• Giampietro Zanette, Stefano Tamburin, Federica Taioli, Matteo Francesco Lauriola, Andrea Badari, Moreno Ferrarini, Tiziana Cavallaro, and Gian Maria Fabrizi.
• Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy.
• Muscle Nerve. 2019 Dec 1; 60 (6): 744-748.

IntroductionNerve 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.MethodsWe 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.ResultsUnivariate 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 (β: -0.19) and fibular compound muscle action potential amplitude (-1.50) significantly influenced CMTNS2 and that median forearm CSA significantly influenced CMTNS2 (β: 5.29) and CMTES2 (4.28).DiscussionNerve size is significantly associated with clinical scores in CMT1A, which suggests that it might represent a potential biomarker of CMT damage and progression.© 2019 Wiley Periodicals, Inc.

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