Jankovic Joseph
Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA.
Lancet Neurol. 2006 Oct;5(10):864-72. doi: 10.1016/S1474-4422(06)70574-9.
Dystonia, defined as a neurological syndrome characterised by involuntary, patterned, sustained, or repetitive muscle contractions of opposing muscles, causing twisting movements and abnormal postures, is one of the most disabling movement disorders. Although gene mutations and other causes are increasingly recognised, most patients have primary dystonia without a specific cause. Although pathogenesis-targeted treatment is still elusive, the currently available symptomatic treatment strategies are quite effective for some types of dystonia in relieving involuntary movements, correcting abnormal posture, preventing contractures, reducing pain, and improving function and quality of life. A small portion of patients have a known cause and respond to specific treatments, such as levodopa in dopa-responsive dystonia or drugs that prevent copper accumulation in Wilson's disease. Therapeutic options must be tailored to the needs of individual patients and include chemodenervation with botulinum toxin injections for patients with focal or segmental dystonia, and medical treatments or deep brain stimulation for patients with generalised dystonia.
肌张力障碍被定义为一种神经综合征,其特征是拮抗肌出现不自主、模式化、持续性或重复性的肌肉收缩,导致扭曲动作和异常姿势,是最致残的运动障碍之一。尽管基因突变和其他病因越来越受到认可,但大多数患者患有原发性肌张力障碍,没有特定病因。尽管针对发病机制的治疗仍然难以实现,但目前可用的对症治疗策略对某些类型的肌张力障碍在缓解不自主运动、纠正异常姿势、预防挛缩、减轻疼痛以及改善功能和生活质量方面相当有效。一小部分患者有已知病因并对特定治疗有反应,如多巴反应性肌张力障碍中的左旋多巴或预防威尔逊病中铜蓄积的药物。治疗方案必须根据个体患者的需求进行调整,包括对局限性或节段性肌张力障碍患者进行肉毒毒素注射化学去神经支配,以及对全身性肌张力障碍患者进行药物治疗或深部脑刺激。