Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognitive Disorders, Chongqing, China.
Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognitive Disorders, Chongqing, China.
Eur J Paediatr Neurol. 2024 Nov;53:25-32. doi: 10.1016/j.ejpn.2024.09.005. Epub 2024 Sep 16.
To identify clinical factors and biomarkers that could contribute to early differential diagnosis of acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) in the pediatric population, with limited evidence.
We conducted an observational retrospective study of children diagnosed with AIDP and A-CIDP between January 2014 and December 2022. Demographic data, clinical features, and routine biomarkers were also analyzed. Statistical analysis was used to identify significant features with high sensitivity and specificity.
We included 91 AIDP and 17 A-CIDP patients. The A-CIDP group had an older median age (6.33 vs. 4.33 years, p = 0.017), required more complex immunotherapies (p < 0.001), and showed a longer time to nadir over 2 weeks (76.5 % vs. 7.7 %, p < 0.001). Gastrointestinal dysfunction (29.4 % vs. 6.59 %, p = 0.014) and numbness (35.3 % vs. 12.1 %, p = 0.027) were more prevalent in A-CIDP. The AIDP patients had a longer median hospitalization stays (13 vs. 11 days, p < 0.05), more prodromal events (90.1 % vs. 64.7 %, p = 0.013), and more frequent cranial nerve palsy (61.5 % vs. 5.88 %, p < 0.001). The disability scores on admission, discharge, and peak were worse in the AIDP group (p < 0.001). AIDP patients showed higher cerebrospinal fluid protein (p = 0.039), albumin quotient (p = 0.048), leukocytes (p = 0.03), neutrophils (p = 0.010), platelet count (p = 0.005), systemic inflammatory index (SII) (p = 0.009), and gamma-glutamyl transferase (p = 0.039). Multivariable regression identified two independent predictors of early A-CIDP detection: time from onset to peak beyond 2 weeks (OR = 37.927, 95%CI = 7.081-203.15) and lower modified Rankin Scale score on admission (OR = 0.308, 95%CI = 0.121-0.788).
Our study found that when the condition continued to deteriorate beyond two weeks with a lower mRS on admission and possibly less cranial nerve involvement, we may favor the diagnosis of pediatric A-CIDP rather than AIDP.
为了在儿科人群中确定有助于急性炎症性脱髓鞘性多发性神经病（AIDP）和急性发作的慢性炎症性脱髓鞘性多发性神经病（A-CIDP）的早期鉴别诊断的临床因素和生物标志物，但证据有限。
我们对 2014 年 1 月至 2022 年 12 月期间诊断为 AIDP 和 A-CIDP 的儿童进行了一项观察性回顾性研究。还分析了人口统计学数据、临床特征和常规生物标志物。使用统计分析来识别具有高灵敏度和特异性的显著特征。
我们纳入了 91 例 AIDP 和 17 例 A-CIDP 患者。A-CIDP 组的中位年龄较大（6.33 岁 vs. 4.33 岁，p=0.017），需要更复杂的免疫治疗（p<0.001），并且在 2 周以上达到最低点的时间更长（76.5% vs. 7.7%，p<0.001）。胃肠道功能障碍（29.4% vs. 6.59%，p=0.014）和麻木（35.3% vs. 12.1%，p=0.027）在 A-CIDP 中更为常见。AIDP 患者的中位住院时间更长（13 天 vs. 11 天，p<0.05），前驱事件更常见（90.1% vs. 64.7%，p=0.013），颅神经麻痹更常见（61.5% vs. 5.88%，p<0.001）。AIDP 组入院时、出院时和峰值时的残疾评分更差（p<0.001）。AIDP 患者的脑脊液蛋白（p=0.039）、白蛋白商（p=0.048）、白细胞（p=0.03）、中性粒细胞（p=0.010）、血小板计数（p=0.005）、全身炎症指数（SII）（p=0.009）和γ-谷氨酰转移酶（p=0.039）更高。多变量回归确定了早期 A-CIDP 检测的两个独立预测因素：发病至高峰超过 2 周的时间（OR=37.927，95%CI=7.081-203.15）和入院时较低的改良 Rankin 量表评分（OR=0.308，95%CI=0.121-0.788）。
我们的研究发现，当病情在 2 周后继续恶化，入院时 mRS 评分较低且可能较少累及颅神经时，我们可能更倾向于诊断为儿科 A-CIDP 而不是 AIDP。
