Kothari M J, Bauer S B, Logigian E L, Shefner J M
Division of Neurology, Brigham and Women's Hospital, Boston, MA 02115, USA.
Muscle Nerve. 1995 Dec;18(12):1373-7. doi: 10.1002/mus.880181205.
Children with myelodysplasia often develop tethering of their spinal cord within 2-3 years after correction of their initial birth defect, leading to either neurological or urological dysfunction. Surveillance of these children includes electrophysiologic/urodynamic (EMG/UDS) studies to monitor improvement or deterioration of their neurological function. We reviewed 39 patients who underwent surgical untethering from 1979 to 1994 to determine whether postoperative EMG/UDS studies were useful in assessing outcome after surgery. All patients had a complete neurologic examination and an EMG/UDS prior to and 3 months after surgery. Twenty patients (51%) showed improvement in their EMG/UDS study after untethering, 15 (39%) demonstrated no significant change, and 4 (10%) showed deterioration. Improvement consisted of either return of previously absent motor units, return of sacral reflexes, or improved bladder dynamics. In a further 3 patients, subsequent deterioration on serial postoperative EMG/UDS testing was the first indication of recurrent tethering. We conclude that serial EMG/UDS evaluation in patients at risk for tethering of the spinal cord may serve as a useful adjunct to close clinical observation.
患有脊髓发育异常的儿童在最初出生缺陷矫正后的2至3年内,常常会出现脊髓栓系,进而导致神经或泌尿系统功能障碍。对这些儿童的监测包括进行电生理/尿动力学(EMG/UDS)检查,以监测其神经功能的改善或恶化情况。我们回顾了1979年至1994年间接受手术松解栓系的39例患者,以确定术后的EMG/UDS检查是否有助于评估手术效果。所有患者在手术前及术后3个月均接受了全面的神经系统检查和EMG/UDS检查。20例患者(51%)在松解栓系后EMG/UDS检查显示有所改善,15例(39%)无显著变化,4例(10%)显示恶化。改善表现为先前缺失的运动单位恢复、骶反射恢复或膀胱动力学改善。另有3例患者,术后连续的EMG/UDS检查显示病情恶化,这是脊髓栓系复发的首个迹象。我们得出结论,对有脊髓栓系风险的患者进行连续的EMG/UDS评估,可作为密切临床观察的有益辅助手段。