Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy.
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy; Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy.
Clin Neurophysiol. 2018 Nov;129(11):2259-2267. doi: 10.1016/j.clinph.2018.08.016. Epub 2018 Sep 1.
Ulnar/median motor nerve conduction velocity (MNCV) is ≤38 m/s in demyelinating Charcot-Marie-Tooth disease (CMT). Previous nerve high resolution ultrasound (HRUS) studies explored demyelinating CMT assuming it as a homogeneous genetic/pathological entity or focused on CMT1A.
To explore the spectrum of nerve HRUS findings in demyelinating CMTs, we recruited patients with CMT1A (N = 44), CMT1B (N = 9), CMTX (N = 8) and CMT4C (N = 4). They underwent nerve conduction study (NCS) and HRUS of the median, ulnar, peroneal nerve, and the brachial plexus.
Median, ulnar and peroneal MNCV significantly differed across CMT subtypes. Cross sectional area (CSA) was markedly and diffusely enlarged at all sites, except entrapment ones, in CMT1A, while it was slightly enlarged or within normal range in the other CMTs. No significant right-to-left difference was found. Age had limited effect on CSA. CSAs of some CMT1A patients largely overlapped with those of other demyelinating CMTs. A combination of three median CSA measures could separate CMT1A from other demyelinating CMTs.
Nerve HRUS findings are heterogeneous in demyelinating CMTs.
Nerve HRUS may separate CMT1A from other demyelinating CMTs. The large demyelinating CMTs HRUS spectrum may be related to its pathophysiological variability.
脱髓鞘性夏科-马里-图思病（CMT）的尺神经/正中神经运动神经传导速度（MNCV）≤38m/s。之前的神经高分辨率超声（HRUS）研究探索了脱髓鞘性 CMT，认为其是一种均质的遗传/病理实体，或专注于 CMT1A。
为了探索脱髓鞘性 CMT 神经 HRUS 结果的范围，我们招募了 CMT1A（N=44）、CMT1B（N=9）、CMTX（N=8）和 CMT4C（N=4）患者。他们接受了正中神经、尺神经、腓总神经和臂丛神经的神经传导研究（NCS）和 HRUS。
CMT 亚型之间正中神经、尺神经和腓总神经的 MNCV 有显著差异。除卡压部位外，CMT1A 所有部位的横截面积（CSA）均显著弥漫性增大，而其他 CMT 则略增大或在正常范围内。未发现明显的左右差异。年龄对 CSA 的影响有限。一些 CMT1A 患者的 CSA 与其他脱髓鞘性 CMT 患者的 CSA 有很大重叠。三种正中神经 CSA 测量值的组合可将 CMT1A 与其他脱髓鞘性 CMT 区分开来。
脱髓鞘性 CMT 神经 HRUS 结果具有异质性。
神经 HRUS 可将 CMT1A 与其他脱髓鞘性 CMT 区分开来。较大的脱髓鞘性 CMT HRUS 谱可能与其病理生理变异性有关。
