a) "cortical encephalitis", a clinically poorly defined syndrome characterised by gray matter lesions on brain MRI, with or without subcortical white matter involvement; b) ADEM; c) NMDAR encephalitis with demyelinating features. Seizures can also occur in isolation, often in clusters of focal motor seizures, in patients with normal brain MRI, heralding the more typical MOG Ab-associated demyelinating syndrome by days to months.
Testing for MOG Abs should be considered in children with isolated and unexplained seizures, and in adults with suspected encephalitis and/or seizures. In these cases, MOG Abs detection is highly relevant for patients' clinical management.
髓鞘少突胶质细胞糖蛋白（MOG）抗体（Abs）与一系列获得性中枢神经系统脱髓鞘疾病有关。最近，越来越多的证据表明，这些 Abs 与癫痫有关，可发生在中枢神经系统脱髓鞘事件的同时，甚至是孤立的现象。在这种情况下，MOG Ab 相关癫痫的临床表现谱以及这些 Abs 对癫痫发生的影响尚不清楚。
我们报告了两例典型的 MOG Ab 相关癫痫发作病例，一例表现为孤立性发作，无脑病或 MRI 改变，一个月后发生脱髓鞘事件，另一例表现为急性播散性脑脊髓炎（ADEM）事件的主要表现为癫痫发作。为了更好地阐述这一主题，我们进行了文献回顾，确定了 49 例 MOG Ab 相关疾病患者在疾病的任何阶段出现癫痫发作，并分析了其临床-治疗、脑 MRI、脑脊液和 EEG 特征。
MOG Ab 相关癫痫发作主要发生在脑炎期间，包括：a）“皮质脑炎”，一种临床上定义不明确的综合征，其特征为脑 MRI 上有灰质病变，伴有或不伴有皮质下白质受累；b）ADEM；c）伴有脱髓鞘特征的 NMDAR 脑炎。癫痫发作也可以孤立发生，通常是局灶性运动性癫痫发作的簇状发作，在脑 MRI 正常的患者中，数天至数月后出现更典型的 MOG Ab 相关脱髓鞘综合征。
对于孤立性和不明原因的癫痫发作的儿童，以及疑似脑炎和/或癫痫发作的成年人，应考虑进行 MOG Abs 检测。在这些情况下，MOG Abs 的检测对患者的临床管理具有重要意义。
