儿童重型吉兰-巴雷综合征与神经节苷脂抗体的相关性

The Relationship Between Severe Guillain-BarréSyndrome and Ganglioside Antibody Spectrum in Children

目的探讨重型吉兰-巴雷综合征(Guillain-Barré syndrome,GBS)患儿的临床特点与神经节苷脂抗体(antiganglioside antibodies,AGAs)之间存在的关联性,并依据分析结果,评估神经节苷脂抗体与疾病分型、临床特征的关系,为临床合理治疗、估计疾病预后提供参考。方法回顾性分析2016年1月—2022年6月山东大学齐鲁医院收诊该疾病患儿的病历资料,根据病情及肌电图检查结果,划分为急性炎性脱髓鞘性多发神经根神经病(acute inflammatory demyelinating polyneuropathy,AIDP)、急性运动轴索性神经病(acute motor axonal neuropathy,AMAN)、急性运动感觉轴索性神经病(acute motor sensory axonal neuropathy,AMSAN)3个亚型。检测患儿血清神经节苷脂抗体,统计抗体总体阳性检出率、各亚型重症GBS患儿抗体的构成比,比较各亚型抗体阳性组和阴性组患儿的临床表现、实验室指标、疾病治疗效果等。结果AIDP组、AMAN组及AMSAN组研究对象的AGAs整体阳性检出率比较,差异无统计学意义(P>0.05),且无性别、年龄差异。AMAN组中GM1-IgG、GQ1b-IgG和GD1a-IgG的阳性检出值均高于AIDP组(P<0.05);AIDP组抗体阳性者,肢体无力、延髓症状(呼吸受累)、肢体麻木、腱反射消失的发生率,入院时下肢肌力减退程度均大于抗体阴性组;脑脊液蛋白升高更明显,肌力改善所需时间更长、出院时下肢肌力更低(P<0.05)。抗体阳性组ΔHughes评分为(1.04±0.80)分,抗体阴性组为(1.69±0.48)分,差异有统计学意义(P<0.05)。AMAN组AGAs阳性研究对象,第Ⅶ对颅神经受累发生率相对更高,对比AGAs阴性者,差异有统计学意义(P<0.05)。结论AMAN组中GM1-IgG、GQ1b-IgG和GD1a-IgG的抗体阳性检出率更高,AIDP患儿的AGAs阳性者相对病情更重,预后更差,AMAN患儿第Ⅶ对颅神经受累风险程度更高。

Objective To explore the correlation between clinical characteristics and anti ganglioside antibodies(AGAs)in children with severe Guillain-Barrésyndrome(GBS),and to evaluate the relationship between agas and disease classification and clinical features based on the analysis results,providing reference for rational clinical treatment and estimating disease prognosis.Methods During the observation period from January 2016 to June 2022,the medical records of children with this disease admitted to Qilu Hospital of Shandong University were reviewed.Based on the condition and electromyographic examination results,acute inflammatory demyelinating polyneuropathy(AIDP),acute motor axonal neuropathy(AMAN),and acute motor sensory axonal neuropathy(AMSAN)three subtypes were divided and serum gangliosides were detected in the children.Lipid antibodies,statistical analysis of the overall positive detection rate of antibodies,the composition ratio of antibodies in various subtypes of severe GBS patients,and comparison of clinical manifestations,laboratory indicators,disease treatment effects,etc.between the positive and negative antibody groups of each subtype.Results The overall positive detection rates of AGAs in the AIDP group,AMAN group,and AMSAN group were compared,and there was no statistically significant difference(P>0.05).Gender and age were not independent influencing factors.The positive detection values of GM1 IgG,GQ1b IgG,and GD1a IgG in the AMAN group were higher than those in the AIDP group(P<0.05).The incidence of limb weakness,bulbar symptoms(respiratory involvement),limb numbness,and disappearance of tendon reflexes in AIDP positive patients is higher than that in the antibody negative group.The degree of lower limb muscle weakness at admission is greater than that in the AIDP positive group;The increase in cerebrospinal fluid protein is more significant,the time required for muscle strength improvement is longer,and the lower limb muscle strength is lower at discharge(P<0.05).Antibody positive group The ΔHughes score was(1.04±0.80)points,while the antibody negative group was(1.69±0.48)points,and the difference met statistical standards(P<0.05).The incidence of seventh cranial nerve involvement was relatively higher in the AMAN group with positive AGAs compared to those with negative AGAs,and the difference met statistical standards(P<0.05).Conclusion The positive detection rate of antibodies against GM1 IgG,GQ1b IgG,and GD1a IgG is higher in the AMAN group.AIDP children with AGAs positive for this disease have a relatively severe condition,poorer prognosis,and AMAN children have a higher risk of seventh cranial nerve involvement.