110例Tourette综合征的临床与脑电图相关性分析

本文报告１１０例Ｔｏｕｒｅｔｔｅ综合的临床和脑电图资料，结果发现：多发性不自主抽动与不自主发声的严重程度之间无相关性；秽语的出现与抽动和发声的严重程度间有一定相关性，秽语与模仿言语的发生间联系较密切；ＥＥＧ异常率为４２．７３％，其异常程度与抽动和发声的严重程度间无相关性，但秽语的出现与ＥＥＧ异常相关。     

抽动-秽语综合征单胺类神经递质与AEEG、SPECT的研究

抽动秽语综合征单胺类神经递质与ＡＥＥＧ、ＳＰＥＣＴ的研究文霞吕冰清金丽娟抽动秽语综合征（ＴＳ）发病学机制不十分清楚，有研究表明与单胺类神经递质代谢异常有关。我们测定了ＴＳ患者单胺类神经递质代谢产物，并进行２４小时动态脑电图描记术（ＡＥＥＧ）和单光...     

托吡酯治疗难治性抽动-秽语综合征临床观察

目的：探讨托吡酯对难治性抽动－秽语综合征的临床疗效及其副反应。方法：对33例既往使用其他药物治疗无效的抽动－秽语综合征，改用氟哌啶醇或泰必利联合托吡酯治疗，并比较其疗前疗后的耶鲁综合抽动严重程度量表（YGTSS）及副反应量表（TESS）得分。结果：疗后YGTSS量表中运动、发声及损害因子分较疗前显著降低，疗后发声与运动亦有显著好转，无明显副反应。结论：托吡酯对抽动－秽语综合征有效，对发声的疗效优于对运动障碍的疗效。     

抽动——秽语综合征临床及预后

抽动＿秽语综合征临床及预后山东省济宁市精神病防治院（２７２１５１）刘立芬陈洪王健苏中华抽动＿秽语综合征是儿童较常见慢性神经精神障碍之一，Ｉｔａｒｄ于１８２５年首先报告。本症是以多发性运动性抽动伴有不自主发声为主要特征［１］。虽然本症少见，但它对儿童的...     

抽动障碍临床、24小时脑电监测和SPECT的研究

目的探讨抽动－秽语综合征（ＴＳ）和慢性运动性抽动障碍（ＣＭＴＤ）的关系及两者脑电图、局部脑血流（ｒＣＢＦ）的异常。方法对３８例ＴＳ和２５例ＣＭＴ进行临床症状和２４小时脑电监测（ＡＥＥＧ）及部分患儿用单光子发射计算机断层扫描（ＳＰＥＣＴ）观察ｒＣＢＦ的变化。结果ＴＳ的临床症状较ＣＭＴＤ严重，两者ＡＥＥＧ异常率分别为５０％和５２％，均以额区明显，５例有痫样放电。ＴＳ和ＣＭＴＤ的ｒＣＢＦ异常呈降低或增加改变，波及范围广泛，与ＡＥＥＧ异常部位无一致性。结论ＣＭＴＤ是ＴＳ的临床表型，两者的脑电图和ｒＣＢＦ异常与癫痫相似，提示与癫痫有一定关系。     

抽动秽语综合征的临床特征分析

目的探讨抽动秽语综合征(Tourette‘s syndrome，TS)的临床特征。方法对69例抽动秽语综合征患者的临床资料进行分析。结果TS占同期儿童神经精神疾病的0.16％。早期均为多发性抽动，73．9％出现发声抽动，42％出现模仿言语；37．7％脑电图异常；47．8％有脑外伤史、难产史、早产史、感染史和母孕期间出现重大生活事件等；7.2％患儿家族中有TS、癫癇和精神分裂症患者。结论本征可能是生物、心理与环境因素相互作用所致。预后良好。     

立体定向外科手术治疗抽动秽语综合征一例报道

患者，男，24岁，因“眼睑及面部不自主抽动19年，伴发生说脏话6年”而入院。诊断为抽动秽语综合征。患者于5岁时渐出现眼睑及面部不自主抽动，诊断为儿童多动症，服用氟哌啶醇4到6毫克／天，病情略有好转。16岁时又出现前臂不自主抽动，服用氟哌啶醇最大剂量16毫克／天，10余天后因副反应严重而自行减量至8毫克／天。18岁时又出现前臂不自主抽动伴发声、说脏话，诊断为抽动秽语综合征，服用氟哌啶醇最大剂量12毫克／天，泰必利0.4克／天，病情略有好转，10个月后，因出现白细胞升高而自行停药。此后患者又多次到医院就诊，均诊断为抽动秽语综合征，先后服用氟哌啶醇12至20毫克／天以及不知名的中药治疗（20天左右），疗效不佳。     

伴与不伴注意缺陷多动障碍的抽动秽语综合征患儿动态脑电图的对照研究

目的　探讨伴与不伴注意缺陷多动障碍（ＡＤＨＤ） 的抽动秽语综合征（ＴＳ） 患儿间脑电图的差异。方法　对８６ 例伴与不伴ＡＤＨＤ 的ＴＳ患儿进行２４ ｈ 动态脑电图（ＡＥＥＧ） 监测。结果　伴ＡＤＨＤ的ＴＳ组（４０ 例）ＡＥＥＧ异常率为７５％ ,单纯ＴＳ组（４６ 例） 异常率为３０％ ,２ 组差异有显著性（ Ｐ＜０－０５） ;ＡＥＥＧ异常的主要表现为慢波异常以及癫痫样波。伴ＡＤＨＤ的ＴＳ组的ＡＥＥＧ 局部异常多于广泛异常,且以额叶受累多见;而单纯ＴＳ组则广泛异常多于局部异常。结论　伴与不伴ＡＤＨＤ的ＴＳ患儿在脑电生理学上存在异质性     

18例Tourette综合征的临床分析

目的：探讨Tourette综合征（TS）的临床表现、病因、影像学特点及治疗效果。方法：对18例TS患者的临床及影像学资料进行回顾性分析。结果：18例除均有头颈部的不自主抽动、挤眉弄眼、噘嘴及耸肩外，伴不自主异常发声10例（55.6％），秽语3例（16.7％），模仿语言1例（5.6％），四肢不自主抽动11例（61.1％）；仅1例查体有伸舌右偏，右上肢肌张力增高，2例有精神诱因；16例脑电图和12例头颅CT结果均正常，MRI检查8例仅1例异常，PET检查异常1例，分别表现为左侧侧脑室下脚微小软化灶和左侧丘脑下部及右额叶中下回低代谢病灶。17例给予氟哌啶醇、1例给予丙戊酸钠为主的药物治疗，并同时辅以心理治疗，结果所有患者均有不同程度的改善。结论：TS病因目前尚不十分明确，MRI和PET对TS的病因诊断有一定的意义。     

多发性抽动症模型研究进展

正多发性抽动症,又称抽动-秽语综合征、Tourette综合征(Tourette symdrom,TS),好发于儿童及青少年时期,是一种以多发性运动性抽动及不自主发声为主要特点的神经精神障碍性疾病[1]。多表现为颜面、颈部、上下肢的无节律动作抽动,口出秽语、不避亲疏的发声抽动及情绪异常波动等情感障碍[2]。TS的发病机制尚不明确,临床中对TS的研究较多,但对于诱导合适的TS模型,在实验研究中尚未形成一致观点。     

氟哌啶醇治疗伴脑电图异常小儿多发性抽动症疗效观察

目的观察氟哌啶醇治疗伴脑电图异常小儿多发性抽动症(TS)的疗效。方法将符合《美国精神疾病诊断与统计手册》第5版诊断标准的54例小儿TS根据脑电图(EEG)结果分为EEG异常TS组和EEG正常TS组,两组患儿均给予氟哌啶醇治疗,应用《耶鲁综合抽动严重程度量表》于治疗前及治疗4周后、16周后评价其疗效。结果两组患儿在治疗前、治疗4周后、治疗16周后总运动抽动评分和总发声抽动评分总体比较,差异均有统计学意义(F=144. 60,P 0. 05; F=202. 08,P 0. 05)。两组患儿在治疗前总运动抽动评分和总发声抽动评分比较,差异均无统计学意义(P0. 05);两组患儿在治疗4周后脑电图异常TS组总运动抽动评分和总发声抽动评分比较,显著高于脑电图正常TS组(P 0. 05);两组患儿在治疗16周后总运动抽动评分和总发声抽动评分比较,差异均无统计学意义(P 0. 05)。结论氟哌啶醇治疗伴脑电图异常小儿TS有效,其近期疗效较脑电图正常小儿TS差,远期疗效与脑电图正常小儿TS无显著性差异。     

Voluntary tic suppression and the normalization of motor cortical beta power in Gilles de la Tourette syndrome: an EEG study

Gilles de la Tourette syndrome (GTS) is a neurological condition characterized by motor and vocal tics. Previous studies suggested that this syndrome is associated with abnormal sensorimotor cortex activity at rest, as well as during the execution of voluntary movements. It has been hypothesized that this abnormality might be interpreted as a form of increased tonic inhibition, probably to suppress tics; however, this hypothesis has not been tested so far. The present study was designed to formally test how voluntary tic suppression in GTS influences the activity of the sensorimotor cortex during the execution of a motor task. We used EEG to record neural activity over the contralateral sensorimotor cortex during a finger movement task in adult GTS patients, in both free ticcing and tic suppression conditions; these data were then compared with those collected during the same task in age‐matched healthy subjects. We focused on the levels of activity in the beta frequency band, which is typically associated with the activation of the motor system, during three different phases: a pre‐movement, a movement, and a post‐movement phase. GTS patients showed decreased levels of beta modulation with respect to the healthy controls, during the execution of the task; however, this abnormal pattern returned to be normal when they were explicitly asked to suppress their tics while moving. This is the first demonstration that voluntary tic suppression in GTS operates through the normalization of the EEG rhythm in the beta frequency range during the execution of a voluntary finger movement.     

Differences in clinical characteristics between Tourette syndrome patients with and without 'generalized tics' or coprolalia

Abstract  The purpose of this study is to examine whether there are differences in clinical characteristics between Tourette syndrome (TS) patients with and without 'generalized tics' (GT) which involve the entire body, and/or coprolalia. Subjects were 64 patients (55 males and 9 females, mean age, 17.4 ± 7.2 years) who visited Tokyo University's outpatient clinic of neuropsychiatry from 1974 to 1993 and who met criteria for Tourette's disorder of DSM-III-R. Data on clinical characteristics, including tic symptoms and courses of their development, complications and developmental histories, treatment and severity, were collected by systematic chart review of all subjects. Tourette syndrome patients with 'generalized tics' tended to show multiple complex vocal tics more frequently than TS patients without GT. Tourette syndrome patients with coprolalia tended to show significantly higher rates of copropraxia, echolalia, and 'cleaning/washing' compulsion than did the TS patients without coprolalia. Tourette syndrome patients with both GT and coprolalia were classified as the severest group in terms of tic symptoms and social impairment. Tourette syndrome patients who had neither of these morbidities were classified into the mildest group in all aspects. Generalized tics and coprolalia seemed to indicate the severest end of the TS spectrum and seemed to be related with a need of intensive treatment.     

Coprolalia in obsessive-compulsive disorder: a missing link

Although recent clinical research has emphasized the relationship between Tourette's syndrome (TS) and obsessive-compulsive disorder (OCD), coprolalia, the most dramatic of the TS symptoms, has not been described in DSM-III OCD. The case presented here shows that coprolalia can indeed occur in OCD. This finding further erodes the phenomenological distinction between these two disorders. It also supports the role of psychological factors in the genesis of tic symptomatology.     

Sign language tics in a prelingually deaf man.

Gilles de la Tourette syndrome is characterized by vocal and motor tics starting in childhood. Vocal tics may be either noises or words, and the vocal language tics may consist of obscenities (coprolalia) and repetitions of speech that has been heard (echolalia). We describe a prelingually deaf man who has the full array of tics seen in Gilles de la Tourette syndrome, but in whom vocal language tics are replaced by equivalent sign language tics. This is, to our knowledge, the first report of sign language tics in a person with prelingual deafness. The implications of this phenomenon for the separation between language and ideas in tics and the equivalence of sign language to spoken language is discussed.     

Freud and the diagnosis of Gilles de la Tourette's illness

A number of observers recently have taken Freud to task for failing to have diagnosed both Frau Emmy von N.'s (1888-9) involuntary ticcing and vocalizations and Daniel Paul Schreber's (1911) coprolalia and convulsive tics as Tourette's syndrome. None of Freud's critics, however, has placed Freud's understanding of motor and vocal tics in historical context. None seems aware of the contests over the classification of tic symptoms in the 1880's and 1890's, nor do they appreciate the extent to which Charcot and Gilles de la Tourette had conceded that motor and vocal tics, as well as coprolalia, could also appear as symptoms of hysteria. By 1893 (when Freud first wrote out his case of Frau Emmy von N.), both Gilles de la Tourette and Charcot had defended themselves against Guinon's claim that conculsive tics were always a symptom of hysteria by distinguishing those tic symptoms that should be classified as "maladie des tics" from those that accompanied hysteria. Yet Charcot and Gilles de la Tourette had agreed by 1890 that tic symptoms were possible outcomes of either maladie des tics or of hysteria. What separated those afflicted with "hysteria", were particular inherited factors and whether or not the patient could be cured of tics and vocalizations. Thus, within Charcotian terms, Freud was hardly obligated to conclude that his ticcing and cursing patients should have been diagnosed with maladie des tics de Gilles de la Tourette.     

Recurrence of childhood multiple tic in late adult life

In contrast to the lifelong persistence of symptoms characteristic of Gilles de la Tourette's syndrome, multiple tic of childhood is considered to be a self-limited disorder that remits by early adulthood. We describe four patients who had a history of multiple tic of childhood, complete absence of tics throughout most of their adult lives, and recurrence of tics in late adult life. All four had multiple tics that began before the age of 9 years and included both motor and vocal tics that changed in location and severity over time. None of the patients exhibited coprolalia. All tics subsided before the age of 20 years, only to recur after the age of 60 years, once again including both motor and vocal tics that changed in location and severity slowly over time. The one patient who was severely bothered by the recurrence of motor and vocal tics responded well to haloperidol. Although they do not fit into any accepted diagnostic category for multiple tic, these patients suggest that multiple tic of childhood can recur in adult life. This suggests that Gilles de la Tourette's syndrome may be a continuum for chronic multiple tic of childhood to full-blown classic Gilles de la Tourette's syndrome.     

Complex phonic tic and disinhibition in Tourette syndrome: case report

Tourette syndrome (TS) is a neuropsychiatric disorder characterized by a combination of multiple motor tics and at least one phonic tic. TS patients often have associated behavioral abnormalities such as obsessive compulsive disorder, attention deficit and hyperactive disorder. Coprolalia, defined as emission of obscenities or swearing, is one type of complex vocal tic, present in 8% to 26% of patients. The pathophysiology of coprolalia and other complex phonic tics remains ill-defined. We report a patient whose complex phonic tic was characterized by repetitively saying "breast cancer" on seeing the son of aunt who suffered from this condition. The patient was unable to suppress the tic and did not meet criteria for obsessive compulsive disorder. The phenomenology herein described supports the theory that complex phonic tics result from disinhibition of the loop connecting the basal ganglia with the limbic cortex.     

A Case of Tardive Tourette-Like Syndrome

Abstract: We have had experience in treating tardive Tourette-like syndrome on a chronic schizophrenic patient. The patient was a 38-year-old woman. A diagnosis of schizophrenia was made in 1971 and she received repeated medications for 17 years. In 1989, she began to show vocal tic with coprolalia and motor tic. The medications were haloperidol 18 mg, zotepine 200 mg, levomepromseine 100 mg, biperiden 3 mg and nitrazepam 10 mg at the beginning of Tourette-like syndrome. We have tried to change the medications but this tardive Tourette-like syndrome continued to hang on. However, the symptoms gradually improved after a change in drugs; cessation of biperiden 3 mg and the administration of clonazepam 3 mg. The present case suggested that tardive Tourette-like syndrome might be a subtype of neuroleptic-associated tardive syndromes which might be treated with clonazepam.     

41例发作性运动诱发性运动障碍临床与神经电生理分析

目的　观察发作性运动诱发性运动障碍 (PKMD)的临床特征及与癫的关系。方法　详细观察 4 1例PKMD的临床特征 ,影像学和脑电图改变。结果　 4 1例均由运动诱发 ,呈发作性运动诱发性舞蹈手足徐动症 30例 ,发作性运动诱发性肌张力障碍 11例 ,发作时意识清楚 ,影像学有异常者 6例 ,脑电图有样放电者 12例 ,其中 2例发作时有样放电 ,脑体感诱发电位半数以上病例有定位侧半球改变。对抗药有良效。结论　本症障碍的部位可能在感觉刺激的传入通路与发作症状的传出通路之间的反射中枢。具有某些癫性质 ,推测与癫发作可能有某些共同的生物学基础