Indicators of Social Competence and Social Participation Among US Children With Tourette Syndrome.

Children with Tourette syndrome often have behavioral and social difficulties, which may be associated with co-occurring mental, emotional, or behavioral disorders. This study investigated social competence, including behavioral problems and social skills, and social activities between children with and without Tourette syndrome using a nationally representative sample. In the 2007 National Survey of Children's Health, parents reported on health care provider diagnosis of Tourette syndrome, co-occurring mental, emotional, or behavioral disorders, and indicators of social competence. Children aged 6-17 years with and without Tourette syndrome were compared. Most (78.7%) children with Tourette syndrome had a co-occurring mental, emotional, or behavioral disorder. Children with Tourette syndrome had significantly lower social competence, exhibited by higher levels of behavior problems (mean score 11.6 for Tourette syndrome and 9.0 for no Tourette syndrome) and lower levels of social skills (mean 15.3) than children without a Tourette syndrome diagnosis (mean 17.1); however, these associations were no longer significant after controlling for co-occurring mental, emotional, or behavioral disorders. Moderate to severe Tourette syndrome was associated with the highest ratings of behavioral problems and the lowest ratings of social skills. Children with and without Tourette syndrome were equally likely to participate in social activities; the difference for children with moderate to severe Tourette syndrome being less likely to participate in activities compared to children with mild Tourette syndrome had a chi-square test P value of .05. In conclusion, Tourette syndrome was associated with lower social competence, particularly for children with moderate to severe Tourette syndrome. Monitoring social functioning and co-occurring conditions among children with Tourette syndrome, and referral for evidence-based interventions when needed, may benefit overall health and functioning.

Therapist-guided and parent-guided internet-delivered behaviour therapy for paediatric Tourette's disorder: a pilot randomised controlled trial with long-term follow-up.

OBJECTIVE: Behaviour therapy (BT) for Tourette's disorder (TD) and persistent (chronic) motor or vocal tic disorder (PTD) is rarely available. We evaluated the feasibility of adapting two existing BT protocols for TD/PTD (habit reversal training (HRT) and exposure and response prevention (ERP)) into a therapist-guided and parent-guided online self-help format. DESIGN: A pilot, single-blind, parallel group randomised controlled trial. SETTING: A specialist outpatient clinic in Sweden. PARTICIPANTS: Twenty-three young people with TD/PTD, aged 8-16. INTERVENTIONS: Two 10-week therapist-guided and parent-guided internet-delivered programmes (called BIP TIC HRT and BIP TIC ERP). OUTCOME: The primary outcome measure was the Yale Global Tic Severity Scale. Blinded evaluators rated symptoms at baseline, post-treatment and 3-month follow-up (primary endpoint). All participants were naturalistically followed up to 12 months after treatment. RESULTS: Patients and parents rated the interventions as highly acceptable, credible and satisfactory. While both interventions resulted in reduced tic-related impairment, parent-rated tic severity and improved quality of life, only BIP TIC ERP resulted in a significant improvement on the primary outcome measure. Within-group effect sizes and responder rates were, respectively: d=1.12 and 75% for BIP TIC ERP, and d=0.50 and 55% for BIP TIC HRT. The therapeutic gains were maintained up to 12 months after the end of the treatment. Adverse events were rare in both groups. The average therapist support time was around 25 min per participant per week. CONCLUSIONS: Internet-delivered BT has the potential to greatly increase access to evidence-based treatment for young people with TD/PTD. Further evaluation of the efficacy and cost-effectiveness of this treatment modality is warranted. TRIAL REGISTRATION NUMBER: NCT02864589; Pre-results.

Automatic Bayes Factors for Testing Equality- and Inequality-Constrained Hypotheses on Variances.

In comparing characteristics of independent populations, researchers frequently expect a certain structure of the population variances. These expectations can be formulated as hypotheses with equality and/or inequality constraints on the variances. In this article, we consider the Bayes factor for testing such (in)equality-constrained hypotheses on variances. Application of Bayes factors requires specification of a prior under every hypothesis to be tested. However, specifying subjective priors for variances based on prior information is a difficult task. We therefore consider so-called automatic or default Bayes factors. These methods avoid the need for the user to specify priors by using information from the sample data. We present three automatic Bayes factors for testing variances. The first is a Bayes factor with equal priors on all variances, where the priors are specified automatically using a small share of the information in the sample data. The second is the fractional Bayes factor, where a fraction of the likelihood is used for automatic prior specification. The third is an adjustment of the fractional Bayes factor such that the parsimony of inequality-constrained hypotheses is properly taken into account. The Bayes factors are evaluated by investigating different properties such as information consistency and large sample consistency. Based on this evaluation, it is concluded that the adjusted fractional Bayes factor is generally recommendable for testing equality- and inequality-constrained hypotheses on variances.

A personal 35 year perspective on Gilles de la Tourette syndrome: assessment, investigations, and management.

After having examined the definition, clinical phenomenology, comorbidity, psychopathology, and phenotypes in the first paper of this Series, here I discuss the assessment, including neuropsychology, and the effects of Gilles de la Tourette syndrome with studies showing that the quality of life of patients with Tourette's syndrome is reduced and that there is a substantial burden on the family. In this paper, I review my local and collaborative studies investigating causal factors (including genetic vulnerability, prenatal and perinatal difficulties, and neuro-immunological factors). I also present my studies on neuro-imaging, electro-encephalograms, and other special investigations, which are helpful in their own right or to exclude other conditions. Finally, I also review our studies on treatment including medications, transcranial magnetic stimulation, biofeedback, target-specific botulinum toxin injections, biofeedback and, in severe refractory adults, psychosurgery and deep brain stimulation. This Review summarises and highlights selected main findings from my clinic (initially The National Hospital for Neurology and Neurosurgery Queen Square and University College London, UK, and, subsequently, at St George's Hospital, London, UK), and several collaborations since 1980. As in Part 1 of this Series, I address the main controversies in the fields and the research of other groups, and I make suggestions for future research.

Quality of life in children with OCD with and without comorbidity.

BACKGROUND: Quality of life (QoL) is a well-established outcome measure. However, in contrast to adult obsessive-compulsive disorder (OCD), little is known about QoL in children with OCD. This study aimed to assess QoL, social competence and school functioning of paediatric patients with OCD by comparing them with the general population and assessing the relations between comorbidity, duration and severity of symptoms, family accommodation and QoL. METHODS: Children and adolescents (n = 135), aged 7-17 (mean 13 [SD 2.7] years; 48.1% female) were assessed at baseline for treatment. QoL was assessed by self-report and caregiver's proxy report on the Questionnaire for Measuring Health-related Quality of Life in Children and Adolescents (KINDL-R) and compared with an age- and sex-matched sample from the general population. Social competence and school functioning were assessed with the Child Behavior Checklist, comorbidity with the Kiddie Schedule for Affective Disorders and Schizophrenia (Present and Lifetime Version), severity of OCD with the Children's Yale-Brown Obsessive Compulsive Scale and the families' involvement with the child's OCD symptoms with the Family Accommodation Scale. RESULTS: QoL and social competence were reduced (p < .001) in patients with OCD compared with controls (KINDL-R mean score 62.40 [SD 13.00] versus 69.72 [12.38] in self-reports and 61.63 [SD 13.27] versus 74.68 [9.97] in parent reports). Patients with comorbidity had lower QoL (p = .001) in proxy ratings than those with OCD only (mean score 56.26 [SD 12.47] versus 64.30 [SD 12.75]). In parent proxy reports, severity of OCD (r = -.28) and family accommodation (r = -.40) correlated moderately negatively with QoL. CONCLUSIONS: To our knowledge, this is the largest QoL study of paediatric OCD. QoL was markedly reduced in children with OCD, especially in those with comorbid psychiatric disorders. Based on our findings, we suggest employing QoL assessment in order to have a more comprehensive understanding of childhood OCD. CLINICAL TRIALS REGISTRATION INFORMATION: This study was registered in Current Controlled Trials; Nordic Long-term Obsessive Compulsive disorder (OCD) Treatment Study (ISRCTN66385119).

'Everybody just thinks I'm weird': a qualitative exploration of the psychosocial experiences of adolescents with Tourette syndrome.

BACKGROUND: Research suggests Tourette syndrome (TS) can have a negative impact on quality of life. To date, little research has examined the perspectives of young people with this condition in depth. METHODS: Six 14- to 16-year-olds with TS took part in semi-structured interviews to explore the perceived impact of this condition on self and on relationships with others. The transcripts were analysed using interpretative phenomenological analysis. RESULTS: The young people felt that TS was a constant presence in their lives, but one they have learnt to cope with well. Most had developed supportive friendships but encountered problems when interacting with the wider peer network. Specific concerns around meeting new people and future employment were voiced. CONCLUSIONS: The adolescents described specific ways in which TS affects quality of life and social interactions, and the effort it can take to cope effectively with this condition.

Predictors during childhood of future health-related quality of life in adults with Gilles de la Tourette syndrome.

BACKGROUND: Gilles de la Tourette syndrome (GTS) is a chronic neurodevelopmental disorder characterised by multiple motor and phonic tics and behavioural problems. Patients with GTS of all ages often report a poor health-related quality of life (HR-QOL). The diagnosis of GTS is usually established in childhood but little is known about factors that predict the long-term well-being of patients, especially in the presence of co-morbid behavioural problems. AIM: To investigate the childhood predictors of HR-QOL in a cohort of adult patients with GTS. METHODS: Forty-six patients with GTS aged 6-16 years underwent a baseline standardised clinical assessment of both tics and behavioural symptoms at a specialist GTS clinic. The same patients were re-assessed aged 16 years and above, with a mean follow-up period of 13 years (range 3-25 years), when they completed the Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL), a disease-specific measure of HR-QOL. RESULTS: Tic severity, premonitory urges and family history of GTS were identified as predictors during childhood of a poorer HR-QOL in adults with GTS by multiple linear regression analysis. Specifically, tic severity significantly predicted poor outcome across physical, psychological and cognitive domains of the GTS-QOL, reflecting widespread effects on HR-QOL. CONCLUSION: Young patients with severe tics associated with characteristic premonitory urges and a family history of tic disorders appear to be at higher risk for poorer HR-QOL as adults. Further prospective research into HR-QOL in GTS is required in order to inform long-term strategic resource allocation.

A phenomenological investigation of women with Tourette or other chronic tic disorders.

There are little data concerning clinical characteristics of women with Tourette disorder and chronic tic disorders in the extant literature and what is available mostly focuses on treatment-seeking individuals. The present research was conducted to provide a phenomenological characterization of tic disorders among 185 adult women with tic disorders. In addition to providing a descriptive overview of specific tic symptoms, tic severity, self-reported history of other psychiatric conditions, and impairment/lifestyle impact due to tics, this study compares 185 women and 275 men between 18 and 79 years old with tic disorders (who completed an identical battery of measures) based on demographic, social/economic status indicators, psychiatric variables (comorbidity, family psychiatric history, symptom presentation), adaptive functioning/quality of life, and impairment variables among a nonclinical adult sample. Finally, this research examines the relationship between tic severity and impairment indicators among women with tics. Sixty-eight percent of women in our sample reported severe motor tics and 40% reported severe phonic tics. Our exploratory data suggest that a sizeable number of adult women with persistent tics are suffering from psychiatric comorbidity and psychosocial consequences such as underachievement and social distress. Tic severity in women may be associated with lifestyle interference as well as with symptoms of depression and anxiety, and such symptoms may be more common among women with tics than in men with tics.

Cost of illness in patients with Gilles de la Tourette's syndrome.

We evaluated the health economic burden of patients with Gilles de la Tourette's syndrome (GTS) in Germany over a 3-month observation period. Direct and indirect costs were evaluated in 200 outpatients with GTS (mean age 35 +/- 11.5 years) in Germany. Patients were recruited from three outpatient departments that specialized in GTS and completed a semi-structured and self-rating interview with questionnaires screening for direct and indirect medical and non-medical costs, health status, depression, amount and severity of symptoms. Costs were obtained from various German medical economic resources. Indirect costs for lost productivity were calculated using the human capital approach. Costs were calculated from the point of view of healthcare and transfer payment providers and the individual patient. Multivariate regression analyses were performed to identify independent cost predictors. Costs are in year 2006-2007 values. Direct costs were 620 +/- 1,697.1euro, including rehabilitation 98.8 +/- 993.6euro, hospitalization 195.8 +/- 1,267.8euro, outpatient treatment 14.0 +/- 40.6euro, ancillary treatment 51.9 +/- 137.4euro. Drug costs were 223.1 +/- 430.4. The indirect medical costs amounted to 2,511.3 +/- 3,809.5euro for productivity loss and to 220.0 +/- 1,092euro for absenteeism. The following variables were found to impact on direct costs: employment status, occupational advancement, depression, quality of life, age. Disease severity had no influence on cost. Because of the earlier age of disease onset, indirect costs are higher than direct costs. Interestingly, disease severity did not influence the resource need in this population. Unfortunately, no cost of illness studies are available for comparison. Further health economic studies, especially cost-effectiveness studies, are necessary for a basis for rational resource allocation.

Tourette's syndrome and the law.

Diminished legal responsibility and mental capacity have been used in defense of individuals with neurological disorders charged with legal misdemeanors, including criminal behavior. The purpose of this report is to 1) critically examine the mechanisms that may predispose patients with Tourette's syndrome (TS) to potentially, legally liable behaviors; 2) report the results of a nation-wide review of state, federal, and appellate cases involving TS; and 3) instigate awareness within the professional legal community regarding unrecognized organically-based behaviors that may predispose TS patients to unwanted legal disciplinary action. TS is a common neurological movement disorder of childhood onset associated with behavioral comorbidities, including impulse control problems, exhibition of obscene language or gestures, rage attacks, inappropriate obsessions, and other behaviors. To our knowledge, there are no studies (to date) addressing the potential impact of TS on the legal system. A comprehensive review of the neurobehavioral mechanisms underlying comorbid issues in TS is outlined. A comprehensive review of all cases tried in state and federal courts between 1985 and 2003, in which TS was somehow implicated, was conducted using the Westlaw database. As of October, 2003, TS was implicated in more than 150 cases found in the federal and state databases, 21 of which were criminal. Other cases are categorized as civil rights, criminal, education, family, labor, and social security cases. The authors conclude that TS rarely leads to criminal behavior, but patients with TS who have behavioral comorbidities are at risk of being involved with the legal system. The medical-legal community must learn to recognize the vulnerability of this patient population to potential mistreatment by the courts of justice.

Establishing the feasibility of direct observation in the assessment of tics in children with chronic tic disorders.

Behavior analysis has been at the forefront in establishing effective treatments for children and adults with chronic tic disorders. As is customary in behavior analysis, the efficacy of these treatments has been established using direct-observation assessment methods. Although behavior-analytic treatments have enjoyed acceptance and integration into mainstream health care practices for tic disorders (e.g., psychiatry and neurology), the use of direct observation as a primary assessment tool has been neglected in favor of less objective methods. Hesitation to use direct observation appears to stem largely from concerns about the generalizability of clinic observations to other settings (e.g., home) and a lack of consensus regarding the most appropriate and feasible techniques for conducting and scoring direct observation. The purpose of the current study was to evaluate and establish a reliable, valid, and feasible direct-observation protocol capable of being transported to research and clinical settings. A total of 43 children with tic disorders, collected from two outpatient specialty clinics, were assessed using direct (videotape samples) and indirect (Yale Global Tic Severity Scale; YGTSS) methods. Videotaped observation samples were collected across 3 consecutive weeks and two different settings (clinic and home), were scored using both exact frequency counts and partial-interval coding, and were compared to data from a common indirect measure of tic severity (the YGTSS). In addition, various lengths of videotaped segments were scored to determine the optimal observation length. Results show that (a) clinic-based observations correspond well to home-based observations, (b) brief direct-observation segments scored with time-sampling methods reliably quantified tics, and (c) indirect methods did not consistently correspond with the direct methods.

Emerging drugs in Tourette syndrome.

Proper education of the patient is the first step in the treatment of Tourette syndrome (TS). Before deciding how to treat the patient, it is important to decide whether to treat the TS-related symptoms. Counselling and behavioural modification may be sufficient for those with mild symptoms. Medications, however, may be considered when symptoms begin to interfere with peer relationships, social interactions, academic or job performance, or with activities of daily living. Therapy must be individualised and the most troublesome symptoms should be targeted first. Antidopaminergic agents are clearly the most effective drugs in the treatment of tics. Although haloperidol and pimozide are the only drugs currently approved by the FDA for the treatment of TS, other dopamine receptor-blocking drugs and tetrabenazine, a dopamine depleting drug, as well as botulinum toxin injections, have been used to treat tics associated with TS. Carefully designed, comparative, longitudinal trials assessing the efficacy and adverse-effect profiles of these drugs, including tardive dyskinesia, are lacking. Selective serotonin reuptake inhibitors are recommended for the treatment of obsessive-compulsive behaviour: a common comorbidity. Psychostimulants, such as methylphenidate, are the treatment of choice for attention deficit hyperactivity disorder. Even though these drugs may transiently increase tics, this does not necessarily constitute a definite contraindication to the use of these drugs in patients with TS. Here, existing and emerging medical treatments in patients with tics and comorbid behavioural disorders associated with TS are reviewed.

Assessment of symptom exacerbations in a longitudinal study of children with Tourette's syndrome or obsessive-compulsive disorder.

OBJECTIVES: The severity of tic and obsessive-compulsive (OC) symptoms varies over time. Consequently, how do we, as clinicians, know when a change in symptom severity occurs that falls outside of the normal range of fluctuation? The goal of this study was to describe the level of symptom severity fluctuation over time and to establish an objective, prospective, and quantitative method for identifying symptom exacerbations in children with Tourette's syndrome, obsessive-compulsive disorder (OCD), or both. A second major aim was to assess whether fluctuations in tic and OC symptom severity covaried with one another. METHOD: Monthly consecutive Yale Global Tic Severity Scale and Children's Yale-Brown Obsessive Compulsive Scale scores were prospectively obtained in 64 children diagnosed with Tourette's syndrome and/or OCD for periods ranging from 3 to 39 months. Exacerbation thresholds were estimated by using state-of-the-art bootstrap methods. These thresholds were then independently evaluated by asking two expert clinicians to identify, retrospectively, clinically significant exacerbations based on a review of all available clinical and research records. RESULTS: The severity of tic and OC symptoms displayed a high degree of intrasubject variability. Exacerbation thresholds, which incorporated the change score from the previous month and the current symptom score, provided the best agreement with those of expert clinicians. When both tic and OC symptoms were present, they showed a significant degree of covariation. CONCLUSIONS: Evidence-based treatments are coming of age. The use of valid, clinician-rated severity scales will likely become a standard part of clinical practice. Bootstrapping methods may provide a quantitative and convenient way to obtain clinically valid thresholds to assess tic and OC symptom exacerbations. This method has the potential to be applied to other symptom domains where exacerbation thresholds are needed.

Neuropsychiatric assessment of Gilles de la Tourette patients: comparative study with other hyperkinetic and hypokinetic movement disorders.

The role of the basal ganglia in conditions with co-occurring movement disorders and neuropsychiatric symptoms is not well known. It has been hypothesized that hyperkinesia -disinhibited behaviors and hypokinesia-inhibited behaviors result from an imbalance between the direct and indirect striatal output pathways, and that differential involvement of these pathways could account for the concurrent abnormalities in movement and behavior observed in these disorders. This study aimed to evaluate whether the pattern and the extent of the neuropsychiatric manifestations of patients with GTS, a hyperkinetic movement disorder of basal ganglia origin, differs from that of patients with other basal ganglia hyperkinetic (e.g., HD) or hypokinetic (e.g., PSP) movement disorders, and to determine whether patients with GTS show a greater frequency of hyperactive behaviors (e.g., agitation, irritability, euphoria, or anxiety) than PSP patients, and are comparable to patients with HD. The Neuropsychiatric Inventory (NPI), a scale with established validity and reliability, was administered to 26 patients with GTS (mean age, 30.2 +/- 2.2 years), and the results were compared with that of 29 patients with HD (mean age, 43.8 +/- 2 years) and 34 with PSP (mean +/- S.D. age, 66.6 +/- 1.2 years). There was no difference between the groups in the total NPI scores. However, there was a double dissociation in behaviors: patients with hyperkinetic disorders (HD and GTS) exhibited significantly more agitation, irritability, anxiety, euphoria, and hyperkinesia, whereas hypokinetic patients (PSP) exhibited more apathy. Patients with GTS showed greater scores than HD patients in all those scores differentiating HD and GTS from PSP patients (e.g., agitation, irritability, anxiety and euphoria), and were differentiated in a logistic regression analysis from both HD and PSP patients in having significantly more anxiety. We found that patients with GTS manifested predominantly hyperactive behaviors similar but more pronounced than those presented by patients with HD, while those with PSP manifested hypoactive behaviors. Based on our findings and the proposed models of basal ganglia dysfunction in these disorders, we suggest that the hyperactive behaviors in GTS are comparable to those observed in HD, being both secondary to an excitatory subcortical output through the medial and orbitofrontal cortical circuits, while in PSP the hypoactive behaviors are secondary to hypostimulation of these circuits. Abnormalities of other brain structures (e.g., amygdala, brainstem nuclei) may account for the significantly higher anxiety scores differentiating GTS from HD patients.

Mental health needs associated with Tourette syndrome.

Tourette Syndrome (TS) is an uncommon developmental disability characterized by repetitive and involuntary verbal and motor tics. A survey of all known affiliates of the Tourette Syndrome Association of Ohio was conducted. A total of 431 questionnaires was returned, and behavioral problems were found to be pervasive in Tourette people. Some 53.1 per cent of the sample had sought some form of counseling for these problems, but this counseling was generally not perceived as being very helpful. Medication was not reported to ameliorate behavioral problems. When compared to a normal population sample, self-ratings of mental health status were low among Tourette persons. Problems experienced by TS persons were aggregated into a Behavioral Problem Scale, which successfully discriminated between levels of need in TS persons.