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1A型遗传性运动感觉神经病:体重指数对神经传导研究及遗传性运动感觉神经病检查评分的影响

Charcot-Marie-Tooth Disease Type 1A: Influence of Body Mass Index on Nerve Conduction Studies and on the Charcot-Marie-Tooth Examination Score.

作者信息

Jerath Nivedita U, Shy Michael E

机构信息

Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, Iowa, U.S.A.

出版信息

J Clin Neurophysiol. 2017 Nov;34(6):508-511. doi: 10.1097/WNP.0000000000000415.

Abstract

PURPOSE

Charcot-Marie-Tooth Disease type 1A (CMT1A) is caused by a duplication of the peripheral myelin protein gene 22 at chromosome 17p11.2-12. There is limited data regarding whether body mass index (BMI) affects electrophysiological or clinical data in those with CMT1A.

METHODS

Electrophysiological data, the Charcot-Marie-Tooth examination score (CMTES) and BMI from 101 patients with known CMT1A were obtained and analyzed.

RESULTS

When controlling for age, a higher BMI does not affect ulnar motor nerve conduction studies in those with CMT1A, but rather components of the CMTES (loss of pinprick and motor strength in the lower extremities).

CONCLUSIONS

BMI and clinical components of the CMTES are correlated, but it is uncertain which came first-whether the loss of lower extremity pinprick sensation and motor strength results in a higher BMI or if higher BMI results in these signs.

摘要

目的

1A型腓骨肌萎缩症(CMT1A)由17号染色体p11.2 - 12处外周髓磷脂蛋白基因22的重复所致。关于体重指数(BMI)是否会影响CMT1A患者的电生理或临床数据,相关资料有限。

方法

获取并分析了101例已知患有CMT1A患者的电生理数据、腓骨肌萎缩症检查评分(CMTES)及BMI。

结果

在控制年龄的情况下,较高的BMI对CMT1A患者的尺神经运动神经传导研究没有影响,但会影响CMTES的部分指标(下肢针刺觉丧失和肌力)。

结论

BMI与CMTES的临床指标相关,但尚不确定何者为先——是下肢针刺觉丧失和肌力下降导致较高的BMI,还是较高的BMI导致了这些体征。

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