Mehrkens Jan Hinnerk, Borggraefe Ingo, Feddersen Berend, Heinen Florian, Bötzel Kai
Department of Neurosurgery, Klinikum Grosshadern, Ludwig-Maximilians-University, Munich, Germany.
J Child Neurol. 2010 Nov;25(11):1355-61. doi: 10.1177/0883073810365369. Epub 2010 May 5.
Primary generalized dystonia presents mainly at a young age and commonly is severely disabling. The authors report the long-term follow-up (mean, 73 months; range, 50-101 months) of 5 pediatric patients (mean age at surgery 13 years; range, 8-16 years) undergoing globus pallidus internus deep brain stimulation. Mean improvement in the Burke-Fahn-Marsden movement score was 67.4% (range, 47.0%-87.5%), 75.4% (range, 61.5%-91.7%), and 83.5% (range, 72.0%-93.3%) at 3 months, 12 months, and long-term follow-up (>36 months), respectively. Hardware problems (electrode dislocation/breakage of extension cable, and imminent perforation of extension cable) were observed in 2 patients (operative revision without sequelae). Except for mild dysarthria in 2 patients, no other therapy-related morbidity was observed. The authors found globus pallidus internus stimulation to offer a very effective and safe therapy in pediatric patients with primary dystonia. Early neurosurgical intervention seems to be crucial to prevent irreversible impairment of motor function.
原发性全身性肌张力障碍主要在年轻时发病，通常会导致严重的功能障碍。作者报告了5例接受苍白球内侧部脑深部电刺激的儿科患者（手术时平均年龄13岁；范围8 - 16岁）的长期随访结果（平均73个月；范围50 - 101个月）。在3个月、12个月和长期随访（>36个月）时，伯克 - 法恩 - 马斯登运动评分的平均改善率分别为67.4%（范围47.0% - 87.5%）、75.4%（范围61.5% - 91.7%）和83.5%（范围72.0% - 93.3%）。2例患者出现硬件问题（电极移位/延长电缆断裂以及延长电缆即将穿孔）（进行了手术修复，无后遗症）。除2例患者有轻度构音障碍外，未观察到其他与治疗相关的并发症。作者发现苍白球内侧部刺激对原发性肌张力障碍儿科患者是一种非常有效且安全的治疗方法。早期神经外科干预对于预防运动功能的不可逆损害似乎至关重要。
