Use of transcranial motor-evoked potentials to provide reliable intraoperative neuromonitoring for the Charcot-Marie-Tooth population undergoing spine deformity surgery

Purpose Intraoperative neuromonitoring (IONM) has historically been difficult to obtain in patients with Charcot-Marie-Tooth (CMT) disease. Transcranial motor-evoked potentials (TcMEPs) have been found to be safe and effective for other spinal deformity patients. Our objective was to determine the effectiveness of TcMEP monitoring in patients with CMT. Methods An IRB-approved, retrospective review of CMT patients undergoing spinal deformity surgery assessing TcMEP, somatosensory-evoked potential (SSEP), and neurogenic motor evoked potential (NMEP) IONM was performed. A 2:1 matched cohort control group of idiopathic spinal deformity patients was used. A waveform grading system was applied to review baseline TcMEP reliability and quality, which was validated via intraclass correlation coefficient amongst five raters. Results Twenty-three CMT patients (26 surgical cases) were identified. The use of TcMEP improved the ability to obtain baseline IONM when compared to SSEP (83% vs. 20%; p < 0.001) and NMEP (83% vs. 18%; p = 0.003). Baseline monitoring was obtained less often for CMT patients using SSEP (20% vs. 100%; p < 0.001) and TcMEP (83% vs. 100%; p = 0.111) compared to idiopathic patients. Sweep length (time from stimulation waveform evaluation) and maximum stimulation voltage were higher in the CMT group (289 ms vs. 111 ms p = 0.007 and 740 V vs. 345 V p = 0.089, respectively). Conclusion TcMEP monitoring significantly improves the ability to provide IONM for CMT patients undergoing spinal deformity surgery. Utilizing longer sweep lengths enhances the ability to attain baseline TcMEP readings, allowing surgeons to more safely proceed with surgery for these complex patients.