Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Neurology, Mayo Clinic, Scottsdale, Arizona, USA.
Epilepsia. 2022 Dec;63(12):3180-3191. doi: 10.1111/epi.17424. Epub 2022 Oct 14.
To report the clinical presentations and outcomes of patients with seizure and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
We retrospectively reviewed the electronic medical records for clinical and paraclinical features among patients with seizures and MOG-IgG (immunoglobulin G) seropositivity.
We identified 213 patients with MOG-IgG seropositivity who fulfilled criteria for MOGAD. Seizures attributed to central nervous system (CNS) autoimmunity were observed in 10% of patients (n = 23: 19 children, 4 adults). The majority (n = 19, 83%) had pediatric disease onset. Focal motor seizures were the most common seizure semiology (16/23; 70%). Focal to bilateral tonic-clonic seizures were present in 12 patients (53%), and 3 patients (13%) developed status epilepticus. All patients had features of encephalitis at onset of seizures. Cerebral cortical encephalitis (CCE) was the most common radiological finding (10 unilateral and 5 bilateral cases). Eight of 23 patients (35%) had only CCE, six of 23 patients (26%) had only acute disseminated encephalomyelitis (ADEM), and seven of 23 patients (30%) had features of both. Fifteen patients (65%) had leptomeningeal enhancement. Three patients (13%) had coexistence of N-methyl-d-aspartate receptor (NMDAR) IgG. Only 3 of 23 patients (13%) developed drug- resistant epilepsy. Although the majority had MOGAD relapses (14/23, 60%) had only 5 of 23 patients had recurrence of episodes of encephalitis with associated seizures. Twenty-one of 23 patients (91%) had seizure freedom at last follow-up.
MOG-IgG evaluation should be considered in patients who present with encephalitis and focal motor and/or focal to bilateral tonic-clonic seizures, especially pediatric patients with magnetic resonance imaging (MRI) brain findings consistent with CCE, ADEM, or other MOGAD presentations. The majority of these seizures are self-limited and do not require maintenance/chronic antiseizure medications. Although seizure recurrence is uncommon, many patients have MOGAD relapses in the form of encephalitis and optic neuritis.
报告伴发髓鞘少突胶质细胞糖蛋白抗体的癫痫发作患者的临床表现和结局。
我们回顾性分析了电子病历中癫痫发作和髓鞘少突胶质细胞糖蛋白 IgG（免疫球蛋白 G）阳性患者的临床和辅助检查特征。
我们共纳入 213 例髓鞘少突胶质细胞糖蛋白 IgG 阳性患者，这些患者符合髓鞘少突胶质细胞糖蛋白相关疾病的诊断标准。10%（n=23）的患者存在中枢神经系统（CNS）自身免疫性癫痫发作，其中 19 例为儿童，4 例为成人。大多数（n=19）患者为儿童起病。局灶性运动性癫痫发作是最常见的癫痫发作表现（16/23；70%）。12 例患者（53%）出现局灶性至双侧强直-阵挛性癫痫发作，3 例（13%）出现癫痫持续状态。所有患者癫痫发作时均有脑炎表现。皮质脑炎（Cerebral Cortical Encephalitis，CCE）是最常见的影像学表现（10 例单侧，5 例双侧）。23 例患者中，8 例（35%）仅有 CCE，6 例（26%）仅有急性播散性脑脊髓炎（acute disseminated encephalomyelitis，ADEM），7 例（30%）兼有 CCE 和 ADEM。15 例（65%）患者有软脑膜强化。3 例（13%）患者同时存在 N-甲基-D-天冬氨酸受体（N-methyl-d-aspartate receptor，NMDAR）抗体阳性。仅有 3 例（13%）患者出现药物难治性癫痫。尽管大多数患者（14/23，60%）存在髓鞘少突胶质细胞糖蛋白相关疾病复发，但仅有 5 例（22%）患者复发的是伴有癫痫发作的脑炎。23 例患者中，21 例（91%）在末次随访时癫痫无发作。
如果患者出现脑炎和局灶性运动性和/或局灶性至双侧强直-阵挛性癫痫发作，尤其是磁共振成像（Magnetic Resonance Imaging，MRI）脑表现符合皮质脑炎、急性播散性脑脊髓炎或其他髓鞘少突胶质细胞糖蛋白相关疾病的儿童患者，应考虑进行髓鞘少突胶质细胞糖蛋白 IgG 评估。这些癫痫发作大多为自限性，无需维持/慢性抗癫痫药物治疗。尽管癫痫发作复发少见，但许多患者以脑炎和视神经炎的形式出现髓鞘少突胶质细胞糖蛋白相关疾病复发。
