Introduction

Charcot Marie tooth disease (CMTD) is also known as Hereditary sensory motor neuropathy. It poses difficulties in attaining intra-operative neuromonitoring signals for deformity correction surgery. In this case report, we intent to mention key points for obtaining good neuromonitoring signals in these cases which increases the safety in scoliosis surgery.

Case presentation

We present a 14-year-old boy, known case of CMTD, presented with progressive deformity of the back. The child was wheelchair-bound and could walk only a few steps with support. He was unable to maintain a sitting balance without using upper limbs making him functionally quadriparatic. The radiographs showed a double scoliotic curve with costo-pelvic impingement. At the onset, no signals were obtained with routine intra-operative neuromonitoring settings.

Discussion

Increasing the sweep length and voltage in our neuro-monitors helped in acquiring the baseline signals and we went ahead to proceed the deformity correction.

中文翻译：

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经颅运动诱发电位 (TcMEP) 在腓骨肌萎缩症脊柱畸形手术中的应用——我们应该知道什么？案例报告

 介绍

夏科玛丽牙病 (CMTD) 也称为遗传性感觉运动神经病。这给畸形矫正手术中获得术中神经监测信号带来了困难。在本病例报告中，我们打算提及在这些病例中获得良好神经监测信号的关键点，从而提高脊柱侧弯手术的安全性。

 案例展示

我们介绍了一名 14 岁男孩，已知患有 CMTD 病例，表现为背部进行性畸形。孩子坐轮椅，靠搀扶只能走几步。如果不使用上肢，他就无法保持坐姿平衡，这使得他在功能上四肢瘫痪。 X光片显示双脊柱侧凸并伴有肋骨骨盆撞击。开始时，常规术中神经监测设置没有获得信号。

 讨论

增加神经监视器中的扫描长度和电压有助于获取基线信号，我们继续进行畸形矫正。