Charcot-Marie-Tooth disease type 1A: Longitudinal change in nerve ultrasound parameters.

Authors :: Kojima Y
Noto YI
Tsuji Y
Kitani-Morii F
Shiga K
Mizuno T
Nakagawa M
Source :: Muscle & nerve [Muscle Nerve] 2020 Dec; Vol. 62 (6), pp. 722-727. Date of Electronic Publication: 2020 Sep 27.
Publication Year :: 2020
Abstract: Background: This study aimed to elucidate the longitudinal changes in nerve ultrasound parameters of adult Charcot-Marie-Tooth disease type 1A (CMT1A) patients.<br />Methods: Fifteen adult patients with CMT1A prospectively underwent nerve ultrasound and clinical assessment (CMT neuropathy score [CMTNS]) at baseline and 5 y later. Nerve cross-sectional area (CSA) and echogenicity were measured in the median and sural nerves. Changes in ultrasound parameters and CMTNS and correlation between changes of ultrasound parameters and CMTNS were analyzed.<br />Results: Median and sural nerve CSAs did not change over 5 y, although CMTNS increased (P < .01). Nerve echogenicity in the sural nerve decreased over 5 y (P = .045). No correlations between changes in nerve ultrasound parameters and CMTNS were identified.<br />Conclusions: No longitudinal changes in nerve size was detected in adult CMT1A. Exploring the factors that determine nerve size in childhood CMT1A may lead to the development of treatments.<br /> (© 2020 Wiley Periodicals LLC.)

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