Hospital Clínic, University of Barcelona, Barcelona, Spain.
J Neurol Neurosurg Psychiatry. 2010 Jan;81(1):65-9. doi: 10.1136/jnnp.2009.174342. Epub 2009 Sep 10.
Dystonia is a complex clinical syndrome originated by a wide range of aetiologies. The diagnosis of dystonia is made after the evaluation of aetiological, phenomenological and genetic factors. Medications, except in patients with dopa-responsive dystonia, are of limited efficacy. Botulinum toxin injections are not applicable to patients with generalised dystonia, since many muscular groups contribute to disability. Clinical studies in children and adults with primary generalised dystonia (PGD) have reported beneficial effects of bilateral GPi deep brain stimulation (DBS) in both motor symptoms and disability produced by dystonia as well as a favourable impact of DBS in the health-related quality of life (HRQoL). Some clinical aspects of GPi stimulation in primary dystonia still remain controversial such as the influence of disease duration or age at onset in determining the postoperative clinical outcome.
The authors report the results of a multicentric study designed to assess the tolerability and clinical effects of bilateral pallidal DBS on motor impairment, functional disability, quality of life, pain and mood in patients with medically refractory primary generalised or segmental dystonia.
肌张力障碍是一种由多种病因引起的复杂临床综合征。肌张力障碍的诊断是在评估病因、表型和遗传因素后做出的。药物治疗除了对多巴反应性肌张力障碍患者有效外，对大多数患者疗效有限。由于许多肌肉群都会导致残疾，肉毒毒素注射不适用于全身性肌张力障碍患者。在儿童和成人原发性全身性肌张力障碍（PGD）的临床研究中，双侧苍白球深部脑刺激（DBS）对运动症状和由肌张力障碍引起的残疾均有显著疗效，且 DBS 对健康相关生活质量（HRQoL）也有积极影响。一些关于原发性肌张力障碍中苍白球刺激的临床方面仍然存在争议，例如疾病持续时间或发病年龄对术后临床结果的影响。
作者报告了一项多中心研究的结果，该研究旨在评估双侧苍白球 DBS 对药物难治性原发性全身性或节段性肌张力障碍患者运动障碍、功能障碍、生活质量、疼痛和情绪的耐受性和临床效果。
