【摘要】 目的探讨不同类型儿童吉兰-巴雷综合征(GBS)的临床表现、神经电生理特征以及免疫治疗的效果。方法回顾性分析昆明医科大学附属儿童医院2015-2020年住院诊治的40例GBS患儿,其中37例纳入本研究。根据神经电生理检查结果分为两个类型,即脱髓鞘型和轴突型,并分析两个类型患儿的临床表现、神经电生理特征和免疫治疗的预后。结果根据临床和神经电生理进行分组分型,其中脱髓鞘组26例,均为急性炎性脱髓鞘性多发神经根神经病(AIDP);轴突组11例,其中急性运动轴突性神经病(AMAN)8例,急性运动感觉轴突性神经病(AMSAN)3例。两组间发病年龄、性别、感觉异常、首发症状、肌张力以及脑脊液蛋白-细胞分离比较差异均无统计学意义(P>0.05)。轴突型颅神经损伤发生率、肌力≤3级以及Hughes评分>3分的患儿数量均较高,差异有统计学意义(P<0.05)。对两个类型随访时反映运动神经功能的指标(DML、dCMAP、MCV、F波潜伏期和出波率)均有不同程度的改善,差异有统计学意义(P<0.05)。与第一次检查相比,脱髓鞘型感觉神经电生理指标异常率增高是一个例外。脱髓鞘型dCMAP的恢复较轴突型快,而轴突型的DML、MCV、F波潜伏期及出波率恢复较脱髓鞘型快。在应用丙种球蛋白(IVIG)治疗后,两种类型GBS在肌力恢复和Hughes评分方面比较差异均无统计学意义(P>0.05)。结论轴突型GBS较脱髓鞘型GBS更易引起颅神经损伤和肢体运动功能障碍。两种类型GBS在应用IVIG治疗后,运动功能恢复无差异。神经电生理检查可以为GBS的诊断、分型和治疗提供重要的依据。更多还原

【Abstract】 Objective To investigate the clinical manifestations,neuro-electrophysiological featg Medical University from 2015 to 2020 were collected and analyzed,of which only 37 wereures,and efficacy of immunotherapy of children’s different types of Guillain Barrésyndrome(GBS).Method 40 cases of GBS pediatric patients treated in Kunming Children’s Hospital Affiliated to Kunmin available for our research.All of the 40 cases were divided into two groups based on the results of nerve-electrophysiological examinations,the demyelinating group(n=26)and the axonal group(n=11).The clinical performance,neuroelectrophysiological characteristics,and immunotherapy prognosis of the two groups were subsequently analyzed.Results According to the clinical and electrophysiological findings,all the 26 cases in the demyelinating group suffered from manifested acute inflammatory demyelinating polyneuropathies(AIDP)while the axonal group included 8 cases with acute motor axonal neuropathy(AMAN)and 3 cases with acute motor-sensory axonal neuropathy(AMSAN).There was no statistical difference in age,gender,paresthesia,initial symptoms,muscle tension,and CSF proteincell separation between the two groups(P>0.05).Compared with the demyelinating group,the incidence of cranial nerve injury,the number of children with muscle strength≤3 and Hughes score>3 in the axonal group were higher,and the differences were statistically significant(P<0.05).The indexes of the two groups with GBS(such as the latency and wave-outgoing rates of DML,dCMAP,MCV and F-wave)showed that motor nerve function was improved to different degrees,and there were statistically significant differences(P<0.05).Compared with the initial examination,there was an exception that the abnormal rate of sensory nerve electrophysiological indexes increased in the demyelinating group.The recovery of dCMAP in demyelinating GBS was faster but the other indicators(the latency and wave-outgoing rates of DML,dCMAP,MCV and F-wave)were slower than in the axonal group.After the treatment of IVIG,the recovery of motor functions were similar in the two groups with GBS(P>0.05).Conclusion Compared with demyelinating GBS,axonal GBS is more likely to cause cranial nerve injury and limb motor dysfunction in children.Under the same treatment(IVIG),there was no difference in the recovery of motor functions between the patients with axonal and demyelinating types.Electrophysiological examination can provide an important evidence for the diagnosis,classification and treatment of GBS.更多还原