Stern Jeremy S
Pract Neurol. 2018 Aug;18(4):262-270. doi: 10.1136/practneurol-2017-001755. Epub 2018 Apr 10.
The Gilles de la Tourette syndrome (or Tourette's syndrome) has a prevalence of 1% of children with a wide range of severity and associated comorbidities. The last 20 years have seen advances in the understanding of the syndrome's complex genetics and underlying neurobiology. Investigation with imaging and neurophysiology techniques indicate it is a neurodevelopmental condition with dysfunction of basal ganglia-cortical interactions, which are now also being studied in animal models. There is also increasing evidence for treatments although it often remains difficult to manage. First-line options include neuroleptics, other drugs and specialised behavioural treatments. Deep brain stimulation is an evolving field, not yet fully established. This review focuses on the phenomenology of tics, how to assess and manage the syndrome, and uses examples of atypical cases to explore the characteristics and limits of its clinical spectrum.
抽动秽语综合征（或图雷特综合征）在儿童中的患病率为1%，严重程度和相关合并症范围广泛。在过去20年里，人们对该综合征复杂的遗传学和潜在神经生物学的认识有了进展。影像学和神经生理学技术研究表明，它是一种神经发育疾病，存在基底神经节 - 皮质相互作用功能障碍，目前动物模型也在对此进行研究。尽管治疗往往仍很困难，但治疗方法的证据也越来越多。一线治疗选择包括抗精神病药物、其他药物和专门的行为治疗。深部脑刺激是一个不断发展的领域，尚未完全确立。本综述重点关注抽动的现象学、该综合征的评估和管理方法，并通过非典型病例示例探讨其临床谱的特征和局限性。
