MOGAD-related epilepsy: a systematic characterization of age-dependent clinical, fluid, imaging and neurophysiological features.

IF 4.8 2区 医学 Q1 CLINICAL NEUROLOGY
Martina Rubin, Gianni Cutillo, Vittorio Viti, Monica Margoni, Paolo Preziosa, Chiara Zanetta, Anna Bellini, Lucia Moiola, Giovanna Franca Fanelli, Maria Assunta Rocca, Massimo Filippi
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Abstract

Background: 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.

Methods: 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.

Results: 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).

Conclusions: Epilepsy is a relevant MOGAD-associated condition with a risk of chronic persistence. Prospective studies are warranted to establish an age-specific therapeutic approach.

摩加迪沙相关癫痫:年龄依赖性临床、体液、影像学和神经生理特征的系统表征
背景: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,在整个生命周期中呈现异质性。虽然频繁,癫痫仍然是一种特征不明显的与摩加迪沙相关的表现。方法:为了描述MOGAD合并癫痫患者的年龄相关临床、体液、影像学和神经生理特征,我们系统地回顾了截至2025年4月的在线知识库,确定了178项符合条件的研究。结果:共有2487例MOGAD患者从临床研究中纳入,其中337例来自病例报告/系列,140例为成人发病,197例为儿科发病。癫痫发作发生率为30.6%(95%可信区间[95% ci] = 28%;33.3%)和7% (95%CI = 4.1%;11.2%)。儿科起病的患者中女性的可能性明显更高(p = 0.003)。皮层脑炎是两个年龄组中最常见的表现,其次是儿科发病的急性脱髓鞘性脑脊髓炎和成人发病的急性脱髓鞘综合征。在两组中,癫痫表现主要发生在发病时。儿科发病的患者更有可能出现癫痫持续状态(p = 0.005)和脑病(p = 0.002),而成人发病的患者脑脊液多细胞增多的频率更高(p)。结论:癫痫是一种与摩加迪沙相关的疾病,具有慢性持续性风险。有必要进行前瞻性研究,以建立针对年龄的治疗方法。
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来源期刊
Journal of Neurology
Journal of Neurology 医学-临床神经学
CiteScore
10.00
自引率
5.00%
发文量
558
审稿时长
1 months
期刊介绍: The Journal of Neurology is an international peer-reviewed journal which provides a source for publishing original communications and reviews on clinical neurology covering the whole field. In addition, Letters to the Editors serve as a forum for clinical cases and the exchange of ideas which highlight important new findings. A section on Neurological progress serves to summarise the major findings in certain fields of neurology. Commentaries on new developments in clinical neuroscience, which may be commissioned or submitted, are published as editorials. Every neurologist interested in the current diagnosis and treatment of neurological disorders needs access to the information contained in this valuable journal.
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