摘要
目的探讨抽动-秽语综合征(GillesdelaTourette’ssyndrome,TS)的临床特点。方法对1983年2月~2001年5月诊治的605例抽动-秽语综合征患者的临床资料进行总结和分析,并对其中500例进行随访调查。结果605例患者的平均发病年龄为7.7岁,男女之比4∶1;病程1~30年;其中102例(16.9%)有抽动症家族史;172例(28.4%)患者伴有性格行为问题;62.9%(112/178)的患者脑电图存在异常波形。CT检查显示,抽动-秽语综合征患者可有轻微异常(7/67例),但检出率较低;SPECT检查表明,患者额、颞、顶叶等处存在脑血流灌注减低现象(5/5例)。病程与患者发病年龄呈负相关(r=-0.191,P<0.01),与秽语发生年龄呈正相关(r=0.189,P<0.05)。结论抽动-秽语综合征多于儿童期发病,男性较女性多见;具有遗传倾向;可能存在轻微脑功能异常;发病年龄与秽语出现年龄对病程有一定的影响。
Objective To study the clinical characteristics of Gilles de la Tourette's syndrome (TS).Methods The clinical data of605patients with Gilles de la Tourette's syndrome diagnosed in this depart-ment during February1983to May2001were reviewed and analyzed.The patients with the following condi-tions eg.chorea secondary to Huntington's disease,hepatolenticular degeneration,and postencephalitis were excluded in this study.Of the605patients500cases were followed up.Results In these patients,the av-erage age at onset of TS was7.7years,male∶female was4∶1,and disease courses ranged from1to30years.There were102cases(16.9%)with family history of convulsion,172patients(28.4%)with problems of behav-ior and character,112/178cases(62.9%)with abnormal electroencephalogram(EEG).Minimal abnormalities such as widened longitudinal and Sylvian fissures,bilateral basal ganglion calcification,frontal lobe atrophy or enlarged lateral ventricles were found in few patients(7/67)by CT scan.Brain SPECT scans showed perfu-sion decrease in frontal,temporal and parietal lobe in5/5cases.The disease courses were negatively corre-lated with the age at onset of TS(r=?0.148,P<0.01),positively correlated with the age at onset of coprolalia(r=0.189,P<0.05).Con clu sion The TS is occurred mostly in childhood with hereditary tendency and more males than females are affected.Minimal brain dysfunction may be existed.The courses of TS are associated with age at onset and coprolalia.[