双侧丘脑底核刺激治疗1型脑铁沉积神经变性病
Bilateral subthalamic nucleus stimulation in the treatment of neurodegeneration with brain iron accumulation type 1.
作者信息
Ge Ming, Zhang Kai, Ma Yu, Meng Fan-gang, Hu Wen-han, Yang An-chao, Zhang Jian-guo
机构信息
Neurosurgical Department, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
出版信息
Stereotact Funct Neurosurg. 2011;89(3):162-6. doi: 10.1159/000323374. Epub 2011 Apr 14.
BACKGROUND
Neurodegeneration with brain iron accumulation type 1 (NBIA1), previously called Hallervorden-Spatz disease, is a rare neurodegenerative condition with abnormal brain iron accumulation. There have been some reports of deep brain stimulation (DBS) in the treatment of NBIA1. However, the target was usually the globus pallidus internus or thalamus.
OBJECTIVES/METHODS: We present a case of NBIA1 in a 16-year-old male who was treated with bilateral subthalamic nucleus (STN)-DBS and explored its efficacy in the treatment of NBIA1. The patient presented with severe generalized dystonia and marked dysarthria and had previously had unsuccessful ablation surgery. MRI confirmed the diagnosis of NBIA1.
RESULTS
He had significant improvement postoperatively, and the benefit of surgery was maintained for 3 years during follow-up. Burke-Fahn-Marsden Dystonia Rating Scale score was 114/120 preoperatively, and dropped to 60/120 at 14 days, 35/120 at 1 month, 28/120 at 3 months, 14/120 at 1 year and 18/120 at 3 years postoperatively.
CONCLUSIONS
Our results suggest that bilateral STN-DBS might be considered as an effective treatment for selective NBIA1 patients.
背景
1型脑铁沉积神经变性病（NBIA1），以前称为Hallervorden-Spatz病，是一种罕见的神经退行性疾病，伴有异常的脑铁沉积。已有一些关于深部脑刺激（DBS）治疗NBIA1的报道。然而，靶点通常是苍白球内侧部或丘脑。
目的/方法：我们报告一例16岁男性NBIA1患者，接受双侧丘脑底核（STN）-DBS治疗，并探讨其治疗NBIA1的疗效。该患者表现为严重的全身性肌张力障碍和明显的构音障碍，此前接受的毁损手术未成功。MRI确诊为NBIA1。
结果
他术后有显著改善，随访期间手术效果维持了3年。术前伯克-法恩-马斯登肌张力障碍评定量表评分为114/120，术后14天降至60/120，1个月时为35/120，3个月时为28/120，1年时为14/120，3年时为18/120。
结论
我们的结果表明，双侧STN-DBS可能被视为选择性NBIA1患者的有效治疗方法。
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