肌电图肌腱反射记录：一种诊断1A型夏科-马里-图思病的准确且舒适的方法。

Electromyographic tendon reflex recording: An accurate and comfortable method for diagnosis of charcot-marie-tooth disease type 1a.

作者信息

García Antonio, Pelayo-Negro Ana L, Álvarez-Paradelo Silvia, Antolín Francisco M, Berciano José

机构信息

Service of Clinical Neurophysiology, University Hospital Marqués de Valdecilla, Instituto de Investigación Marqués de Valdecilla, University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas, Santander, Spain.

Service of Neurology, University Hospital Marqués de Valdecilla, Instituto de Investigación Marqués de Valdecilla, University of Cantabria and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas, Santander, Spain.

出版信息

Muscle Nerve. 2015 Jul;52(1):39-44. doi: 10.1002/mus.24499. Epub 2015 May 14.

DOI:10.1002/mus.24499

PMID:25363904

Abstract

INTRODUCTION

We analyzed the utility of tendon reflex (T-reflex) testing in Charcot-Marie-Tooth disease type 1A (CMT1A).

METHODS

A total of 82 subjects from 27 unrelated CMT1A pedigrees were evaluated prospectively. The series also comprised 28 adult healthy controls. Electrophysiology included evaluation of biceps T-reflex and soleus T-reflex.

RESULTS

Seventy-one individuals (62 adults and 9 children) had clinical and electrophysiological features of CMT1A. The remaining 11 (8 adults and 3 children) were unaffected. On electrophysiological testing, the biceps T-reflex was elicited in 58 of 62 (93%) adult CMT1A patients and in all 9 affected children. Latencies of the biceps T-reflex were always markedly prolonged, and a cut-off limit of 16.25 ms clearly separated adult patients and controls or unaffected kin adult individuals. In affected children, the soleus T-reflex latency was also prolonged when compared with age and height normative data.

CONCLUSION

T-reflex testing is an accurate diagnostic technique for CMT1A patients.

摘要

引言

我们分析了腱反射（T反射）测试在1A型遗传性运动感觉神经病（CMT1A）中的效用。

方法

对来自27个无关CMT1A家系的82名受试者进行了前瞻性评估。该系列还包括28名成年健康对照者。电生理检查包括对肱二头肌T反射和比目鱼肌T反射的评估。

结果

71人（62名成年人和9名儿童）具有CMT1A的临床和电生理特征。其余11人（8名成年人和3名儿童）未受影响。在电生理测试中，62名成年CMT1A患者中的58名（93%）和所有9名受影响儿童均可引出肱二头肌T反射。肱二头肌T反射的潜伏期总是明显延长，16.25毫秒的截止值可清楚地区分成年患者与对照者或未受影响的亲属成年个体。在受影响的儿童中，与年龄和身高的正常数据相比，比目鱼肌T反射潜伏期也延长。