aDepartment of Neurology, Boston Children's Hospital bHarvard Medical School cDepartment of Neurology, Massachusetts General Hospital dDepartment of Neurosurgery, Boston Children's Hospital, Boston, Massachusetts, USA.
Curr Opin Pediatr. 2017 Dec;29(6):691-696. doi: 10.1097/MOP.0000000000000548.
Deep brain stimulation (DBS) has recently emerged as an important management option in children with medically refractory dystonia. DBS is most commonly used, best studied, and thought to be most efficacious for a select group of childhood or adolescent onset monogenic dystonias (designated with a standard 'DYT' prefix). We review how to clinically recognize these types of dystonia and the relative efficacy of DBS for key monogenic dystonias.
Though used for dystonia in adults for several years, DBS has only lately been used in children. Recent evidence shows that patients with shorter duration of dystonia often experience greater benefit following DBS. This suggests that early recognition of the appropriate dystonic phenotypes and consideration of DBS in these patients may improve the management of dystonia.
DBS should be considered early in patients who have medically refractory dystonia, especially for the monogenic dystonias that have a high response rate to DBS. It is important to differentiate between these monogenic dystonias and dystonias of other causes to properly prognosticate for these patients and to determine whether DBS is an appropriate management option.
深部脑刺激（DBS）最近已成为治疗药物难治性脑瘫患儿的重要治疗手段。DBS 主要用于治疗少数特定的儿童或青少年发病的单基因脑瘫（用标准的“DYT”前缀指定），其疗效已被广泛研究和认可。我们将对如何识别这些类型的脑瘫以及 DBS 对主要单基因脑瘫的相对疗效进行综述。
尽管 DBS 已用于成人脑瘫多年，但直到最近才开始用于儿童脑瘫患者。最近的证据表明，脑瘫病程较短的患者在接受 DBS 治疗后往往获益更大。这表明，早期识别出合适的脑瘫表型并考虑对这些患者进行 DBS 治疗可能会改善脑瘫的治疗效果。
对于药物难治性脑瘫患者，尤其是对 DBS 反应率较高的单基因脑瘫患者，应早期考虑 DBS 治疗。区分这些单基因脑瘫和其他原因引起的脑瘫对于正确预测这些患者的预后和确定 DBS 是否为合适的治疗方案非常重要。
