Priyadarshini Debashree, Anuhya Velaga, Mahapatra Anuspandana
Department of Paediatrics, IMS & SUM Hospital, Siksha 'O' Anusandhan, Deemed to be University, Bhubaneswar, 751003, Odisha, India.
Ital J Pediatr. 2025 Jun 7;51(1):179. doi: 10.1186/s13052-025-02037-0.
Guillain-Barré Syndrome (GBS) is a rare but serious immune-mediated neuropathy characterized by acute-onset motor weakness and potential respiratory failure. Its pathogenesis often involves molecular mimicry triggered by antecedent infections, leading to autoimmune targeting of peripheral nerves. Epidemiological data suggest a correlation between infectious outbreaks and increased GBS incidence, as exemplified by the 2025 surge in cases associated with Campylobacter jejuni enteritis in Pune, India.
This prospective observational study was conducted over 24 months in the Pediatric Intensive Care Unit (PICU) of a tertiary care hospital. Ethical approval was obtained. Consecutive enrolment included children aged 2-14 years diagnosed with GBS who presented within two weeks of symptom onset. Comprehensive clinical, electrophysiological, and treatment data were collected. Patients were prospectively followed for six months, and outcomes were assessed using the GBS Disability Score. The modified Erasmus GBS outcome Score (mEGOS) and Erasmus GBS Respiratory Insufficiency Score (EGRIS) were applied for prognostic evaluation.
The study cohort comprised 27 children, with males aged 5-9 years being the most commonly affected. The mean (± SD) age was 6.56 (± 3.00) years. All participants reported antecedent illnesses, predominantly gastroenteritis. Clinically, symmetric motor weakness was observed in 81.5%, and 77.8% exhibited sensory involvement. Respiratory compromise requiring mechanical ventilation occurred in 11.1% of patients. Electrophysiological studies identified acute motor axonal neuropathy (AMAN) as the predominant variant, with acute motor sensory axonal neuropathy (AMSAN) demonstrating more severe clinical courses, including higher ventilation requirements and poorer functional outcomes. Prognostic assessment revealed median (IQR) mEGOS scores of 5 (4,6) at admission and 4 (3,6) at 1-week post-admission. These scores significantly predicted outcomes at 4 weeks, 3 months, and 6 months, with higher scores correlating with greater disability. The cohort's mean EGRIS score was 5.67, with higher scores predictive of increased mechanical ventilation requirements.Notably, all patients achieved favourable outcomes with no mortality, highlighting the effectiveness of the implemented management protocol.
Our findings demonstrate that mEGOS and EGRIS are effective prognostic tools in pediatric GBS. The mEGOS reliably predicts functional outcomes at multiple recovery stages, while the EGRIS is particularly useful in early identification of patients at risk for respiratory failure requiring mechanical ventilation.
吉兰-巴雷综合征（GBS）是一种罕见但严重的免疫介导性神经病，其特征为急性起病的运动无力和潜在的呼吸衰竭。其发病机制通常涉及前驱感染引发的分子模拟，导致外周神经成为自身免疫攻击的目标。流行病学数据表明，传染病暴发与GBS发病率增加之间存在关联，例如2025年印度浦那空肠弯曲菌肠炎相关病例的激增。
这项前瞻性观察性研究在一家三级医院的儿科重症监护病房（PICU）进行了24个月。获得了伦理批准。连续纳入2至14岁、在症状出现两周内确诊为GBS的儿童。收集了全面的临床、电生理和治疗数据。对患者进行了为期六个月的前瞻性随访，并使用GBS残疾评分评估结果。采用改良的伊拉斯谟GBS结局评分（mEGOS）和伊拉斯谟GBS呼吸功能不全评分（EGRIS）进行预后评估。
研究队列包括27名儿童，最常受影响的是5至9岁的男性。平均（±标准差）年龄为6.56（±3.00）岁。所有参与者均报告有前驱疾病，主要是胃肠炎。临床上，81.5%的患者出现对称性运动无力，77.8%的患者有感觉受累。11.1%的患者出现需要机械通气的呼吸功能不全。电生理研究确定急性运动轴索性神经病（AMAN）为主要类型，急性运动感觉轴索性神经病（AMSAN）的临床病程更为严重，包括更高的通气需求和更差的功能结局。预后评估显示，入院时mEGOS评分中位数（IQR）为5（4,6），入院后1周为4（3,6）。这些评分在4周、3个月和6个月时显著预测了结局，评分越高与残疾程度越高相关。该队列的平均EGRIS评分为5.67，评分越高预示机械通气需求增加。值得注意的是，所有患者均取得了良好结局，无死亡病例，突出了所实施管理方案的有效性。
我们的研究结果表明，mEGOS和EGRIS是儿科GBS有效的预后工具。mEGOS能可靠地预测多个恢复阶段的功能结局，而EGRIS在早期识别有呼吸衰竭需要机械通气风险的患者方面特别有用。
