Rubin Martina, Cutillo Gianni, Viti Vittorio, Margoni Monica, Preziosa Paolo, Zanetta Chiara, Bellini Anna, Moiola Lucia, Fanelli Giovanna Franca, Rocca Maria Assunta, Filippi Massimo
Neuroimaging Research Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Neurology Unit, IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132, Milan, Italy.
J Neurol. 2025 Jul 14;272(8):508. doi: 10.1007/s00415-025-13245-3.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare central nervous system (CNS) demyelinating disease presenting heterogeneously across lifespan. Although frequent, epilepsy remains a poorly characterized MOGAD-associated manifestation.
To describe age-related clinical, fluid, imaging, and neurophysiological features in MOGAD patients with epilepsy, we systematically reviewed online repositories up to April 2025, identifying 178 eligible studies.
A total of 2487 MOGAD patients were included from clinical studies, and of 337 from case reports/series, 140 with adult-onset and 197 with pediatric-onset disease. Seizures prevalence was 30.6% (95% confidence interval [95%CI] = 28%; 33.3%) in pediatric-onset and 7% (95%CI = 4.1%; 11.2%) in adult-onset cohorts. Pediatric-onset patients were significantly more likely to be female (p = 0.003). Cortical encephalitis was the most common presentation in both age groups, followed by acute demyelinating encephalomyelitis in pediatric-onset and acute demyelinating syndrome in adult-onset patients. In both groups, epileptic manifestations predominantly occurred at disease onset. Pediatric-onset patients were more likely to experience status epilepticus (p = 0.005) and encephalopathy (p = 0.002), whereas adult-onset exhibited higher frequency of cerebrospinal fluid pleocytosis (p < 0.001). Co-positivity with antibodies related to other encephalitides was present in 37.3% of patients, most commonly with anti-N-methyl-D-aspartate receptor (anti-NMDAR) IgG (88.3%), showing no age-dependent differences. No significant age-related differences were observed in leptomeningeal enhancement. Adult-onset patients more frequently showed parietal lobe involvement (p = 0.018) and fewer temporal lobe lesions (p = 0.004) compared to pediatric-onset. Despite comparable use of immunotherapy and anti-seizure medications across groups, chronic epilepsy was more prevalent among pediatric-onset patients (p < 0.001).
Epilepsy is a relevant MOGAD-associated condition with a risk of chronic persistence. Prospective studies are warranted to establish an age-specific therapeutic approach.
髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）是一种罕见的中枢神经系统（CNS）脱髓鞘疾病，在整个生命周期中表现各异。癫痫虽然常见，但仍是MOGAD相关表现中特征描述较少的一种。
为描述患有癫痫的MOGAD患者的年龄相关临床、体液、影像学和神经生理学特征，我们系统回顾了截至2025年4月的在线资料库，确定了178项符合条件的研究。
临床研究共纳入2487例MOGAD患者，病例报告/系列研究纳入337例，其中140例为成人起病，197例为儿童起病。儿童起病队列中癫痫发作患病率为30.6%（95%置信区间[95%CI]=28%；33.3%），成人起病队列中为7%（95%CI=4.1%；11.2%）。儿童起病患者女性比例显著更高（p=0.003）。皮质脑炎是两个年龄组中最常见的表现，其次是儿童起病组的急性脱髓鞘性脑脊髓炎和成人起病组的急性脱髓鞘综合征。在两组中，癫痫表现主要发生在疾病发作时。儿童起病患者更易发生癫痫持续状态（p=0.005）和脑病（p=0.002），而成人起病组脑脊液淋巴细胞增多的频率更高（p<0.001）。37.3%的患者与其他脑炎相关抗体呈共阳性，最常见的是抗N-甲基-D-天冬氨酸受体（抗NMDAR）IgG（88.3%），未显示出年龄依赖性差异。软脑膜强化方面未观察到显著的年龄相关差异。与儿童起病患者相比，成人起病患者更常出现顶叶受累（p=0.018），颞叶病变较少（p=0.004）。尽管各组间免疫治疗和抗癫痫药物的使用情况相当，但慢性癫痫在儿童起病患者中更为普遍（p<0.001）。
癫痫是一种与MOGAD相关的疾病，有慢性持续的风险。有必要进行前瞻性研究以建立针对特定年龄的治疗方法。
