慢性炎性脱髓鞘性多发性神经病郎飞结/结旁疾病患者中枢神经和自主神经受累临床特征分析

目的分析慢性炎性脱髓鞘性多发性神经病郎飞结/结旁疾病患者中枢神经系统受累(central nervous system involvement,CNSI)和自主神经损伤(autonomic nerve injury,ANI)的特点,评估其电生理检查的亚临床损害特征。方法回顾性分析2018年9月至2021年5月北京天坛医院确诊的10例郎飞结/结旁疾病患者的临床特点、功能评分和电生理检查,分别评估瞬目反射(blink reflex,BR)、面神经传导(facial nerve conduction,FNC)、交感皮肤反应(sympathetic skin response,SSR)和心率变异性(heart rate variation,HRV)对CNSI以及ANI的评估价值。结果CNSI组MRC总分(MRC-sum score)显著低于非中枢神经系统受累(non-CNS involvement,NCNSI)组(33.5分比55.8分,P=0.016),CNSI组总体神经病变限制性量表(overall neuropathy limitation scale,ONLS)评分和改良Rankin量表(modified Rankin scale,MRS)评分显著高于NCNSI组(5.5分比1.8分,P=0.015;3.5分比1.1分,P=0.005)。临床症状与BR Kappa系数为0.20(P=0.350),临床症状与FNC Kappa系数为0.18(P=0.440),临床症状与SSR检查Kappa系数为–0.54(P=0.100),临床症状与HRV检查Kappa系数为0.10(P=0.760)。年龄与口轮匝肌潜伏期、SSR下肢潜伏期和最大值/最小值(E/I)具有相关性(r=–0.740,P=0.009;r=0.615,P=0.011;r=–0.779,P=0.013)。病程与E/I、SSR上肢潜伏期和下肢潜伏期具有相关性(r=0.722,P=0.028;r=–0.548,P=0.019;r=–0.695,P=0.003)。13项小纤维神经病和症状问卷(small fiber neuropathy and symptoms inventory questionnaire,SFN–SIQ)与SSR下肢潜伏期和下肢波幅具有相关性(r=0.781,P=0.022;r=–0.878,P=0.004)。结论郎飞结/结旁疾病患者CNSI和ANI并不少见,CNSI临床症状更重,运动功能损害更明显,BR和FNC可反映CNSI亚临床损害特征,SSR可反映ANI并评估其严重程度,下肢检测异常更具敏感性。

Left paraduodenal hernia in an adult complicated by ascending colon cancer: A case report

Paraduodenal hernia is the most common internal hernia. The clinical symptoms of paraduodenal hernia may be intermittent and nonspecific. Therefore, it is difficult to diagnose preoperatively. Abdominal computed tomography （CT） scan currently plays an important role in the evaluation and management of paraduodenal hernia before surgical operation. We report one unique case of preoperatively diagnosed left paraduodenal hernia complicated by advanced ascending colon cancer and reviews of Japanese literature.