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.     

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.     

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.     

Tourette's syndrome: psychopathology in adolescents

The aim of this study is to foster an understanding of the clinical characteristics, in terms of general psychopathology, of Tourette syndrome (TS), as it manifests in adolescent patients, and to compare these results with those of previous studies conducted in Western countries. In total, 38 male and 5 female adolescent patients who visited a pediatric neurological specialty clinic for TS between 1 January and 1 March 2003, and who met the World Health Organisation criteria for TS, were studied. These patients were clinically interviewed and assessed with the Symptom-Checklist-90-R, Family APGAR and Tic Symptoms questionnaire, a self-report questionnaire based on DSM-IV TS diagnostic criteria. Among this population, the mean age of onset of motor tics was 9.65 +/- 3.7 years, and motor tics of the head and eyes were usually the first symptoms to manifest; coprolalia occurred in 44.1% (n = 19) of our patients. Many of our findings were similar to those reported in trials conducted in other regions of the world. TS symptoms showed stronger correlation with emotional distress in older patients.     

A large Italian family with Gilles de la Tourette syndrome: clinical study and analysis of the SLITRK1 gene.

Our objective was to report the clinical characteristics and to investigate the role of SLITRK1 gene in a large Italian family with Tourette syndrome (TS). The diagnosis of TS and chronic motor tics (CMT) was made according to "The Tourette Syndrome Classification Study Group" (1993). Psychiatric diagnoses were made by administering the Structured Clinical Interview for DSM and the Yale-Brown Obsessive Compulsive Scale. Genetic study included direct sequencing and copy number analysis of the SLITRK1 gene, and haplotype analysis. We found tics or other behavioral manifestations in 15 subjects. Of these, 5 received a diagnosis of definite TS, 5 were classified as having definite CMT, 2 had definite nonspecific tic disorder, and 3 patients had obsessive-compulsive disorder without motor or phonic tics. Tics mainly involved the craniocervical district. Many patients with tics had coexisting psychiatric disorders, especially obsessive-compulsive disorder, performed poorly at school and had social problems. Direct sequencing and copy number analysis of the SLITRK1 gene, and haplotype analysis suggested that the SLITRK1 locus was not involved in this family. In conclusion, the distinctive clinical features in this family are the motor tics mainly involving the face and the neck and the severe coexisting psychiatric disorders. The negative results of the SLITRK1 analysis point to genetic heterogeneity in TS.     

Premonitory urges and repetitive behaviours in adult patients with Tourette syndrome

Tourette syndrome (TS) is a neurodevelopmental disorder characterised by multiple motor and phonic tics, which are associated with sensory symptoms (premonitory urges). Little is known about the nature of the subjective urges and repetitive behaviours which are intrinsic to TS. This study assessed the relationship between specific urges and repetitive behaviours in 108 consecutive adult patients recruited at a specialist TS Clinic. Analysis of self-report measures of sensory symptoms (Premonitory Urge for Tics Scale) and repetitive behaviours (Motor tic, Obsessions and compulsions, Vocal tic Evaluation Survey) showed that different types of urges are associated with simple/complex tics and obsessive–compulsive symptoms.     

110例Tourete综合征的临床与脑电图相关性分析

本文报告１１０例Ｔｏｕｒｅｔｅ综合征（ＴＳ）的临床和脑电图（ＥＥＧ）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。ＥＥＧ异常虽属非特异性改变，但仍提示患者有脑功能活动异常或可能存在中枢神经系统器质性损害     

The Influence of Environmental Consequences and Internalizing Symptoms on Children’s Tic Severity

Although there is evidence that environmental consequences for displaying tics and internalizing symptoms are related to tic severity in children with TS, less is known about the inter-relationships of these variables or how these factors jointly contribute to tic severity. This study included 45 children with Tourette syndrome. Caregivers reported on children’s environmental consequences for displaying tics, internalizing symptoms, and tic severity. Results indicated that children with higher levels of internalizing symptoms experienced significantly more environmental consequences for displaying tics. Children with higher levels of separation anxiety symptoms demonstrated significantly greater tic severity. Environmental consequences for displaying tics accounted for significantly more variance in predicting tic severity than anxiety symptoms. This preliminary evidence suggests that environmental consequences for displaying tics, such as receiving accommodations or attention from others, have a greater influence on children’s tic severity than emotional factors.     

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.     

A survey of Tourette syndrome patients and their families: the 1987 Ohio Tourette Survey

A large sample of patients (N = 763) with Tourette syndrome (TS) responded to a questionnaire dealing primarily with symptoms, treatment history, and associated disorders. The shrinking duration between symptom onset and diagnostic confirmation confirms that diagnostic awareness of TS is increasing. A significant proportion of the sample identified several common factors that occurred in the year before symptom onset. A significantly lower proportion of subjects whose initial symptoms included facial tics reported a positive response to haloperidol. Obsessive characteristics were associated with age and were more common in females. Patients with attention deficit disorder (ADD) had earlier age at onset of TS symptoms and were diagnosed earlier than patients without ADD. In general the data provide some support for a stress-diathesis model and also support previous suggestions about the genetics of Tourette syndrome and related conditions.     

Tics and its disorders

Tourette syndrome (TS) is familial neuropsychiatric disorder that is characterized by motor and phonic tics that begin in childhood. Once thought of as a rare and debilitating disorder, in the last decade new scientific knowledge suggests that TS and related tic disorders are more common and less debilitating for the majority of individuals. Evidence points toward a spectrum of TS symptomatology that extends beyond the tics disorder to probably include obsessive-compulsive disorder, attention deficit hyperactivity disorder, and mood disorders. Tourette syndrome and its differential diagnosis are discussed in this article with a focus on new developments in classification, etiology, epidemiology, genetics, pathophysiology, and clinical management.     

感觉性抽动的立体定向苍白球射频毁损术

目的研究分析22例Tourette综合征（TS）的感觉性抽动特点及实施单侧苍白球腹后部毁损术的治疗作用。方法对22例经过系统的精神科药物及心理行为治疗失败的TS患者实施立体定向苍白球射频毁损术,应用YGTSS评分量表和感觉性先兆问卷对运动性抽动和感觉性抽动进行评估。结果22例患者中18例（81.8%）有各种类型的感觉性抽动,位于头/面部者最多（72.2%）,术后感觉性抽动发作频率较术前均有所下降,YGTSS各项评分均显著下降（P〈0.01）,其中运动抽动的改善率最高。结论感觉性抽动是TS的常见症状,单侧苍白球腹后部毁损术能全面减轻TS各种症状,但远期疗效有待进一步观察。     

Tourette Syndrome and social functioning in a Canadian population

Two hundred and ten patients with Tourette Syndrome (TS) and/or their parents completed a survey, answering questions about the frequency and disruptiveness of vocal and motor tics, behaviour problems and sleep disturbances. Respondents also rated the impact of TS symptoms on social relationships and level of personal and social functioning. Motor tics were reported to be more problematic than were vocal tics. Disruptive behavioural problems included obsessive-compulsive rituals, hyperactivity, anxiety, temper tantrums, mood swings, aggressiveness and coprolalia. Respondents also reported problems getting to sleep, bad dreams, somnambulism and enuresis. More than 40% of respondents reported problems in dating, and problems in making and keeping friends. Family members, friends and physicians were reported to be the most understanding and tolerant of TS symptoms; employers were rated as being the least understanding. More than 30% of respondents reported some problems in coping, but more than 50% of respondents also rated their mental health as good or excellent.     

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.     

Prevalence of tic disorders and Tourette syndrome in a Swedish school population

The aim of the study was to find the epidemiological distribution of tic disorders and Tourette syndrome (TS) in Swedish school children aged 7 to 15 years. A total population of 4,479 children and their parents were asked to fill in a questionnaire covering both motor and vocal tics. A three-stage procedure was used: screening, interview, and clinical investigation. Two hundred and ninety-seven children (190 males, 107 females) were found to have tics. TS, according to DSM-IV criteria, was found in 0.6% of the total population, another 0.8% had chronic motor tics, and 0.5% had chronic vocal tics. Further, 4.8% of the children had transient tics. All together 6.6% of 7- to 15-year-old children currently had or had experienced some kind tic disorder during the last year. Prevalence of different tic disorders was higher among younger children and in males, and was highly associated with school dysfunction. The prevalence of TS was higher than was previously thought but other tic disorders were more common in this childhood population.     

Is onset of Tourette syndrome influenced by life events?

The aim of this study is to investigate the possible relationship between stressful life events, personality, and onset of Tourette syndrome in children. The study group included 93 subjects aged 7-18 years: 41 with Tourette syndrome (TS), 28 with obsessive-compulsive disorder (OCD), and 24 healthy controls. Diagnoses were based on the Child Schedule for Schizophrenia and Affective Disorders (K-SADS). All children were tested with the Screen for Child Anxiety Related Emotional Disorders, Children's Yale Brown Obsessive Compulsive Scale, Beck Depression Inventory or Children's Depression Inventory, the Life Experience Survey, and the Junior Temperament and Character Inventory. The findings were compared among the groups. Subjects with Tourette syndrome and healthy controls had significantly less stressful life events than subjects with (OCD). There were no significant differences between the TS subjects and the healthy controls. This finding applied to total lifetime events, total lifetime negative events, and events in the year before and after illness onset. Subjects with TS and the healthy controls also showed a significantly lesser impact of life events than subjects with OCD. The Tourette syndrome group showed a significantly lesser impact of stressful life events than controls. Harm avoidance tended to be higher in the patients with Tourette syndrome and comorbid attention deficit hyperactivity disorder and obsessive-compulsive disorder than in patients with Tourette syndrome only. There seemed to be no association between life events, diagnosis, and personality. Although there is some research suggesting that tics can be influenced by the environment, the onset of Tourette syndrome does not seem to be related to stressful life events, nor to an interaction between stressful life events and personality.     

Tourette syndrome in Japan: A nationwide questionnaire survey of psychiatrists and pediatricians

In order to shed light on the clinical picture of patients with Tourette syndrome (TS) treated at medical institutions in Japan, a nationwide survey covering both pediatric patients and psychiatric patients was conducted. We mailed 316 questionnaires on experience in treating TS cases and the patients' present conditions etc. to specialists such as psychiatrists and pediatricians. A total of 164 responded. The survey found 154 TS patients being treated at the time of survey, 45 (29.2%) had obsessive–compulsive symptoms (OCS), and 10 (6.5%) had family histories of TS. It was suggested that TS is often associated with OCS and that familial cases of TS are slightly less common in Japan than they are in the USA. Of the 116 respondents who described their experiences, 85 (73.2%) said that they had treated one or more patients displaying the symptoms of frequent coprolalia, and 42 (36.2%) said that they had treated one or more patients suffering from developmental disorders. Based on these findings, we speculated that the rate of coprolalia in Japan is a little higher than the previously reported 4% and that TS is often associated with developmental disorders.     

A functional neuroanatomy of tics in Tourette syndrome

BACKGROUND: Tics are involuntary, brief, stereotyped motor and vocal behaviors often associated with irresistible urges. They are a defining symptom of the classic neuropsychiatric disorder, Tourette syndrome (TS), and constitute an example of disordered human volition. The neural correlates of tics are not well understood and have not been imaged selectively. METHODS: Event-related [(15)O]H(2)O positron emission tomography techniques combined with time-synchronized audio and videotaping were used to determine the duration of, frequency of, and radiotracer input during tics in each of 72 scans from 6 patients with TS. This permitted a voxel-by-voxel correlational analysis within Statistical Parametric Mapping of patterns of neural activity associated with the tics. RESULTS: Brain regions in which activity was significantly correlated with tic occurrence in the group included medial and lateral premotor cortices, anterior cingulate cortex, dorsolateral-rostral prefrontal cortex, inferior parietal cortex, putamen, and caudate, as well as primary motor cortex, the Broca's area, superior temporal gyrus, insula, and claustrum. In an individual patient with prominent coprolalia, such vocal tics were associated with activity in prerolandic and postrolandic language regions, insula, caudate, thalamus, and cerebellum, while activity in sensorimotor cortex was noted with motor tics. CONCLUSIONS: Aberrant activity in the interrelated sensorimotor, language, executive, and paralimbic circuits identified in this study may account for the initiation and execution of diverse motor and vocal behaviors that characterize tics in TS, as well as for the urges that often accompany them. Arch Gen Psychiatry. 2000;57:741-748     

The relationship between tourette syndrome, attention deficit hyperactivity disorder, and stimulant medication: a critical review

The relationship between tic disorders and attention deficit hyperactivity disorder (ADHD) is of great clinical importance because both disorders can lead to emotional, social, and academic difficulties. To further complicate this interrelationship is the concern that the use of psychostimulant medication to treat ADHD will help the hyperactivity and attention problems but will lead to the onset of tics or will worsen preexisting tics. The first part of this review investigates how often Tourette syndrome (TS) is associated with ADHD and finds that ADHD has been reported in 35% to 90% of children with TS. The second part of the review looks at whether the ADHD seen in TS is the same as in children who do not have tics. Recent studies lead to the conclusion that the ADHD seen in TS is the same, although the attentional difficulties seen in TS are influenced also by the distraction of the tics themselves as well as by internal distractions such as is seen in comorbid anxiety or obsessive-compulsive behavior. The final part of the review investigates the question of whether psychostimulants worsen or cause tics. Twenty-two studies were found that investigated this possible relationship. Earlier studies were confounded by the natural pattern seen in TS in which tics spontaneously wax and wane in occurrence, intensity, and frequency. More recent double-blind, placebo-controlled studies have shown that psychostimulants are equally effective in improving ADHD symptoms whether the disorder is associated with tics or not. When group data are analyzed, there is no significant increase in tics when psychostimulants are used in patients with tics compared with controls. Individual patients, however, may experience an increase in tics. This increase is not appreciated in analysis of group data. In conclusion, it is medically appropriate to provide treatment with psychostimulant medication in persons with tics where the ADHD symptoms are significantly disturbing their quality of life.     

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.