Nerve size correlates with clinical severity in Charcot-Marie-Tooth disease 1A

被引:17
作者
Zanette, Giampietro [1 ]
Tamburin, Stefano [2 ,3 ]
Taioli, Federica [2 ,3 ]
Lauriola, Matteo Francesco [1 ]
Badari, Andrea [1 ]
Ferrarini, Moreno [2 ,3 ]
Cavallaro, Tiziana [3 ]
Fabrizi, Gian Maria [2 ,3 ]
机构
[1] Pederzoli Hosp, Neurol Div, Verona, Italy
[2] Univ Verona, Dept Neurosci Biomed & Movement Sci, Piazzale Scuro 10, I-37134 Verona, Italy
[3] Dept Neurosci AOUI Verona, Neurol Div, Verona, Italy
关键词
clinical scores; cross sectional area; CSA; high resolution ultrasound; HRUS; inherited neuropathy; multivariate analysis; NCS; nerve conduction study; CMT NEUROPATHY SCORE; OUTCOME MEASURES; ULTRASOUND; DUPLICATION; RELIABILITY; MOTOR;
D O I
10.1002/mus.26688
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Introduction Nerve cross-sectional area (CSA) is larger than normal in Charcot-Marie-Tooth disease 1A (CMT1A), although to a variable extent. We explored whether CSA is correlated with CMT clinical severity measured with neuropathy score version 2 (CMTNS2) and its examination subscore (CMTES2) in CMT1A. Methods We assessed 56 patients with CMT1A (42 families). They underwent nerve conduction study (NCS) and nerve high-resolution ultrasound (HRUS) of the left median, ulnar, and fibular nerves. Results Univariate analysis showed NCS and HRUS variables to be significantly correlated with CMTNS2 and CMTES2 and with each other. Multivariate analysis showed that ulnar motor nerve conduction velocity (beta: -0.19) and fibular compound muscle action potential amplitude (-1.50) significantly influenced CMTNS2 and that median forearm CSA significantly influenced CMTNS2 (beta: 5.29) and CMTES2 (4.28). Discussion Nerve size is significantly associated with clinical scores in CMT1A, which suggests that it might represent a potential biomarker of CMT damage and progression.
引用
收藏
页码:744 / 748
页数:5
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