1Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi.
2Department of Neurology, All India Institute of Medical Sciences, New Delhi; and.
Neurosurg Focus. 2022 Oct;53(4):E12. doi: 10.3171/2022.7.FOCUS22332.
Dystonias are relatively rare disorders characterized by sustained or intermittent muscle contractions causing abnormal movements or postures. Generalized dystonia is a therapeutic challenge because medications are unable to control dystonia adequately in most patients. These patients may be candidates for surgical therapy. The commonly used surgical procedures in these patients are pallidotomy and deep brain stimulation. Limited studies are available on the role of pallidotomy in children with acquired/heredodegenerative generalized dystonia. The objective of this study was to describe the authors' experience with bilateral pallidotomy in this group of patients.
The authors retrospectively reviewed all pediatric patients (less than 18 years of age) with acquired/heredodegenerative generalized dystonia who underwent bilateral simultaneous pallidotomy at their center between January 2014 and January 2021. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores before and after surgery were recorded. Complications arising after the procedure were recorded as well.
Ten patients (8 male and 2 female) with a mean (range) age of 11.1 (5-17) years were included in this study. The mean duration between disease onset and surgical intervention was 3.9 years. Two patients presented in status dystonicus. The mean ± SD (range) preoperative BFMDRS score of the patients without status dystonicus (n = 8) was 80 ± 18.9 (59.5-108). The mean ± SD BFMDRS score at the time of discharge from the hospital after surgery was 58.8 ± 37.9. Three patients had more than 20% change in BFMDRS score at the time of discharge from the hospital. The mean improvement was 25.5% at the end of 1 year. Of 5 surviving patients in the non-status dystonicus group, 3 patients had more than 40% change in BFMDRS score while the other 2 patients developed recurrence at the last follow-up (4.5 years). Status dystonicus abated after bilateral pallidotomy in both patients. Permanent bulbar complications were seen in 2 patients.
Bilateral pallidotomy may result in clinically significant improvement in children with acquired/heredodegenerative generalized dystonia, although the benefits should be closely weighed against the risk of irreversible bulbar dysfunction. It is a viable option for children in resource-limited settings.
肌张力障碍是一种相对罕见的疾病，其特征为持续或间歇性肌肉收缩导致异常运动或姿势。全身性肌张力障碍是一种治疗挑战，因为大多数患者的药物无法充分控制肌张力障碍。这些患者可能是手术治疗的候选者。这些患者中常用的手术方法是苍白球切开术和深部脑刺激术。关于苍白球切开术在获得性/遗传性退行性全身性肌张力障碍儿童中的作用，仅有有限的研究。本研究的目的是描述作者在该组患者中进行双侧苍白球切开术的经验。
作者回顾性分析了 2014 年 1 月至 2021 年 1 月期间在他们的中心接受双侧同时苍白球切开术的所有患有获得性/遗传性退行性全身性肌张力障碍的儿科患者（年龄小于 18 岁）。记录手术前后 Burke-Fahn-Marsden 肌张力障碍评定量表（BFMDRS）评分。还记录了手术后出现的并发症。
本研究纳入了 10 名患者（8 名男性和 2 名女性），平均年龄（范围）为 11.1（5-17）岁。从疾病发作到手术干预的平均时间为 3.9 年。2 名患者出现张力障碍危象。无张力障碍危象的 8 名患者（n=8）术前 BFMDRS 评分的均值±标准差（范围）为 80±18.9（59.5-108）。术后出院时 BFMDRS 评分的均值±标准差为 58.8±37.9。出院时有 3 名患者的 BFMDRS 评分变化超过 20%。在 1 年结束时，平均改善率为 25.5%。非张力障碍危象组中，5 名存活患者中有 3 名患者的 BFMDRS 评分变化超过 40%，而另外 2 名患者在最后一次随访（4.5 年）时复发。双侧苍白球切开术后张力障碍危象均缓解。2 名患者出现永久性球部并发症。
双侧苍白球切开术可能会使获得性/遗传性退行性全身性肌张力障碍儿童的临床症状显著改善，尽管应密切权衡手术的获益与不可逆球部功能障碍的风险。对于资源有限的地区的儿童来说，这是一种可行的选择。
