Gilles De La Tourette Syndrome-Quality Research Articles

The psychometric properties of Chinese version of the Gilles de la Tourette syndrome-quality of life scale (GTS-QOL) for children and adolescents

BackgroundGilles de la Tourette syndrome (GTS) is a persistent neurological disorder that profoundly affects the quality of life for afflicted individuals, however, tailored health-related quality of life (QOL) measures for Chinese pediatric GTS patients are lacking. This pioneering study aims to develop a QOL scale for Chinese children with GTS.MethodsA cohort of 1,121 children (aged 6–16 years) diagnosed with GTS participated in scale development. Internal consistency was evaluated using Cronbach’s alpha. Exploratory factor analysis (EFA) and confirmatory factor analysis (CFA) were performed to assess the factor structure of the GTS-QOL-Chinese, examining goodness-of-fit indices, factor loadings of individual items, and covariances between factors. Validity was determined through interscale correlations and comparisons with external measures to establish convergent and discriminant validity.ResultsFollowing reliability and validity analyses, a Chinese version of the 24-item GTS-specific quality of life scale was conclusively established, encompassing five subscales (psychological, compulsive, cognitive, physical, social). The scale demonstrated high internal consistency with a Cronbach’s alpha coefficient of 0.93, and validity was substantiated through interscale correlations ranging from 0.46 to 0.68. EFA identified five factors, explaining 61.26% of the total variation. CFA further confirmed the scale’s robustness, with all goodness-of-fit indicators meeting acceptable criteria (AGFI = 0.86, PCFI = 0.78, GFI = 0.89, CFI = 0.89, RMSEA = 0.07, SRMR = 0.06).ConclusionsThe Chinese version of the GTS-QOL scale has exhibits robust reliability and validity and is valuable for assessing the quality of life of Chinese children and adolescents with GTS. This scale may assist in tailoring interventions and improving patient care.

Clinical tics and quality of life in children and adolescents with tic disorders: The mediating role of sleep

BackgroundSleep conditions are important in children and adolescents with tic disorders. Psychiatric symptoms and tic symptoms themselves can detrimentally affect the quality of life (QOL) in individuals with tic disorders. However, there is currently a lack of explicit research evidence elucidating the relationship between sleep and the QOL in individuals with tic disorders. MethodsWe assessed 150 children/adolescents aged 4–14 years old diagnosed with tic disorders. Participants' tic symptoms were evaluated using the Yale Global Tic Severity Scale (YGTSS), while their sleep conditions were assessed using the Children's Sleep Habits Questionnaire (CSHQ), and quality of life was measured using the Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL), all utilizing standardized measurement instruments. Detailed statistical descriptions, correlation analyses, and mediation analyses of the data were performed. ResultsWe observed significant correlations among tic symptoms, sleep, and quality of life. Then, we confirmed the mediating role of sleep in the relationship between tic symptoms and quality of life (β = .591, 95%CI: .252–1.007). We also found that among different sleep variables, particularly bedtime resistance (β = .088, 95 % CI: .003–.260), played a significant mediating role. ConclusionsSleep was found to be a significantly mediator between tic symptoms and quality of life across various domains, especially bedtime resistance played an important mediating role. This indicates that assessment and management of sleep conditions in children/adolescents with tic disorders are important. These findings provide potentially valuable insights into the clinical evaluation of children and adolescents with tic disorders and the potential improvement of their quality of life in the future.

The Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL): A Validation in Japanese Patients.

Background: Though Gilles de la Tourette's syndrome (GTS) has significant impact on the quality of life of its patients, measures of health-related quality of life (HR-QOL) specific to adolescents and adults with GTS were not developed until recently. The present study provides evidence on the validity of the Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL), the first disease-specific HR-QOL instrument for GTS patients, for the first time in an East Asian sample.Methods: One hundred and two Japanese individuals aged 13 and above with GTS were included in our study. Internal consistency was evaluated using Cronbach's alpha. The 4-factor structure of the GTS-QOL was assessed using confirmatory factor analysis, using goodness of fit indices, factor loadings of each questionnaire item, and covariances between factors. Validity was assessed using interscale correlations. Convergent and discriminate construct validity was evaluated using correlations with other scales such as the 28-item General Health Questionnaire, the Yale Global Tic Severity Scale, and the short version of the Padua Inventory.Results: Scaling assumptions were met. Internal consistency reliability was high, with a Cronbach's alpha of 0.96. Confirmatory factor analysis revealed sufficient factor loadings and goodness of fit. All measures of goodness of fit corroborated the fit of the 4-factor model. Standardized covariances between factors in the confirmatory factor analysis were >0.8. There were significant correlations with other well-validated scales, and thus convergent and discriminate construct validity was sufficient.Conclusion: The GTS-QOL is a valid and reliable instrument to measure disease-specific HR-QOL of GTS patients in Japan.

Efficacy and Safety of Fixed-Dose Deutetrabenazine in Children and Adolescents for Tics Associated With Tourette Syndrome

Tourette syndrome is a neurodevelopmental disorder characterized by childhood onset of motor and phonic tics, often accompanied by behavioral and psychiatric comorbidities. Deutetrabenazine is a vesicular monoamine transporter 2 inhibitor approved in the US for the treatment of chorea associated with Huntington disease and tardive dyskinesia. To report results of the ARTISTS 2 (Alternatives for Reducing Tics in Tourette Syndrome 2) study examining deutetrabenazine for treatment of Tourette syndrome. This phase 3, randomized, double-blind, placebo-controlled, parallel-group, fixed-dose study was conducted over 8 weeks with a 1-week follow-up (June 21, 2018, to December 9, 2019). Children and adolescents aged 6 to 16 years with a diagnosis of Tourette syndrome and active tics causing distress or impairment were enrolled in the study. Children were recruited from 52 sites in 10 countries. Data were analyzed from February 4 to April 22, 2020. Participants were randomized (1:1:1) to low-dose deutetrabenazine (up to 36 mg/d), high-dose deutetrabenazine (up to 48 mg/d), or a matching placebo, which were titrated over 4 weeks to the target dose followed by a 4-week maintenance period. The primary efficacy end point was change from baseline to week 8 in the Yale Global Tic Severity Scale-Total Tic Score (YGTSS-TTS) for high-dose deutetrabenazine. Key secondary end points included changes in YGTSS-TTS for low-dose deutetrabenazine, Tourette Syndrome Clinical Global Impression score, Tourette Syndrome Patient Global Impression of Impact score, and Child and Adolescent Gilles de la Tourette Syndrome-Quality of Life Activities of Daily Living subscale score. Safety assessments included incidence of treatment-emergent adverse events, laboratory parameters, vital signs, and questionnaires. The study included 158 children and adolescents (mean [SD] age, 11.7 [2.6] years). A total of 119 participants (75%) were boys; 7 (4%), Asian; 1 (1%), Black; 32 (20%), Hispanic; 4 (3%), Native American; 135 (85%), White; 2 (1%), multiracial; 9 (6%), other race; and 1 (0.6%), of unknown ethnic origin. Fifty-two participants were randomized to the high-dose deutetrabenazine group, 54 to the low-dose deutetrabenazine group, and 52 to the placebo group. Baseline characteristics for participants were similar between groups. Of the total 158 participants, 64 (41%) were aged 6 to 11 years, and 94 (59%) were aged 12 to 16 years at baseline. Mean time since Tourette syndrome diagnosis was 3.3 (2.8) years, and mean baseline YGTSS-TTS was 33.8 (6.6) points. At week 8, the difference in YGTSS-TTS was not significant between the high-dose deutetrabenazine and placebo groups (least-squares mean difference, -0.8 points; 95% CI, -3.9 to 2.3 points; P = .60; Cohen d, -0.11). There were no nominally significant differences between groups for key secondary end points. Treatment-emergent adverse events were reported for 34 participants (65%) treated with high-dose deutetrabenazine, 24 (44%) treated with low-dose deutetrabenazine, and 25 (49%) treated with placebo and were generally mild or moderate. In this fixed-dose randomized clinical trial of deutetrabenazine in children and adolescents with Tourette syndrome, the primary efficacy end point was not met. No new safety signals were identified. ClinicalTrials.gov Identifier: NCT03571256.

Safety and Efficacy of Flexible-Dose Deutetrabenazine in Children and Adolescents With Tourette Syndrome

Tourette syndrome is a neurodevelopmental disorder characterized by childhood onset of motor and phonic tics; treatments for tics are associated with safety concerns. Deutetrabenazine is a selective vesicular monoamine transporter 2 inhibitor approved for the treatment of chorea associated with Huntington disease and tardive dyskinesia in adults. To examine whether deutetrabenazine is effective and safe for the treatment of Tourette syndrome in children and adolescents. This phase 2/3, randomized, double-masked, placebo-controlled, parallel-group, dose-titration study included children and adolescents (aged 6-16 years) with Tourette syndrome with active tics causing distress or impairment (ie, Yale Global Tic Severity Scale-Total Tic Score [YGTSS-TTS] ≥20). The trial was conducted over 12 weeks, with 1 week of follow-up from February 2018 to November 2019 at 36 centers in the United States, Canada, Denmark, Russia, Serbia, and Spain. Data analysis was conducted from January 31 to April 22, 2020. Patients were randomized (1:1) to receive deutetrabenazine or placebo, titrated during 7 weeks to an optimal level, followed by a 5-week maintenance period. The maximum total daily deutetrabenazine dose was 48 mg/d. The primary efficacy end point was change from baseline to week 12 in YGTSS-TTS. Key secondary end points included changes in Tourette Syndrome-Clinical Global Impression, Tourette Syndrome-Patient Global Impression of Impact, and Child and Adolescent Gilles de la Tourette Syndrome-Quality of Life Activities of Daily Living subscale score. Safety was assessed based on treatment-emergent adverse events, vital signs, questionnaires, and laboratory parameters. A total of 119 participants were randomized to deutetrabenazine (59 participants; mean [SD] age, 11.5 [2.5] years; 53 [90%] boys; 49 [83%] White; 3 [5%] Black) and placebo (60 participants; mean [SD] age, 11.5 [2.6] years; 51 [85%] boys; 53 [88%] White; 3 [5%] Black). At week 12, the difference in YGTSS-TTS score was not significant between deutetrabenazine and placebo (least squares mean difference, -0.7; 95% CI, -4.1 to 2.8; P = .69; Cohen d, -0.07). There were no nominally significant differences between groups for key secondary end points. Treatment-emergent adverse events were reported for 38 patients (66%) and 33 patients (56%) receiving deutetrabenazine and placebo, respectively, and were generally mild or moderate. In this study of deutetrabenazine in children and adolescents with Tourette syndrome, the primary efficacy end point was not met. No new safety signals were identified. These results may be informative for future studies of treatments for tics in Tourette syndrome. ClinicalTrials.gov Identifier: NCT03452943.

Cross-cultural adaptation and psychometric evaluation of the French version of the Gilles de la Tourette Syndrome Quality of Life Scale (GTS-QOL).

The Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL) is a self-rated disease-specific questionnaire to assess health-related quality of life of subjects with GTS. Our aim was to perform the cross-cultural adaptation of the GTS-QOL into French and to assess its psychometric properties. The GTS-QOL was cross-culturally adapted by conducting forward and backward translations, following international guidelines. The psychometric properties of the GTS-QOL-French were assessed in 109 participants aged 16 years and above with regard to factor structure, internal consistency, reliability and convergent validity with the MOVES (Motor tic, Obsessions and compulsions, Vocal tic Evaluation Survey) and the WHOQOL-BREF (World Health Organization Quality of Life Brief). Exploratory factor analysis of the GTS-QOL-French resulted in a 6-factor solution and did not replicate the original structure in four subscales. The results showed good acceptability (missing values per subscale ranging from 0% to 0.9%), good internal consistency (Cronbach's alpha ranging from 0.68 to 0.94) and good test-retest reliability (intraclass correlation coefficients ranging from 0.70 to 0.81). Convergent validity with the MOVES and WHOQOL-BREF scales showed high correlations. Our study provides evidence of the good psychometric properties of the GTS-QOL-French. The cross-cultural adaptation and validation of this specific instrument will make it possible to assess health-related quality of life in French-speaking subjects with GTS. The GTS-QOL-French could be recommended for use in future research.

Health-related quality of life in patients with Gilles de la Tourette syndrome at the transition between adolescence and adulthood

Gilles de la Tourette syndrome (GTS) is a neurodevelopmental condition characterised by tics and co-morbid behavioural problems, affecting predominantly male patients. Tic severity typically fluctuates over time, with a consistent pattern showing improvement after adolescence in a considerable proportion of patients. Both tics and behavioural co-morbidities have been shown to have the potential to affect patients’ health-related quality of life (HR-QoL) in children and adults with persisting symptoms. In this study, we present the results of the first investigation of HR-QoL in patients with Gilles de la Tourette syndrome at the transition between adolescence and adulthood using a disease-specific HR-QoL measure, the Gilles de la Tourette Syndrome-Quality of Life-Children and Adolescents scale. Our results showed that patients with GTS and more severe co-morbid anxiety symptoms reported lower HR-QoL across all domains, highlighting the impact of anxiety on patient’s well-being at a critical stage of development. Routine screening for anxiety symptoms is recommended in all patients with GTS seen at transition clinics from paediatric to adult care, to implement effective behavioural and pharmacological interventions as appropriate.

Tourette syndrome, co-morbidities and quality of life.

Tourette syndrome is often associated with attention deficit hyperactivity disorder, obsessive compulsive disorder and other co-morbidities, the presence of which can reduce health-related quality of life. The relationship between the number and type of co-morbidities and tic severity upon health-related quality of life has been insufficiently examined in Tourette syndrome populations and not at all in the Australian context. We hypothesised that an increased number of co-morbid diagnoses would be inversely related to health-related quality of life and that the presence of attention deficit hyperactivity disorder and obsessive compulsive disorder in particular would negatively impact health-related quality of life. In all, 83 people with a previously established diagnosis of Tourette syndrome, who responded to a letter of invitation sent to the Tourette Syndrome Association of Australia past-member database, formed the study sample. Participants completed the Gilles de la Tourette Syndrome-Quality of Life Scale and a short form of the National Hospital Interview Schedule to assess tics and related behaviours. Participants with pure-Tourette syndrome had significantly better health-related quality of life than those with Tourette syndrome and three or more co-morbid diagnoses. Few differences were observed between the pure-Tourette syndrome and Tourette syndrome and one or two co-morbid diagnoses groups. Analysis of the impact of individual co-morbid disorders and Tourette syndrome symptoms on health-related quality of life indicated that attention deficit hyperactivity disorder exerted a significant negative effect, as did the presence of complex tics, especially coprolalia and copropraxia. When these variables were examined in multiple regression analysis, number of co-morbidities and the presence of coprophenomena emerged as significant predictors of health-related quality of life. While tics are the defining feature of Tourette syndrome, it appears to be the presence of co-morbidities, attention deficit hyperactivity disorder, in particular, and coprophenomena that have the greater impact on health-related quality of life. This has implications for symptom-targeting in the treatment of Tourette syndrome since all available treatments are symptomatic and not disease modifying.

Long-term Outcome of Globus Pallidus Internus Deep Brain Stimulation in Patients With Tourette Syndrome

ObjectiveTo evaluate the effectiveness of deep brain stimulation (DBS) of the globus pallidus internus (GPi) on tic severity and common comorbidities in patients with severe Tourette syndrome that is refractory to pharmacological treatment and psychotherapy. Patients and MethodsWe retrospectively assessed the long-term clinical outcomes of 13 patients with treatment-refractory Tourette syndrome who underwent DBS targeting the GPi at the Beijing Tiantan Hospital from January 1, 2006, through May 31, 2013. The primary outcome was a change in tic severity as measured by the Yale Global Tic Severity Scale, and the secondary outcome was a change in associated behavioral disorders and mood as measured by the Gilles de la Tourette Syndrome–Quality of Life Scale assessment. ResultsCompared with baseline, the mean reduction in the total Yale Global Tic Severity Scale scores at last follow-up (mean, 41.9 months; range, 13-80 months) was 52.1% (range, 4.3%-83.6%), and the mean improvement rates at 1 month, 6 months, 12 months, 18 months, 24 months, 30 months, and 36 or more months were 11.8%, 20.0%, 26.8%, 36.7%, 44.7%, 49.0%, and 56.7%, respectively. A paired-sample t test revealed significant improvement of tic symptoms after 6 months of DBS programming (P<.05). The Gilles de la Tourette Syndrome–Quality of Life Scale score improved by a mean of 45.7% (range, 11.0%-77.2%). ConclusionThis study is currently the largest reported GPi DBS case series of patients with treatment-refractory TS with the longest follow-up. Our results support the potential beneficial effect of GPi DBS on disabling tic reduction and improvement of quality of life.

Disease-Specific Quality of Life in Young Patients With Tourette Syndrome

Tourette syndrome is a neurodevelopmental disorder characterized by multiple tics and is often associated with comorbid behavioral problems. Research with generic instruments in child populations showed that comorbid disorders can have a greater impact on health-related quality of life than tic severity. This study investigated the usefulness of a newly developed disease-specific instrument, the Gilles de la Tourette Syndrome-Quality of Life Scale for Children and Adolescents (GTS-QOL-C&A), in assessing health-related quality of life in young patients with Tourette syndrome with and without behavioral comorbidity. We recruited 75 patients with Tourette syndrome (60 males; age 12.4 ± 3.2 years). All participants were evaluated by a neuropsychiatrist and completed a standardized psychometric battery, including the GTS-QOL-C&A, Child Depression Inventory, and Multidimensional Anxiety Scale for Children. Forty-two patients (56%) fulfilled diagnostic criteria for at least one comorbidity: obsessive-compulsive disorder (n= 25 patients [33.3%]); attention deficit/hyperactivity disorder (n= 6 patients [8%]); both (n= 11 patients [14.7%]). The GTS-QOL-C&A demonstrated usefulness in differentiating "pure" Tourette syndrome from Tourette syndrome "plus" behavioral problems with regard to health-related quality of life scores for the obsessive-compulsive subscale. In addition to focusing on core tic symptoms, the GTS-QOL-C&A showed sensitivity to the impact of behavioral comorbidities on health-related quality of life and can usefully complement existing nonspecific instruments.

The Gilles de la Tourette Syndrome-Quality of Life Scale for children and adolescents (C&A-GTS-QOL): development and validation of the Italian version.

Background: Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients’ health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410–1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS. Methods: A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6–12 year-olds and a self-report questionnaire for 13–18 year-olds. Results: The C&A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach’s alpha > 0.7) and validity was supported by interscale correlations (range 0.4–0.7), principal-component factor analysis and correlations with other rating scales and clinical variables. Conclusions: The present version of the C&A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity.

The Gilles De La Tourette Syndrome-Quality of Life Scale for Children and Adolescents (C&amp;A-GTS-QOL): Development and Validation of the Italian Version

Background:Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients’ health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410–1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS.Methods:A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&amp;A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6–12 year-olds and a self-report questionnaire for 13–18 year-olds.Results:The C&amp;A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach’s alpha &gt; 0.7) and validity was supported by interscale correlations (range 0.4–0.7), principal-component factor analysis and correlations with other rating scales and clinical variables.Conclusions:The present version of the C&amp;A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity.

Selective Sound Sensitivity Syndrome (Misophonia) in a Patient With Tourette Syndrome

Selective Sound Sensitivity Syndrome (Misophonia) in a Patient With Tourette Syndrome

P4 What predicts long-term quality of life in young patients with Gilles de la Tourette Syndrome?

<h3>Objective</h3> 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). Although the diagnosis of GTS is usually established in childhood, little is known about factors that predict the long-term well-being of these patients, especially in the presence of co-morbid behavioural problems. We set out to prospectively investigate the childhood predictors of HR-QOL in adult patients with GTS. <h3>Method</h3> 46 patients with GTS aged 6–16 years underwent a standardised clinical assessment of both tics and behavioural symptoms at a specialist GTS clinic. The same patients were re-assessed as adults (&gt;16 years of age), 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. <h3>Results</h3> Multiple linear regression analysis identified tic severity, premonitory urges and family history of GTS as childhood predictors of poorer HR-QOL in adults with GTS. Specifically, tic severity significantly predicted poor outcome across physical, psychological and cognitive domains of the GTS-QOL, reflecting its widespread effect on HR-QOL. <h3>Conclusion</h3> 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.

Parent and self-report health-related quality of life measures in young patients with Tourette syndrome.

Tourette syndrome is a neurodevelopmental disorder characterized by tics and comorbid behavioral problems. This study compared child- and parent-reported quality of life and everyday functioning. We assessed 75 children with Tourette syndrome, of which 42 (56%) had comorbid conditions (obsessive-compulsive disorder = 25; attention-deficit hyperactivity disorder = 6; both comorbidities = 4). All patients completed psychometric instruments, including the Gilles de la Tourette Syndrome–Quality of Life Scale for Children and Adolescents (child report) and the Child Tourette’s Syndrome Impairment Scale (parent report). Data were compared for patients with pure Tourette syndrome, Tourette syndrome + obsessive-compulsive disorder, Tourette syndrome + attention-deficit hyperactivity disorder, and Tourette syndrome + both comorbidities. There were no group differences in quality of life. However, there were differences for total, school, and home activities impairment scores. Children and parents may not share similar views about the impact of Tourette syndrome on functioning. The measurement of health-related quality of life in Tourette syndrome is more complex in children than adults.

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

BackgroundGilles 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. AimTo investigate the childhood predictors of HR-QOL in a cohort of adult patients with GTS. MethodsForty-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. ResultsTic 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. ConclusionYoung 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.

The role of impulse control disorders in Tourette syndrome: An exploratory study

IntroductionTourette syndrome (TS) is a neurodevelopmental condition characterised by the presence of multiple motor tics and one or more phonic tics, often associated with co-morbid behavioural problems. Impulse control disorders (ICDs) are a set of disorders where patients have significant difficulties in controlling their urges to perform rewarding behaviours. ICDs are expected to be common in patients with TS, as many problems reported in TS are related to difficulties in controlling impulsivity. AimsThis exploratory study aimed to determine the clinical characteristics of ICDs in adult patients with TS, and to investigate the relationship between the presence of ICDs and health-related quality of life (HR-QOL). MethodsThirty-one patients with a diagnosis of TS were screened for ICDs using the Minnesota Impulsive Disorders Interview (MIDI). HR-QOL was assessed using a generic instrument, the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and a disease-specific scale, the Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL). ResultsTwenty-three out of 31 participants (74.2%) had at least one ICD. The most common ICDs were intermittent explosive disorder (51.6%) and compulsive buying disorder (41.9%). The number of ICDs significantly correlated with reduced HR-QOL (p=0.011) as measured by the GTS-QOL, but not by the SF-36. ConclusionsICDs are common in patients with TS. HR-QOL measures specific to this patient population show that the presence of co-morbid ICDs results in poorer HR-QOL.

Deep brain stimulation for Gilles de la Tourette syndrome: A case series targeting subregions of the globus pallidus internus

Deep brain stimulation remains an experimental treatment for patients with Gilles de la Tourette syndrome. Currently, a major controversial issue is the choice of brain target that leads to optimal patient outcomes within a presumed network of basal ganglia and cortical pathways involved in tic pathogenesis. This report describes our experience with patients with severe refractory Gilles de la Tourette syndrome treated with globus pallidus internus deep brain stimulation. Five patients were selected for surgery, 2 targeting the posteroventral globus pallidus internus and 2 targeting the anteromedial region. The remaining patient was first targeted on the posterolateral region, but after 18 months the electrodes were relocated in the anteromedial area. Tics were clinically assessed in all patients pre- and postoperatively using the Modified Rush Video protocol and the Yale Global Tic Severity Scale. Obsessive-compulsive behaviors were quantified with the Yale-Brown Obsessive Compulsive Scale. The Gilles de la Tourette Syndrome-Quality of Life Scale was also completed. All patients experienced improvements in tic severity but to variable extents. More convincing improvements were seen in patients with electrodes sited in the anteromedial region of the globus pallidus internus than in those with posterolateral implants. Mean reduction in the Modified Rush Video Rating scale for each group was 54% and 37%, respectively. Our open-label limited experience supports the use of the anteromedial globus pallidus internus as a promising target for future planned randomized double-blind trials of deep brain stimulation for patients with Gilles de la Tourette syndrome.