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.     

Cannabinoids: possible role in patho-physiology and therapy of Gilles de la Tourette syndrome

High densities of cannabinoid receptors were found in the basal ganglia and hippocampus, indicating a putative functional role of cannabinoids in movement and behaviour. Anecdotal reports suggested beneficial effects of marijuana in Tourette's syndrome (TS). We therefore interviewed 64 TS patients with regard to use of marijuana and its influence on TS symptomatology. Of 17 patients (27%) who reported prior use of marijuana, 14 subjects (82%) experienced a reduction or complete remission of motor and vocal tics and an amelioration of premonitory urges and obsessive-compulsive symptoms. Our results provide more evidence that marijuana improves tics and behavioural disorders in TS. It can be speculated that cannabinoids might act through specific receptors, and that the cannabinoid system might play a major role in TS pathology.     

The pharmacology of Tourette syndrome

Tourette syndrome (TS) is a chronic neuropsychiatric disorder characterised by multiple motor and vocal tics, plus associated behavioural symptoms. Tics are defined as sudden, rapid, repetitive non-rhythmic movements (motor tics) or vocalisations (vocal tics). Tics are distressing symptoms and can lead to considerable disruption to social functioning and quality of life. Converging evidence from different lines of research suggests that the pathophysiology of TS involves altered dopaminergic transmission in the cortico-striatal–thalamo-cortical circuits, along with other neurotransmitter systems. Pharmacotherapy is currently the treatment of choice in patients with moderate-to-severe tics, particularly when associated with deterioration in social, occupational or academic performance. This review will focus on the recent evidence base supporting the use of different medication classes for the treatment of tics in TS. The recent publication of the European and Canadian guidelines on the management of TS are based on experts’ consensus and highlight the need for randomised controlled trials, especially with regards to newly developed pharmacological agents.     

Severity of Tourette's syndrome in one large kindred. Implication for determination of disease prevalence rate

An accurate prevalence rate for Tourette's syndrome (TS) has not been established. To assess severity of illness, a potential source of bias in determining prevalence rate, we administered standardized questionnaires and examinations to 159 members of a large Mennonite kindred showing apparent autosomal dominant transmission of motor and vocal tics (TS) or chronic motor tics (CMTs). Fifty-four family members were diagnosed as having definite or probable TS or CMTs. For these 54 subjects, 30% (n = 16) were unaware of tics noted by the examiners and only 18.5% (n = 10) had sought medical care. Our findings suggest that most cases of TS and CMTs are mild and do not come to medical attention. These tic disorders are probably much more prevalent than generally appreciated.     

Dystonic tics in patients with Tourette's syndrome

Dystonic tics, in contrast to clonic tics, are relatively slow and temporarily sustained twisting, pulling, or squeezing movements producing briefly maintained abnormal postures. Because dystonic tics often present diagnostic difficulties, we studied these motor tics in 156 patients with Tourette's syndrome (TS). In addition to clonic motor and vocal/phonic tics, seen in all patients, 89 (57%) of them exhibited one or more dystonic tics. The most common dystonic tics were oculogyric deviations (43 patients), blepharospasm (23), and dystonic neck movements (11). Blinking (110) and facial twitching (86) were the most common clonic tics. Except for possibly a greater familial occurrence, a higher frequency of previous head trauma, and associated attention deficit disorder, the group of patients with dystonic tics did not significantly differ in any of the major clinical variables from those TS patients without dystonic tics. We conclude that dystonic tics are typical motor manifestations of TS and that patients with these motor tics are not different from those with more typical clonic tics.     

Clinical analysis of Gilles de la Tourette syndrome based on 126 cases

BACKGROUND AND PURPOSE: Gilles de la Tourette syndrome (TS) is characterized by the presence of multiple motor and vocal tics, as well as other neuropsychiatric disorders. The aim of the study was to evaluate the frequency of particular clinical symptoms in patients diagnosed with TS. MATERIAL AND METHODS: A hundred twenty-six individuals were studied. A brief questionnaire including data from the medical history and neurological examination was used. RESULTS: TS was much more frequent in males (80%; 101/126) than in females. The mean age at onset was 7.6 (2-17) years. The onset of the disease was usually slow. Abrupt onset of the disease, usually after infection, was noted in 11% (12/114) of patients. The mean delay in diagnosis was 3.9 years. In most patients tics were moderate (64%; 81/126). Mild and severe intensity of tics were reported in 15% (19/126) and 21% (26/126) of patients, respectively. 77% (97/126) of individuals with TS had comorbidities. The mean comorbidity score was 2.79 per patient. Anger control problems, sleep difficulties, self-injurious behaviour and coprolalia were strongly associated with comorbidity. The most common reported comorbidity was attention deficit hyperactivity disorder (59%; 74/126). Family history was positive in 46% (57/125) of patients, most often in TS patients with onset between ages 2 and 4 years (70%; 14/20). Haloperidol was the most commonly used medication in our cohort (60%; 57/95). 22% (27/122) of patients did not receive any symptomatic treatment. CONCLUSIONS: The appropriate diagnosis was delayed for about four years after the onset of the disease. Comorbidity and behavioural problems were frequent features of TS. Genetic factors can play an important role in the aetiology of TS.     

Treatment of Tourette's syndrome with Delta 9-tetrahydrocannabinol (THC): a randomized crossover trial

Anecdotal reports in Tourette's syndrome (TS) have suggested that marijuana (cannabis sativa) and delta-9-tetrahydrocannabinol (Delta(9)-THC), the major psychoactive ingredient of marijuana, reduce tics and associated behavioral disorders. We performed a randomized double-blind placebo-controlled crossover single-dose trial of Delta(9)-THC (5.0, 7.5 or 10.0 mg) in 12 adult TS patients. Tic severity was assessed using a self-rating scale (Tourette's syndrome Symptom List, TSSL) and examiner ratings (Shapiro Tourette's syndrome Severity Scale, Yale Global Tic Severity Scale, Tourette's syndrome Global Scale). Using the TSSL, patients also rated the severity of associated behavioral disorders. Clinical changes were correlated to maximum plasma levels of THC and its metabolites 11-hydroxy-Delta(9)-tetrahydrocannabinol (11-OH-THC) and 11-nor-Delta(9)-tetrahydrocannabinol-9-carboxylic acid (THC-COOH). Using the TSSL, there was a significant improvement of tics (p=0.015) and obsessive-compulsive behavior (OCB) (p = 0.041) after treatment with Delta(9)-THC compared to placebo. Examiner ratings demonstrated a significant difference for the subscore "complex motor tics" (p = 0.015) and a trend towards a significant improvement for the subscores "motor tics" (p = 0.065), "simple motor tics" (p = 0.093), and "vocal tics" (p = 0.093). No serious adverse reactions occurred. Five patients experienced mild, transient side effects. There was a significant correlation between tic improvement and maximum 11-OH-THC plasma concentration. Results obtained from this pilot study suggest that a single-dose treatment with Delta(9)-THC is effective and safe in treating tics and OCB in TS. It can be speculated that clinical effects may be caused by 11-OH-THC. A more long-term study is required to confirm these results.     

Accompaniment of Tourette syndrome and neuropsychiatric symptoms

Tourette syndrome (TS) is a neuropsychiatric disorder characterized by multiple motor tics and one or more vocal tics. Attention deficit, obsessive-compulsive disorder, personality problems, and impulsivity may be associated with TS. Functional neuro-imaging studies indicate that the ventral striatum is the most affected brain area in TS. We report a case with TS who had cerebellar atrophy, cerebellar arachnoid cyst, bilateral congenital triangular alopecia, bilateral strabismus, and oxycephaly. The association among these symptoms and TS is rare, and the possible relationship of these symptoms should be investigated with further studies.     

Premonitory sensory phenomenon in Tourette's syndrome.

We administered a questionnaire designed to probe for premonitory sensations associated with motor tics to 50 patients with Tourette's syndrome (TS). Premonitory sensations were reported by 46 (92%) patients, and the most common sensation was an urge to move and an impulse to tic ("had to do it"). Intensification of premonitory sensations, if prevented from performing a motor tic, was reported also in 37 patients (74%), 36 patients (72%) reported relief of premonitory sensations after performing the tic, and 27 of 40 (68%) described a motor tic as a voluntary motor response to an involuntary sensation, rather than a completely involuntary movement. The "just right" sensation correlated with the presence of co-morbid obsessive-compulsive disorder. We conclude that premonitory sensations are an important aspect of motor tics and some patients perceive motor tics as a voluntary movement in response to an involuntary sensation.     

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.     

Pre-movement gating of somatosensory evoked potentials in Tourette syndrome

ObjectiveTourette syndrome (TS) is a neurobehavioral disorder characterized by motor and vocal tics. Simple tics are purposeless involuntary movements that spontaneously resolve during middle adolescence. Complex tics appear to be semi-voluntary movements that may become intractable when associated with obsessive–compulsive disorder (OCD). Sensory tics or urges preceded by tics suggest sensorimotor processing impairment in TS. We aimed to clarify its pathophysiology by exploring the pre-movement gating (attenuation) of somatosensory evoked potentials (SEPs).MethodsWe examined 42 patients (aged 9–48 years), 4 of whom underwent follow-up assessment, along with 19 healthy controls. We defined patients with only simple tics as TS-S and patients with complex tics as TS-C. Pre-movement gating of SEPs was assessed using a previously described method. Frontal N30 (FrN30) amplitudes were compared between pre-movement and resting states. The gating ratio of pre-movement/resting amplitude of the FrN30 component was assessed: the larger the ratio, the less the gating.ResultsThe gating ratio for TS-C patients was larger than that of TS-S patients and healthy controls, but a statistical difference between TS-S and TS-C appeared after 15 years and over (p < 0.001). There were no significant differences in the gating ratio between TS-S patients and healthy controls. The gating ratio was related to the severity of OCD (p < 0.05).ConclusionSensorimotor processing was preserved for simple tics but impaired in complex tics, specifically after middle adolescence. Our study supports an age-dependent dysfunction of both motor and non-motor cortico-striato-thalamo-cortical circuits in complex tics. SEP gating seems promising as a tool for assessing age-dependent sensorimotor disintegration in TS.     

Chronic tics and Tourette syndrome in patients with obsessive-compulsive disorder

Tourette syndrome (TS) and chronic motor/vocal tics (CMVT) are both common disorders in patients with obsessive compulsive disorder (OCD). However, there have been few studies evaluating the differences between the OCD with TS and OCD with CMVT subgroups. This study was conducted in order to further investigate possible differences between the expression of the OCD phenotype in OCD with TS and that seen in OCD with CMVT. One hundred and fifty-nine outpatients referred to an OCD research program were evaluated using the following instruments: the Structured Clinical Interview for DSM-IV; the Yale-Brown Obsessive Compulsive Scale; the Yale Global Tic Severity Scale; and the USP-HARVARD Repetitive Behaviors Interview. Patients were divided into three groups: OCD patients without tics (OCD − TICS, n = 98), OCD patients with chronic motor or vocal tics (OCD + CMVT, n = 31) and OCD patients with TS (OCD + TS, n = 30). OCD + CMVT patients were similar to OCD + TS patients regarding the frequency of intrusive sounds, repeating behaviors, counting and tic-like compulsions (in both cases more frequent than in OCD − TICS patients). For age at obsessive-compulsive (OC) symptom onset, sensory phenomena score, number of comorbidities, frequency of somatic obsessions, bodily sensations and just-right perceptions, OCD + CMVT patients tended to be in between the other two groups. Our results suggest that there are qualitative and quantitative differences in the phenotypic expression of tic disorders in OCD patients, depending on whether the subject has TS or only CMVT.     

Tourette's syndrome in adults

Tourette's syndrome (TS) is defined as motor and phonic tics starting before age 18 years, and therefore most studies have focused on childhood TS, whereas the disorder in adults has not been well characterized. We reviewed medical records of all new TS patients referred to our Movement Disorders Clinic over the past 5 years, 19 years or older on initial evaluation and compared them with 100 TS patients 18 years or younger. The mean age at initial visit of 43 adult TS patients was 58.8 ± 6.7 years, whereas the mean age at initial visit of children with TS was 12.9 ± 2.0 years. Of the adult TS patients, 35 (81.4%) had a history of tics with onset before the age of 18 years (mean age at onset: 8.5 ± 3.4 years), with 8 (18.6%) reporting first occurrence of tics after the age of 18 years (mean age at onset: 37.8 ± 13.2 years). Only two (4.7%) patients reported tic onset after the age of 50 years. Adult patients with TS had significantly more facial and truncal tics, and a greater prevalence of substance abuse and mood disorders, but fewer phonic tics, and lower rates of attention‐deficit hyperactivity disorder and oppositional behavior than children with TS. Adult TS largely represents reemergence or exacerbation of childhood‐onset TS. During the course of TS, phonic and complex motor tics, self‐injurious behaviors, and attention‐deficit hyperactivity disorder tend to improve, but facial, neck, and trunk tics dominate the adult TS phenotype. In addition, adults with TS are more likely to exhibit substance abuse and mood disorders compared with children with TS. © 2010 Movement Disorder Society     

Tourette's syndrome - a review

Tourette's syndrome (TS) is a neuropsychiatric disorder characterised by the occurrence of chronic motor and vocal tics that usually begin in childhood. A prevalence of 4-5/10.000 individuals is estimated. Tourette's syndrome patients frequently show comorbidity with other psychiatric disorders such as obsessive compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), anxiety, and affective disorders. Some forms of OCD seem to share a common genetic etiology with TS and to be a facultative part of the TS phenotypic spectrum. Based on MRI, positron emission tomography (PET), and single photon emission computed tomography (SPECT), data alterations in the cortico-striato-pallido-thalamo-cortical functional systems have been discussed. Within these systems, dopaminergic neurotransmission is thought to play an important role in the pathophysiology of TS. Autoimmunological mechanisms seem to be important in some subtypes of TS and OCD that are triggered or exacerbated by infections with hemolytic streptococci. In these cases, immune modulatory therapy proved to be efficient. To date, there is no established treatment regimen for TS. The medications used most frequently are antipsychotics.     

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.     

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     

A controlled family history study of Tourette's syndrome, I: Attention-deficit hyperactivity disorder and learning disorders

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.     

Gilles de la Tourette's syndrome

Tourette's syndrome (TS) is a childhood onset neurological disorder characterized by motor and vocal tics. It may be associated with a number of co-morbidities including attention deficit hyperactivity disorder, obsessive compulsive symptomatology, and behaviour disorders. Prevalence of TS is higher than previously thought, and may be present in up to 2% of the population. Tourette's syndrome has a significant genetic component. Inheritance may involve several mechanisms including autosomal dominant, bilinear, or polygenic mechanisms. Pathophysiology is still unknown, although is thought to involve striatocortical circuits. Treatment begins with modification of the work and home environment. For more severe cases, medications such as tetrabenazine and neuroleptics may be helpful. Treatment of co-morbidities needs to be considered, as these may result in moredisability than the tics themselves.