Cif Laura, Coubes Philippe
Unités de Neurochirurgie Fonctionnelle et Pédiatrique, Département de Neurochirurgie, Centre Hospitalier Universitaire Montpellier, France; Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), France; Institut de Génomique Fonctionnelle, Centre National de la Recherche Scientifique, Unité Mixte de la Recherche 5203, France; Université Montpellier, 34000, Montpellier, France; Laboratoire de Recherche en Neurosciences Cliniques (LRENC), France.
Unités de Neurochirurgie Fonctionnelle et Pédiatrique, Département de Neurochirurgie, Centre Hospitalier Universitaire Montpellier, France; Unité de Recherche sur les Comportements et Mouvements Anormaux (URCMA), France; Institut de Génomique Fonctionnelle, Centre National de la Recherche Scientifique, Unité Mixte de la Recherche 5203, France; Université Montpellier, 34000, Montpellier, France.
Eur J Paediatr Neurol. 2017 Jan;21(1):109-117. doi: 10.1016/j.ejpn.2016.08.010. Epub 2016 Sep 7.
Heterogeneous by the underlying pathobiology and clinical presentation, childhood onset dystonia is most frequently progressive, with related disability and limitations in functions of daily living. Consequently, there is an obvious need for efficient symptomatic therapies.
Following lesional surgery to basal ganglia (BG) and thalamus, deep brain stimulation (DBS) is a more conservative and adjustable intervention to and validated for internal segment of the globus pallidus (GPi), highly efficient in treating isolated "primary" dystonia and associated symptoms such as subcortical myoclonus. The role of DBS in acquired, neurometabolic and degenerative disorders with dystonia deserves further exploration to confirm as an efficient and lasting therapy. However, the pathobiological background with distribution of the sequellae over the central nervous system and related clinical features, will limit DBS efficacy in these conditions. Cumulative arguments propose DBS in severe life threatening dystonic conditions called status dystonicus as first line therapy, irrespective of the underlying cause. There are no currently available validated selection criteria for DBS in pediatric dystonia. Concurrent targets such as subthalamic nucleus (STN) and several motor nuclei of the thalamus are under exploration and only little information is available in children. DBS programming in paediatric population was adopted from experience in adults. The choice of neuromodulatory DBS parameters could influence not only the initial therapeutic outcome of dystonic symptoms but also its maintenance over time and potentially the occurrence of DBS related side effects.
DBS allows efficient symptomatic treatment of severe dystonia in children and advances pathophysiological knowledge about local and distributed abnormal neural activity over the motor cortical-subcortical networks in dystonia and other movement disorders.
儿童期起病的肌张力障碍在潜在病理生物学和临床表现上具有异质性，大多呈进行性发展，伴有相关残疾和日常生活功能受限。因此，显然需要有效的对症治疗方法。
在对基底神经节（BG）和丘脑进行病灶性手术后，深部脑刺激（DBS）是一种更为保守且可调节的干预措施，已在苍白球内侧部（GPi）得到验证，在治疗孤立的“原发性”肌张力障碍及相关症状（如皮质下肌阵挛）方面效率很高。DBS在后天性、神经代谢性和退行性肌张力障碍疾病中的作用值得进一步探索，以确认其作为一种有效且持久的治疗方法。然而，病理生物学背景以及后遗症在中枢神经系统的分布和相关临床特征，将限制DBS在这些情况下的疗效。越来越多的论据支持在称为肌张力障碍状态的严重危及生命的肌张力障碍情况下，将DBS作为一线治疗方法，而不论其潜在病因如何。目前尚无针对儿童肌张力障碍DBS的有效选择标准。诸如丘脑底核（STN）和丘脑的几个运动核团等并行靶点正在研究中，而关于儿童的信息很少。儿科人群的DBS程控是借鉴成人经验。神经调节性DBS参数的选择不仅会影响肌张力障碍症状的初始治疗效果，还会影响其长期维持情况，并可能影响DBS相关副作用的发生。
DBS可有效对症治疗儿童严重肌张力障碍，并推进了对肌张力障碍和其他运动障碍中运动皮质 - 皮质下网络局部和分布式异常神经活动的病理生理学认识。
