Tourette's syndrome and its borderland.

Tourette's syndrome and its borderland.
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DOI:
10.1136/practneurol-2017-001755
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发表时间:
2018-08-01
影响因子:
2.8
通讯作者:
Stern, Jeremy S
Stern, Jeremy S
中科院分区:
其他
文献类型:
--
作者:
Stern, Jeremy S

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抽动症(或抽动症)在儿童中的患病率为1%,严重程度广泛,并伴有相关的合并症。在过去的20年里,人们对该综合征复杂的遗传学和潜在的神经生物学的理解取得了进展。影像和神经生理学技术的研究表明,这是一种神经发育状况,伴有基底节-皮质相互作用的功能障碍,目前也在动物模型中进行研究。也有越来越多的证据表明可以进行治疗,尽管它往往仍然很难管理。一线选择包括神经镇静剂、其他药物和专门的行为治疗。脑深部刺激是一个不断发展的领域,尚未完全确立。本文就抽动的现象学、如何评估和处理抽动综合征,并以非典型病例为例,探讨抽动的临床特点和局限性。
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 20years 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.