Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia
Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia
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DOI：
10.1111/dmcn.12117
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发表时间：
2013-06-01
影响因子：
3.8
通讯作者：
Lin, Jean-Pierre
中科院分区：
文献类型：
--
作者：
Lumsden, Daniel E.;Kaminska, Margaret;Lin, Jean-Pierre
本研究的目的是研究肌张力障碍的病因、持续时间、挛缩和深度脑刺激（DBS）手术的年龄对顽固性、致残性原发性、继发性静态或继发性进行性肌张力障碍（包括神经变性伴脑铁积累（NBIA））患儿预后的影响。方法采用BurkeFahnMarsden肌张力障碍评定量表（BFMDRS）对70例2005年6月至2011年7月连续接受DBS的儿童进行基线和术后6个月和12个月的肌张力障碍严重程度进行评估。结果2名儿童（3%）接受单侧DBS治疗半肌张力障碍，并被排除，5名儿童（7%）在6个月内发生感染，需要部分DBS切除，其余63名儿童（90%）接受双侧DBS随访（男性34名，女性29名；全组手术时平均年龄10y - 4mo， SD 4y - 2mo，范围114y）。17例患儿被分类为原发性肌张力障碍：平均年龄12岁11个月，平均年龄4岁6个月，平均年龄4岁6个月至17岁3个月；28例继发性静态肌张力障碍：平均年龄10岁2个月，平均年龄4岁9个月（范围3y - 3mo20）；5例为继发性进行性肌张力障碍：平均年龄8岁11个月，平均年龄3岁9个月（范围5y 5mo13y 1mo）；13例为NBIA性肌张力障碍：平均年龄10岁2个月，SD 3岁11个月（范围114y）。患有原发性肌张力障碍的儿童在BFMDRS运动评分上比其他病因类别的儿童表现出更大的改善(KruskalWallis测试，p
Aim The aim of this study was to examine the impact of dystonia aetiology and duration, contracture, and age at deep brain stimulation (DBS) surgery on outcome in a cohort of children with medically refractory, disabling primary, secondary-static, or secondary-progressive dystonias, including neurodegeneration with brain iron accumulation (NBIA). Method Dystonia severity was assessed using the BurkeFahnMarsden Dystonia Rating Scale (BFMDRS) motor score at baseline and 6 and 12months postoperatively in a cohort of 70 consecutive children undergoing DBS between June 2005 and July 2011. Results Two children (3%) received unilateral DBS for hemidystonia and were excluded and five (7%) developed infections requiring part-DBS removal within 6months, leaving 63 children (90%) undergoing bilateral DBS for follow-up (34 males, 29 females; mean age at surgery for the whole group 10y 4mo, SD 4y 2mo, range 114y). Seventeen children were classified with primary dystonia: mean age 12years 11months, SD 4years 6months range 4years 6months to 17years 3months; 28 as having secondary-static dystonia: mean age 10years 2months, SD 4years 9months (range 3y 3mo20y); five as having secondary-progressive dystonia: mean age 8years 11months, SD 3years 9months (range 5y 5mo13y 1mo); and 13 as having NBIA dystonia: mean age 10years 2months, SD 3years 11months (range 114y). Children with primary dystonias demonstrated greater improvements in BFMDRS motor score than those in the other aetiological categories (KruskalWallis test, p