Houeto Jean-Luc, Giré Pauline
Service de neurologie et CMRR, CHU de Poitiers, F-86021 Poitiers Cedex, France.
Presse Med. 2008 Feb;37(2 Pt 2):263-70. doi: 10.1016/j.lpm.2007.11.007. Epub 2008 Jan 10.
The term "Tourette syndrome" designates the combination of tics with other symptoms. Gilles de la Tourette disease is one of its most frequent causes. It combines motor and vocal tics, with no identifiable cause, with self-mutilation and variable psychiatric comorbidity that may include obsessive-compulsive disorder (OCD) and other anxiety disorders, mood and personality disorders, and a syndrome of hyperactivity with attention disorders. The prevalence of Tourette syndrome is estimated at 0.1-1% of the general population. The condition begins during childhood and develops in a succession of periods of relative aggravation and remission of the tics. Most patients show improvement at the end of adolescence, but symptoms can persist into adulthood in approximately one third of patients. The cause of Gilles de la Tourette disease is unknown, but the role of genetic susceptibility has been suggested together with dysfunctions of the dopaminergic system and of neuron networks in associative and limbic areas of the basal ganglia and the prefrontal cortex. Treatment of Tourette syndrome and severe tics is often difficult and requires a multidisciplinary approach (neurologist, psychiatrist, psychologist and social workers). In mild forms, information and psychological management are usually recommended. Drug treatments--including neuroleptics--are essential in the moderate to severe forms of the disease. Psychiatric comorbidities, when present, often justify specific treatment. For the most severe forms of Gilles de la Tourette disease, preliminary results of treatment by deep brain stimulation of the associative and limb areas of the thalamus or pallidum have produced real hope of treatment, but nonetheless require confirmation.
“抽动秽语综合征”一词指抽动症状与其他症状的组合。吉尔斯·德拉图雷特病是其最常见的病因之一。它合并了运动性和发声性抽动,病因不明,还伴有自残行为以及多种精神共病,可能包括强迫症(OCD)和其他焦虑症、情绪及人格障碍,以及伴有注意力障碍的多动综合征。抽动秽语综合征在普通人群中的患病率估计为0.1% - 1%。该病始于儿童期,抽动症状会在一系列相对加重和缓解的阶段中发展。大多数患者在青春期结束时症状会有所改善,但约三分之一的患者症状会持续至成年期。吉尔斯·德拉图雷特病的病因尚不清楚,但已有人提出遗传易感性的作用,同时还涉及多巴胺能系统以及基底神经节和前额叶皮质联合区与边缘区的神经网络功能障碍。抽动秽语综合征和严重抽动的治疗通常很困难,需要多学科方法(神经科医生、精神科医生、心理学家和社会工作者)。对于轻度病例,通常建议提供信息和进行心理管理。药物治疗——包括抗精神病药物——对于中度至重度病例至关重要。存在精神共病时,往往需要进行特定治疗。对于最严重的吉尔斯·德拉图雷特病病例,通过对丘脑或苍白球的联合区和边缘区进行深部脑刺激治疗取得的初步结果带来了真正的治疗希望,但仍需进一步证实。