难治性抽动秽语综合征的临床特点

目的探讨难治性Tourette综合征(Tourettesyndrom,TS)的临床特征。方法采用自制TS一般情况调查表、YGTSS(耶鲁综合抽动严重程度量表)、CBCL(Achenbach儿童行为量表)、WISC—CR(韦氏儿童智力量表中国修订版)对32例难治性TS(难治性组)患者和随机抽取的31例普通TS患者(普通组)分别进行测试和对照分析。结果(1)难治性TS患者在发病年龄、病前诱因、病程等方面与普通TS患者之间存在显著差异(P<0.05,P<0.01)。(2)难治性组CBCL总分显著高于普通组(P<0.01);难治性组19例6～11岁男性与普通组21例6～11岁男性CBCL测验比较,前者交往不良、强迫行为等6个行为问题因子得分显著高于后者(P<0.05,P<0.01),其CBCL总分也显著高于后者(P<0.01)。(3)在智力测验中,难治性组不仅其VIQ、PIQ、FIQ均显著低于普通组(P<0.05,P<0.01),而且在10项分测验中,算术分测验、理解分测验和译码分测验量表分均显著低于普通组。结论难治性TS具有自己的临床特征。     

A case of Tourette syndrome treated with nifedipine

A 22-year-old man with severe Tourette syndrome successfully treated with nifedipine is described. Controlled studies are called for.     

Clinical course of Tourette syndrome

Objective

Tourette syndrome (TS) is a childhood-onset neuropsychiatric disorder characterized by multiple motor and vocal tics lasting at least a year in duration. Children with TS often experience comorbid conditions such as obsessive-compulsive disorder (OCD) and attention-deficit disorder. The goal of this article was to review the long-term clinical course of tics and comorbid conditions in children with TS.

Method

We conducted a traditional literature search to locate relevant articles regarding long-term outcome and prognosis in TS and tic disorders.

Results

Tics typically have an onset between the ages of 4 and 6 years and reach their worst-ever severity between the ages of 10 and 12 years. On average, tic severity declines during adolescence. By early adulthood, roughly three-quarters of children with TS will have greatly diminished tic symptoms and over one-third will be tic free. Comorbid conditions, such as OCD and other anxiety and depressive disorders, are more common during the adolescence and early adulthood of individuals with TS than in the general population.

Conclusion

Although tics are the sine qua non of TS, they are often not the most enduring or impairing symptoms in children with TS. Measures used to enhance self-esteem, such as encouraging strong friendships and the exploration of interests, are crucial to ensuring positive adulthood outcome in TS.     

感觉症状与Tourette综合征预后的关系

目的：分析Tourette综合征感觉症状对预后的影响.方法：随机选取Tourette综合征男性患儿120例,使用感觉性症状问卷进行评定,其中伴有感觉症状的患儿66例,不伴感觉症状的患儿54例,采用耶鲁抽动障碍严重程度量表（YGTSS）、抑郁自评量表（SDS）以及焦虑自评量表（SAS）评定,使用氟哌啶醇治疗4周后再次评定.结果：不伴有感觉症状的Tourette综合征患儿较伴有感觉症状的患儿抽动症状好转明显（P＜0.01）;抑郁自评、焦虑自评严重程度轻.结论：Tourette综合征常伴有各种感觉症状,重视感觉症状将对疗效可产生有利的影响.     

24小时动态脑电图监测Tourette综合征癫痫样放电

目的：探讨Ｔｏｕｒｅｔｔｅ综合征（ＴＳ）癫痫样脑波的是临床意义。方法：利用动态脑电图仪对８６例ＴＳ患儿作２４小时监测。结果：８６例ＴＳ患儿中有１３例（１５．１％）监测到ＥＥＧ癫痫样放电。其中５例（６％）合并癫痫,８例（９．３％）无癫痫发作。结论：ＴＳ的癫痫样波一部分与癫痫有关,另一部分意义未明,可能遗传有关。     

Tourette syndrome in the elderly

An 82-year-old housewife presented with motor and phonic tics and coprolalia. These had developed in the months following an acute confusional state associated with posterior neck pain 10 years previously. Neurological examination, including MRI scan, was otherwise normal. The index illness was presumed to be encephalitis lethargica.     

Functional neuroimaging in Tourette syndrome

Functional neuroimaging of neuropsychiatric disorders is a complex discipline requiring skills in medical science, philosophy, and technical physics. This review first examines the broad categories of functional imaging studies that have been utilized in this area, comparing the strengths and weaknesses of each approach. This review then looks at much of the available literature on functional imaging in Tourette syndrome (TS) and provides a synthesis of data. The review will also examine the different methodologies employed and will suggest which methodologies are most likely to lead to elucidation of the pathophysiology of TS and related conditions.     

Tourette syndrome and comorbid early-onset schizophrenia

Objective

A study of the shared phenomenology between Tourette syndrome (TS) and schizophrenia.

Method

An illustrative case report is presented. We used a chart review of 399 clinically ascertained patients with TS to identify 10 cases meeting criteria for schizophrenia. From our 10 patients, salient clinical characteristics were then tabulated. We then extracted similar clinical characteristics from a previously published series of patients with comorbid TS and schizophrenia in order to combine cases and allow for a comparison between childhood-onset schizophrenia (COS), adolescent-onset schizophrenia (AdolOS), and adult-onset schizophrenia (AduOS) cases in these groups.

Results

We found 10 cases of schizophrenia (all were males) in the 399 TS patients for a prevalence rate of 2.5% (95% CI 0.96–4.04). Mean age of tic onset for TS diagnostic criteria ranged from 2-14 years with a mean of 8.2 years. The mean age of diagnosis for schizophrenia was 14.2 (range 9–23 years). We found six cases of schizophrenia with onset of positive psychotic symptoms by 13 years of age, two cases with onset after 13 years of age and before 18 years of age, and two cases with onset after 18 years of age. Attention deficit hyperactivity disorder was present at a higher rate (70%) than one would expect in a clinically ascertained group of patients with TS. Comparison between COS, AdolOS and AduOS in our pooled cases noted a sex bias skewed toward males. Catatonic symptoms may be more likely in child or adolescent onset cases and negative symptoms more likely in AduOS cases.

Conclusions

The 2.5% prevalence of schizophrenia in our TS sample exceeds the 1% expected rate of schizophrenia in the general population (chi-square=9.14; P=.0025). The six cases of COS (before 13 years of age) exceeds the expected rate of 1–2 per 100,000 (chi-square=4499; P=.0001). The 752-fold increase in observed rates of comorbid TS and COS over expected rates suggests a role for unknown common underlying etiologic factors. Based on clinical features, patients with TS and comorbid COS, AdolOS, or AduOS do not have different conditions. We conclude with suggestions for further research.     

Electrophysiological measures and dual-task performance in Tourette syndrome indicate deficient divided attention mechanisms

Tourette syndrome has been associated with impairments of attentional functions such as distractability, even in subjects without co-morbid attention deficit hyperactivity disorder. Based on the results of earlier research we hypothesized that Tourette syndrome patients might employ altered control mechanisms of attentional processes and have concurrent difficulties in allocating their attentional resources among competing tasks. Event-related brain potentials (ERPs) were recorded in a group of Tourette syndrome patients and in a matched control group during a dual task experiment. This experiment required the simultaneous detection of visual and auditory target stimuli which were manipulated to yield two different difficulty levels each of which were varied orthogonally. The behavioural parameters confirmed the intended performance differences between difficult-to-detect targets and easy-to-detect targets. This was paralleled by lower amplitudes and longer latencies of the corresponding P3b-ERP subcomponents. Although Tourette syndrome patients were unimpaired in overall performance they showed an increased interference of visual task demands with auditory target perception. In parallel they also exhibited a reduced amplitude of the P3b component to auditory targets. The findings show that Tourette syndrome patients are not generally impaired in their dual task performance. The allocation of attentional resources to competing tasks however, is altered. We speculate that this may be related to deficient inhibitory functions.     

Tourette综合征患者的智力水平研究

目的测试TS患者的智力水平及分测验结构.方法采用韦氏儿童智力量表(C-WISC)对64例患儿和60例正常小儿进行智力评估.结果 Tourette综合征患者的智商多在正常范围或边缘水平,除词汇、知识外,其余各分测验及PIQ、VIQ、FIQ均显著低于对照组(P＜0.05),PIQ与VIQ之间的平衡性较差.结论Tourette综合征患者的智商较正常少儿平均水平为低,且智力发展不平衡者较多.     

Malignant Tourette syndrome.

The aim of this work was to draw attention to potentially life-threatening symptoms associated with Tourette syndrome (TS) and to explore their relationship to TS comorbidities. Medical records of all patients with TS evaluated at our Movement Disorders Clinic between July 2003 and July 2006 were reviewed. Data on patients with malignant TS, defined as >or=2 emergency room (ER) visits or >or=1 hospitalizations for TS symptoms or its associated behavioral comorbidities, were entered into a dataset and analyzed. Five illustrative cases are described. Of 333 TS patients evaluated during the 3-year period, 17 (5.1%) met the criteria for malignant TS. Hospital admission or ER visits were for tic-related injuries, self-injurious behavior (SIB), uncontrollable violence and temper, and suicidal ideation/attempts. Compared with patients with nonmalignant TS, those with malignant TS were significantly more likely to have a personal history of obsessive compulsive behavior/disorder (OCB/OCD), complex phonic tics, coprolalia, copropraxia, SIB, mood disorder, suicidal ideation, and poor response to medications. Although TS is rarely a disabling disorder, about 5% of patients referred to a specialty clinic have life-threatening symptoms. Malignant TS is associated with greater severity of motor symptoms and the presence of >or=2 behavioral comorbidities. OCD/OCB in particular may play a central role in malignant TS; obsessive compulsive qualities were associated with life-threatening tics, SIB, and suicidal ideation. Malignant TS is more refractory to medical treatment than nonmalignant TS.     

抽动秽语综合征患者基底节结构特点分析

目的探讨抽动秽语综合征(Tourette syndrome,TS)患者可能存在的基底节结构改变.方法对10例右利手男性TS患者和10例匹配正常健康人进行头颅MRI检查,应用三维重建技术分别对尾状核、壳核、苍白球和全脑体积进行测量,比较两组之间的基底节体积差异,分析左右侧结构体积的对称性.结果对照组左侧基底节体积大于右侧(P＜0.05);TS组双侧基底节各结构体积均明显减小,左侧更显著,左右侧体积比较无显著差异(P＞0.05);基底节各结构体积与症状严重程度不相关.结论正常右利手人群中存在着基底节结构体积不对称性,左侧偏大;双侧基底节体积减小、丧失正常的不对称性,是TS患者的特征性结构改变.     

感觉症状对Tourette综合征疗效的影响

目的 探讨感觉症状对Tourette综合征疗效的影响.方法 随机选取男性Tourette综合征患儿100例,采用自编抽动障碍感觉性症状问卷进行评定,其中伴有感觉症状的56例,不伴感觉症状的44例,采用耶鲁抽动障碍严重程度量表(YGTSS)分别于治疗前及使用氟哌啶醇治疗后第1、第4及第8周末进行评定.结果 伴有感觉症状的Tourette综合征患儿治疗后YGTSS减分低于不伴有感觉症状的患儿,有极显著性差异(P＜0.01);结果 显示,伴有感觉症状的Tourette综合征患儿疗效较差.结论 Tourette综合征常伴有各种感觉症状,且感觉症状对疗效有不利的影响.     

利培酮治疗Tourette综合征临床研究

目的 :探讨利培酮治疗 Tourette综合征 ( TS)的疗效及安全范围。　方法 :采用耶鲁TS症状严重程度量表和不良反应症状量表进行评定 ,并作实验室监测 ,对 3 7例 TS患者进行 8周的利培酮治疗研究。　结果 :64 .9%的病人疗效显著 ,86.5%症状改善。平均显效时间 ( 2 8.0± 1 1 .7)天 ,有效日剂量为 ( 2 .5± 0 .8) mg,副反应少而轻微。　结论 :利培酮有望成为治疗 TS的良好药物。     

利培酮治疗Tourette综合征对照研究

目的:进一步验证利培酮治疗Tourette综合征的疗效和不良反应. 方法:60例Tourette综合征患者分为两组,每组30例,分别给予利培酮和氟哌啶醇治疗.疗程6周.治疗前和治疗6周分别进行耶鲁综合抽动严重性量表(YGTSS)和副反应量表(TESS)评定疗效和不良反应. 结果:两组治疗后YGTSS总分和各维度评分均显著低于治疗前,利培酮组显著高于氟哌啶醇组,利培酮组显效率为67.9%明显低于氟哌啶醇组96.7%;利培酮组不良反应明显小于氟哌啶醇组. 结论:利培酮和氟哌啶醇均能有效治疗Tourette综合征,但利培酮疗效不及氟哌啶醇,利培酮不良反应少而轻.     

Blink reflex abnormalities in children with Tourette syndrome

Tourette syndrome (TS) is a common disorder which typically occurs during childhood or early adolescence. There is no definitive diagnostic test for TS. The objective of this study was to demonstrate whether neurophysiological abnormalities of the blink reflex can be observed in children with TS. We enrolled 15 children with TS, diagnosed according to DSM IV Diagnostic Criteria, and 15 controls. The blink reflex was elicited by stimulating the supraorbital nerve in order to measure the early response (R1), homolateral and contralateral R2 (late) responses, amplitude of R1 and duration of R2. The mean duration of R2 was significantly longer in TS patients than in the controls ( P  < 0.001, Student's t -test). An abnormal pattern of the blink reflex can be, even in childhood, an early neurophysiologic marker of TS, which is not related to the duration of TS or to the age of onset.     

药物难治性抽动秽语综合征的立体定向手术治疗

目的 总结立体定向一侧丘脑腹外侧核(VL)/板中间核(Ud)加ZI区毁损联合双侧扣带回前部(AC)毁损治疗药物难治性抽动秽语综合征(TS)的经验.方法 回顾性分析36例TS的临床资料,术前均行脑电图、脑影像学、脑PET等检查,抽动情况以耶鲁抽动量表(YGTSS)评价,强迫观念程度以耶鲁-布朗强迫量表(YBOCS)评价....     

Clinical characteristics of Tourette syndrome

Abstract The aim of the present study was to examine the clinical characteristics of Tourette syndrome (TS) in terms of symptomatology, outcome and organic and genetic factors, and to compare these with results of previous studies on TS in Western countries and in Japan, on the basis of precise information taken from a large number of TS patients under psychiatric treatment in Japan. There was a total of 64 TS patients (55 males and nine females) selected from those who visited our outpatient clinic from 1974 to 1993 and were found to meet the criteria for Tourette's disorder of DSM-III-R. The mean patient age at entry to the present study was 17.4 years (SD:7.2). All data were collected through a systematic chart review of subjects, including data on tic symptoms and the course of their development; complications and developmental histories; family histories; medical and psychological examinations; treatment; severity and outcome. A check of the data showed that the mean age at onset was 6.9 years (SD:2.7). An analysis of the progression of the symptoms revealed that 'generalized tics' afflicting the entire body were found in 64.1% of subjects and coprolalia was found in 50%. The main complications were obsessive-compulsive symptoms (OCS) in 62.5% of patients and attention deficit hyperactivity disorder (ADHD) in 17.2%. Of their parents, 7.0% had tic disorders except TS and 1.6% had TS. Contrary to results from previous studies of TS, our study revealed that at least the incidence of coprolalia in TS patients in Japan is not lower than in Western countries. However, the frequency of familial cases seemed to be lower than previously reported for Western patients. Outcome was fairly related with 'generalized tics,' OCS, aggressiveness and ADHD.     

Gilles de la Tourette syndrome

The study of childhood movement disorders is still in an early stage. Tics and related disorders are recognized as one of the most common movement disorders. This contribution reviews the main clinical, epidemiological, pathophysiological, and treatment issues on tics and Gilles de la Tourette syndrome. Although these disorders are not life threatening, they may be psychologically or functionally disabling. Early diagnosis and special management permit the alleviation of symptoms. Received: 1 June 1998 Accepted: 27 June 1998