Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, USA.
Mov Disord. 2011 May;26(6):1149-56. doi: 10.1002/mds.23618. Epub 2011 Apr 11.
Tourette syndrome is a common childhood-onset neurobehavioral disorder characterized by multiple motor and phonic tics affecting boys more frequently than girls. Premonitory sensory urges prior to tic execution are common, and this phenomenon helps to distinguish tics from other hyperkinetic movement disorders. Tourette syndrome is commonly associated with attention deficit hyperactivity disorder, obsessive-compulsive disorder, learning difficulties, and impulse control disorder. The pathophysiology of this complex disorder is not well understood. Involvement of basal ganglia-related circuits and dopaminergic system has been suggested by various imaging and postmortem studies. Although it is considered a genetic disorder, possibly modified by environmental factors, an intense search has thus far failed to find causative genes. Symptomatic treatment of tics chiefly utilizes various alpha adrenergic agonists, antidopaminergic drugs, topiramate, botulinum toxin, and deep brain stimulation. Habit reversal therapy and other behavioral approaches may be a reasonable option for some cases. Improved understanding of Tourette syndrome should lead to better symptomatic and more effective pathogenesis-targeted therapies.
妥瑞氏症候群是一种常见的儿童发病的神经行为障碍，其特征是多种运动和发声抽搐，男孩比女孩更为常见。抽搐发作前常有前驱感觉冲动，这一现象有助于将抽搐与其他运动过度障碍区分开来。妥瑞氏症候群常伴有注意缺陷多动障碍、强迫症、学习困难和冲动控制障碍。这种复杂疾病的病理生理学尚未完全理解。各种影像学和尸检研究表明，基底神经节相关回路和多巴胺能系统参与其中。尽管妥瑞氏症候群被认为是一种遗传疾病，可能受到环境因素的影响，但目前为止，尽管进行了深入的研究，仍未能找到致病基因。抽动的对症治疗主要采用各种α肾上腺素能激动剂、抗多巴胺药物、托吡酯、肉毒杆菌毒素和深部脑刺激。习惯逆转疗法和其他行为方法可能是某些病例的合理选择。对妥瑞氏症候群的认识的提高应能导致更好的对症治疗和更有效的针对发病机制的治疗。
