Abstract
PurposeLittle is known about the reliability and value of intraoperative neurophysiological monitoring (IONM) in patients with Duchenne muscular dystrophy (DMD) undergoing scoliosis correction surgery. The aim of this study was to investigate the feasibility of IONM and the cortical excitability in these patients.MethodsFifteen patients with DMD and scoliosis and 15 patients with adolescent idiopathic scoliosis (AIS) underwent scoliosis correction surgery with the use of IONM. IONM consisted of transcranial electrical stimulation motor evoked potential (Tc-MEP) and somatosensory evoked potential (SSEP) monitoring. The highest Tc-MEP amplitudes were collected to test the feasibility. Preoperative compound muscle action potentials (CMAPs) and transcranial magnetic stimulation (TMS)-MEPs were recorded to test the cortical excitability. SSEPs were scored as elicitable or not elicitable.ResultsTc-MEP amplitudes were significantly lower in the DMD group for both the gastrocnemius and tibialis anterior muscles. However, the abductor hallucis muscle had similar amplitudes in both the DMD as the AIS group. TMS/CMAP and Tc-MEP/CMAP ratios were similar in the DMD and AIS group (P = 0.126 and P = 0.792 respectively).ConclusionsTc-MEP and SSEP monitoring is feasible, particularly when Tc-MEPs are recorded from the abductor hallucis muscle in patients with DMD. Similar TMS/CMAP and Tc-MEP/CMAP ratios show that there were no differences observed in cortical excitability between the groups. IONM seems a feasible and valuable neurophysiological tool to signal possible surgically induced damage to the spinal cord during scoliosis correction surgery in patients with DMD.
Highlights
Duchenne muscular dystrophy (DMD), a recessive X-linked muscle disease, is the most common of the muscular dystrophies
In all 15 patients with DMD (100%), transcranial electrical stimulation motor evoked potential (Tc-MEP) were elicitable during surgery
We investigated the feasibility of intraoperative neurophysiological monitoring (IONM) and the cortical excitability in patients with DMD and scoliosis and compared the findings with those from patients with adolescent idiopathic scoliosis (AIS)
Summary
Duchenne muscular dystrophy (DMD), a recessive X-linked muscle disease, is the most common of the muscular dystrophies. It is caused by mutations in the dystrophin gene which encodes dystrophin, an important structural component of muscle tissue. Loss of dystrophin causes progressive muscle weakness, with the proximal muscles more affected than the distal muscles [1]. Patients are at risk of developing scoliosis due to weakness of the spinal muscles in combination with rapid growth of the spinal column. Lebel et al followed up patients with DMD for 15 years and found that 20% develop scoliosis and need spinal deformity surgery [2]. The risks of surgery for these patients are, significant, and the decision
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