摘要目的:比较先天性多发性关节屈曲挛缩症（AMC）和青少年特发性脊柱侧凸（AIS）患者脊柱后路矫形术中神经电生理监测（IONM）结果并分析先天性脊柱畸形对AMC患者IONM的影响，评估IONM在AMC患者矫形手术中的应用价值。方法:横断面研究。回顾性分析2013年7月至2022年1月在南京鼓楼医院接受手术治疗的19例AMC患者的临床资料。选择同期就诊并手术治疗的年龄及弯型相似的57例女性AIS患者作为对照组。AMC组男13例，女6例，年龄（15.2±5.6）岁，术前主弯侧凸Cobb角为60.8°±27.7°；对照组年龄（14.6±4.4）岁，侧凸Cobb角为55.2°±14.2°。比较分析两组患者基线状态下体感诱发电位（SSEPs）、经颅电刺激躯体运动诱发电位（TCeMEPs）的潜伏期和波幅，并比较伴与不伴先天性脊柱畸形的AMC患者IONM结果的差异性。结果:19例AMC患者术中均成功获得SSEPs基线（100%），14例成功获得TCeMEPs基线。57例AIS患者均成功获得SSEPs及TCeMEPs监测基线。SSEPs-P40潜伏期、SSEPs-N50潜伏期、SSEPs-波幅、TCeMEPs-潜伏期、TCeMEPs-波幅在AMC与AIS患者间差异无统计学意义（均 P>0.05）。凹凸侧间TCeMEPs-波幅差值在AMC患者中较AIS患者高，但差异无统计学意义［（147.0±185.6）μV比（68.1±311.4）μV， P=0.198］。SSEPs-波幅在伴先天性脊柱畸形AMC患者的凹侧为（1.4±1.1）μV，在不伴先天性脊柱畸形AMC患者的凹侧为（2.6±1.2）μV；SSEPs-波幅在伴先天性脊柱畸形AMC患者的凸侧为（1.4±0.8）μV，在不伴先天性脊柱畸形AMC患者的凸侧为（2.6±1.3）μV，两组间差异均有统计学意义（均 P<0.05）。 结论:AMC患者的SSEPs-P40潜伏期、SSEPs-N50潜伏期、SSEPs-波幅、TCeMEPs-潜伏期、TCeMEPs-波幅与AIS患者相似。合并先天性脊柱畸形AMC患者的SSEPs-波幅较不合并先天性脊柱畸形的AMC患者有降低趋势。

abstractsObjective:To compare the intraoperative neurophysiological monitoring (IONM) results between patients with arthrogryposis multiplex congenita (AMC) and adolescent idiopathic scoliosis (AIS) and to analyze the influence of congenital spinal deformity on IONM in AMC patients, thus to evaluate the efficiency of IONM in AMC patients.Methods:A cross-sectional study. The clinical data of 19 AMC patients underwent correction surgery from July 2013 to January 2022 in Nanjing Drum Tower Hospital were retrospectively reviewed. There were 13 males and 6 females with a mean age of (15.2±5.6) years, and the average Cobb angle of main curve was 60.8°±27.7°. And 57 female AIS patients of similar age and curve type with the AMC patients during the same period were selected as the control group, with an average age of (14.6±4.4) years and a mean Cobb angle of 55.2°±14.2°. The latency and amplitude of samatosensory evoked potentials (SSEPs) and transcranial electric motor evoked potentials (TCeMEPs) were compared between the two groups. The difference in IONM data between AMC patients with and without congenital spinal deformity was also evaluated.Results:The success rates of SSEPs and TCeMEPs were 100% and 14/19 for AMC patients, 100% and 100% for AIS patients. The SSEPs-P40 latency, SSEPs-N50 latency, SSEPs-amplitude, TCeMEPs-latency, TCeMEPs-amplitude showed no significant difference between AMC patients and AIS patients ( P>0.05 for all). The side-difference of TCeMEPs-amplitude showed an increasing trend in AMC patients when compared with that in AIS patients, but there was no statistical difference between the two groups [(147.0±185.6) μV vs (68.1±311.4) μV, P=0.198]. The SSEPs-amplitude value was (1.4±1.1) μV on concave side in AMC patients with congenital spinal deformity, and it was (2.6±1.2) μV on concave side in AMC patients without congenital spinal deformity ( P=0.041). The SSEPs-amplitude value was (1.4±0.8) μV on convex side in AMC patients with congenital spinal deformity, and it was (2.6±1.3) μV on convex side in AMC patients without congenital spinal deformity ( P=0.028). Conclusions:The values of SSEPs-P40 latency, SSEPs-N50 latency, SSEPs-amplitude, TCeMEPs-latency and TCeMEPs-amplitude are similar in AMC and AIS patients. The SSEPs-amplitude of AMC patients with congenital spinal deformity is lower than that of AMC patients without congenital spinal deformity.