Scholarlink

Background <p>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&#xa0;characterized MOGAD-associated manifestation.</p> Methods <p>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.</p> Results <p>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 (<i>p</i> = 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 (<i>p</i> = 0.005) and encephalopathy (<i>p</i> = 0.002), whereas adult-onset exhibited higher frequency of cerebrospinal fluid pleocytosis (<i>p</i> &lt; 0.001). Co-positivity with antibodies related to other encephalitides was present in 37.3% of patients, most commonly with anti-<i>N</i>-methyl-<span>d</span>-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 (<i>p</i> = 0.018) and fewer temporal lobe lesions (<i>p</i> = 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 (<i>p</i> &lt; 0.001).</p> Conclusions <p>Epilepsy is a relevant MOGAD-associated condition with a risk of chronic persistence. Prospective studies are warranted to establish an age-specific therapeutic approach.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

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

  • 期刊论文

  • 发表日期：

    2025年7月14日

查看全文
  • Martina Rubin,
  • Gianni Cutillo,
  • Vittorio Viti,
  • Monica Margoni,
  • Paolo Preziosa,
  • Chiara Zanetta,
  • Anna Bellini,
  • Lucia Moiola,
  • Giovanna Franca Fanelli,
  • Maria Assunta Rocca,
  • Massimo Filippi

摘要

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.