粘多糖贮积症中三种常用的电生理评估:荟萃分析  

Three Commonly Used Electrophysiological Assessments in Mucopolysaccharidosis (MPS): A Meta-Analysis

作　　者：康琳敏 Elizabeth Haley Charles Wessel 林煌 Catherine Palmer 

机构地区：[1]匹兹堡大学传播科学与疾病系,美国匹兹堡 [2]匹兹堡大学健康科学图书馆系统,美国匹兹堡 [3]匹兹堡大学生物统计学系,美国匹兹堡 [4]匹兹堡大学医学中心(UPMC),美国匹兹堡

出　　处：《临床医学进展》2019年第1期67-79,共13页Advances in Clinical Medicine

摘　　要：目的:三种电生理检查通常用于辅助诊断与粘多糖贮积症相关的神经异常,包括:神经传导速度,体感诱发电位和听觉脑干反应。这项荟萃分析报告了基于现有文献的一项估计,即单独或组合中的哪些电生理检测具有更高的检测粘多糖贮积症神经生理异常的可能性。方法:在PubMed (1946~现在)中进行系统搜索,其中包括MEDLINE和Embase.com (1974~现在)。结果:纳入10项研究进行荟萃分析。通过这三项试验检测到的粘多糖贮积症中神经生理异常的比例如下:神经传导速度为84% (95% CI = 0.63~0.94),体感诱发电位为63% (95% CI = 0.39~0.82), 听觉脑干反应为9% (95% CI = 0.00~0.94)。结论:本研究发现测试方式(即神经传导速度,体感诱发电位或听觉脑干反应)和粘多糖贮积症亚型种类能够用于解释研究中的神经异常率差异,但发表年份和患者年龄则不能。我们仍需进一步的研究来确认是否联合使用这些测试会比单独使用这些测试中的任何一个能给出对粘多糖贮积症中神经生理异常更为敏感的诊断。意义:这是第一个通过神经传导速度,体感诱发电位和/或听觉脑干反应测量来描述粘多糖贮积症中神经生理异常比例的荟萃分析。Objective: Three electrophysiological tests are commonly used for the auxiliary diagnosis of neural abnormalities associated with Mucopolysaccharidosis (MPS) including: Nerve Conduction Velocity (NCV), Somatosensory Evoked Potential (SEP) and Auditory Brainstem Response (ABR). This meta-analysis reports an estimate from existent literature of which of these electrophysiological test(s) in isolation or in combination has/have a higher likelihood of detecting neurophysiological abnormalities in Mucopolysaccharidosis. Methods: A systematic search in PubMed (1946-2017) which includes MEDLINE and Embase.com (1974-2017) was conducted. Results: Ten studies were included for meta-analysis. The proportion of neurophysiological abnormalities in MPS in general, as detected by these three tests, was as follows: NCV was 84% (95% CI = 0.63 - 0.94), SEP was 63% (95% CI = 0.39 - 0.82), and the ABR was 9% (95% CI = 0.00 - 0.94). Conclusions: Test modalities (i.e., NCV, SEP and ABR) and MPS subtypes, but not publication year and age, were found to account for the neural abnormality rate differences across studies. Further studies are needed to confirm whether using some combination of these tests will be more sensitive to neurophysiologic abnor-malities than any one of these tests in isolation. Significance: This is the first meta-analysis to de-scribe the proportion of neurophysiological abnormalities in MPS measured via NCV, SEP and/or ABR.

关 键 词：粘多糖贮积症 听觉脑干反应 神经传导速度 体感诱发电位 电生理学 

分 类 号：R5[医药卫生—内科学]