感觉神经豁免模式在儿童免疫介导的急慢性炎性周围神经病中的诊断价值分析

Diagnostic value of sensory sparing patterns in childhood immune-mediated acute or chronic inflammatory polyneuropathy

目的探讨免疫介导的急慢性炎性周围神经病患儿感觉神经豁免模式的特点及意义。方法选择自2014年9月至2019年8月广州市第一人民医院儿科、神经内科收治或广州市儿童医院送至广州市第一人民医院肌电图室检查的免疫介导的吉兰-巴雷综合征(GBS)和慢性炎性脱髓鞘性多发性神经根神经病(CIDP)患儿(年龄<14岁)40例,归为免疫炎性组;同样来源的其他病因的多发性周围神经病患儿(年龄<14岁)15例归为其他病因组。对2组患儿神经传导速度测定中感觉神经受损的特点进行对比分析并观察是否存在腓肠神经豁免(SS)模式、桡神经豁免(RS)模式,以及感觉比率(SR)>1。SR=[腓肠神经感觉神经动作电位(SNAPs)+桡神经SNAPs]/(正中神经SNAPs+尺神经SNAPs)。结果(1)40例免疫炎性组患儿中,38例为GBS,2例为CIDP。38例GBS中34例为急性炎性脱髓鞘性多发神经根神经病(AIDP)型,3例为急性运动轴索性神经病(AMAN)型,1例为急性运动感觉轴索型。15例其他病因组患儿中,9例为腓骨肌萎缩症(CMT)1型,1例为CMT 2型,1例为CMT中间型,2例为遗传性压力易感性周围神经病,2例为线粒体病导致的多发周围神经受累。(2)与其他病因组患儿比较,免疫炎性组患儿出现SS(72.5% vs. 6.7%)、RS(42.5% vs. 6.7%)、SR>1(75.0% vs. 13.3%)的比例显著升高,差异有统计学意义(P<0.05)。SS模式在诊断儿童炎性脱髓鞘性周围神经病中的敏感性为0.73,特异性为0.93,阳性似然比为10.88。SR>1特异性相对较低(0.87),RS模式敏感性相对较低(0.41)。结论感觉神经豁免模式,特别是SS模式对儿童免疫介导的急慢性炎性周围神经病的诊断具有一定意义。

Objective To investigate the characteristics and significance of sural sparing pattern and radial sparing pattern in children immune-mediated acute or chronic inflammatory peripheral neuropathy. Methods Forty children with Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) aged<14 years, admitted to our hospital from September 2014 to August 2019, were chosen as immune-inflammatory group;15 children (<14 years old) with multiple peripheral neuropathy of other etiologies from the same source were classified as other etiologies group. The characteristics of sensory nerve damage measured by nerve conduction velocity in the two groups were compared. The existences of sural sparing (SS) pattern and radial sparing (RS) pattern, and sensory ratio (SR)>1 were conformed. SR=(sural nerve sensory nerve action potentials+radial nerve sensory nerve action potentials)/(median nerve sensory nerve action potentials+ulnar nerve sensory nerve action potentials). Results (1) In 40 patients from immune-inflammatory group, 38 were GBS and two were with CIDP;Among the 38 with GBS, 34 were with acute inflammatory demyelinating polyneuropathies (AIDP), 3 were with acute motor axonal neuropathy (AMAN), and one was with acute motor sensory axonal neuropathy. Among the 15 patients from the other etiologies group, 9 were with charcot-marie-tooth (CMT) type 1, one was with CMT type 2, one was with CMT intermediate type, and two were with hereditary neuropathy with liability to pressure palsies (HNPP), and two were with mitochondrial disease involves polyneuropathy.(2) As compared with patients from other etiologies group, patients from immune-inflammatory group had significantly higher positive rates of SS (72.5% vs. 6.7%), RS(42.5% vs. 6.7%) and SR>1 (75.0% vs. 13.3%). In the diagnoses of childhood acute or chronic inflammatory polyneuropathy, the sensitivity of SS pattern was 0.73, specificity was 0.93, and positive likelihood ratio was 10.88. SR>1 had moderate specificity (0.87) and low sensitivity (0.41). Conclusion The sensory sparing patterns, especially SS pattern, have valuable clinical significance in the diagnoses of acute or chronic inflammatory peripheral neuropathy in children.