Comings D E, Comings B G
Department of Medical Genetics, City of Hope Medical Center, Duarte, Calif. 91010.
J Clin Psychiatry. 1990 Jul;51(7):275-80.
Gilles de la Tourette's syndrome (TS) is a common, hereditary neuropsychiatric disorder. While its diagnostic feature is the presence of suppressible motor and vocal tics, a wide range of impulsive, compulsive, attentional, learning, mood, and anxiety disorders are also present in many patients. To determine if attentional and learning problems are part of the expression of the Gts gene (or genes), the authors analyzed family histories of 130 TS probands with 1851 relatives and 25 control probands with 541 relatives--a total of 2392 relatives. The frequency of attention-deficit hyperactivity disorder (ADHD) or learning disorders was significantly increased in the relatives of the TS probands. The data on first-degree relatives suggest that when the Gts gene is expressed in this form, in two thirds of the cases tics are also present and in one third they are not. These observations are consistent with the proposal that ADHD and learning disorders form an integral part of the expression of the Gts gene or genes.
吉尔斯·德拉图雷特综合征(TS)是一种常见的遗传性神经精神疾病。虽然其诊断特征是存在可抑制的运动和发声抽动,但许多患者还存在广泛的冲动、强迫、注意力、学习、情绪和焦虑障碍。为了确定注意力和学习问题是否是Gts基因(或多个基因)表达的一部分,作者分析了130名TS先证者及其1851名亲属以及25名对照先证者及其541名亲属的家族史——总共2392名亲属。TS先证者亲属中注意力缺陷多动障碍(ADHD)或学习障碍的发生率显著增加。一级亲属的数据表明,当Gts基因以这种形式表达时,三分之二的病例也会出现抽动,三分之一则不会。这些观察结果与ADHD和学习障碍是Gts基因或多个基因表达不可或缺的一部分这一观点一致。