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Tics are brief, sudden, non-rhythmic, repetitive movements. Tics can be motor or vocal. Further, both motor and vocal tics can be either simple or complex. Simple tics typically involve only one group of muscles and are brief and meaningless, whereas complex tics may last longer and appear more purposeful. Tic disorders usually begin in childhood and are classified according to the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) into four groups: (1) provisional tic disorder, (2) chronic motor or vocal tic disorder, (3) Tourette disorder (or Gilles de la Tourette syndrome), (4) tic disorder not otherwise specified. As tics can resemble almost any other movement disorder, phenotypic analysis alone is insufficient and patients must be questioned whether the execution is preceded by a premonitory sensation (urge to do, urge to move) and whether a temporary control of the movement can be achieved. Also, relief following execution of the tic is frequently reported. There are no biomarkers available for tics and diagnosis therefore remains strictly clinical.
This study aimed to utilise graph theory to explore the functional brain networks in individuals with tic disorders and to investigate resting-state functional connectivity changes in critical brain regions associated with tic disorders.
Methods:
Participants comprised individuals with tic disorders and age-matched healthy controls, ranging from 6 to 18 years old, all recruited from Korea University Guro Hospital. We ensured a medication-naïve cohort by excluding participants exposed to psychotropic medications for at least three weeks prior to the study. Data included structural and resting-state functional MRI scans, analysed with the CONN-fMRI Functional Connectivity toolbox v20b. The analysis included 22 patients (18 males, 4 females) and 26 controls (14 males, 12 females).
Results:
Significantly increased global efficiency was observed in the left inferior frontal gyrus pars opercularis among tic disorder patients compared to controls. Furthermore, this region displayed enhanced resting-state functional connectivity with its right counterpart in patients versus controls.
Conclusion:
The inferior frontal gyrus pars opercularis, known for its inhibitory role, may reflect adaptive functional adjustments in response to tic symptoms. Increased hubness of the inferior frontal gyrus pars opercularis possibly represents functional adjustments in response to tic symptoms. The identified brain region with increased efficiency and connectivity presents a promising avenue for further research into tic expression and control mechanisms.
Kushner's monograph (published in 2000) explores clinicians’ developing understanding of a syndrome first described 200 years ago. This article highlights changing concepts of the disorder, its aetiology and treatment over its long history, as described by Kushner, and considers the current differential diagnosis of DSM-5 ‘Tourette's disorder’. It points out Kushner's astute observation that the clinical syndrome originally described by Itard and Gilles de la Tourette as ‘maladie des tics’ is not what is defined in the 21st century as Tourette syndrome.
This study examined children at the onset of tic disorder (tics for less than 9 months: NT group), a population on which little research exists. Here, we investigate relationships between the baseline shape and volume of subcortical nuclei, diagnosis, and tic symptom outcomes.
Methods
187 children were assessed at baseline and a 12-month follow-up: 88 with NT, 60 tic-free healthy controls (HC), and 39 with chronic tic disorder/Tourette syndrome (TS), using T1-weighted MRI and total tic scores (TTS) from the Yale Global Tic Severity Scale to evaluate symptom change. Subcortical surface maps were generated using FreeSurfer-initialized large deformation diffeomorphic metric mapping. Linear regression models correlated baseline structural shapes with follow-up TTS while accounting for covariates, with relationships mapped onto structure surfaces.
Results
We found that the NT group had a larger right hippocampus compared to HC. Surface maps illustrate distinct patterns of inward deformation in the putamen and outward deformation in the thalamus for NT compared to controls. We also found patterns of outward deformation in almost all studied structures when comparing the TS group to controls. The NT group also showed consistent outward deformation compared to TS in the caudate, accumbens, putamen, and thalamus. Subsequent analyses including clinical symptoms revealed that a larger pallidum and thalamus at baseline correlated with less improvement of tic symptoms at follow-up.
Conclusion
These observations constitute some of the first prognostic biomarkers for tic disorders and suggest that these subregional shape and volume differences may be associated with the outcome of tic disorders.
Comprehensive Behavioral Intervention for Tics (CBIT) is recommended as a first-line treatment for Tourette syndrome in children and adults. While there is strong evidence proving its efficacy, the mechanisms of reduction in tic severity during CBIT are still poorly understood. In a recent study, our group identified a functional brain network involved in tic suppression in children with TS. We reasoned that voluntary tic suppression and CBIT may share some mechanisms and thus we wanted to assess whether functional connectivity during tic suppression was associated with CBIT outcome.
Methods
Thirty-two children with TS, aged 8 to 13 years old, participated in a randomized controlled trial of CBIT v. a treatment-as-usual control condition. EEG was recorded during tic suppression in all participants at baseline and endpoint. We used a source-reconstructed EEG connectivity pipeline to assess functional connectivity during tic suppression.
Results
Functional connectivity during tic suppression did not change from baseline to endpoint. However, baseline tic suppression-related functional connectivity specifically predicted the decrease in vocal tic severity from baseline to endpoint in the CBIT group. Supplementary analyses revealed that the functional connectivity between the right superior frontal gyrus and the right angular gyrus was mainly driving this effect.
Conclusions
This study revealed that functional connectivity during tic suppression at baseline predicted reduction in vocal tic severity. These results suggest probable overlap between the mechanisms of voluntary tic suppression and those of behavior therapy for tics.
To better understand current practices of U.S.-based physicians in the management of Tourette syndrome (TS) and identify gaps that may be addressed by future education.
Methods
Two survey instruments were developed to gather data on management of TS and perceptions from physicians and caregivers of children with TS. The clinician survey was developed in consultation with a TS physician expert and utilized clinical vignettes to assess and quantify practice patterns. The caregiver survey was adapted from the clinician survey and other published studies and gathered details on diagnosis, treatment, and perceptions regarding management.
Results
Data included responses from 138 neurologists (including 57 pediatric neurologists), 162 psychiatrists (including 42 pediatric psychiatrists), and 67 caregivers. Most (65%) pediatric neurologists rely solely on clinical findings to make a diagnosis, whereas the majority of other specialists utilize additional testing (eg, neuroimaging, lab testing, and genetics). Most psychiatrists (96%) utilize standardized criteria to make a diagnosis, whereas 22% of neurologists do not. Many physicians (44% of psychiatrists and 20% of neurologists) use pharmacotherapy to treat a patient with “slightly bothersome” tics and no functional impairment, whereas caregivers favored behavioral therapy. Most (76%) caregivers preferred to make the final treatment decision, whereas 80% of physicians preferred equal or physician-directed decision-making.
Conclusions
This study provides insight into practice patterns and perceptions of U.S.-based neurologists and psychiatrists in managing TS. Results highlight the potential value of physician education, including diagnostic approach, tic management and monitoring, involvement of caregivers in decision-making, and updates on TS management.
Tourette syndrome (TS) is a neurodevelopmental disorder characterized by the presence of motor and phonic tics. It is at least three times more common in males compared with females; however, the clinical phenomenology between sexes has not been fully examined. We aimed to contrast the clinical features between males and females with TS and chronic tic disorder.
Methods
We studied 201 consecutive patients fulfilling the diagnostic criteria for TS, persistent (or chronic) motor and vocal tic disorder and provisional tic disorder that were considered within the TS spectrum disorder. We performed blinded evaluations of video-recordings and retrospectively reviewed the clinical charts of all patients.
Results
Age ranges between 4 and 65 years. Males represented 77.6% of patients in the cohort. Overall, no differences were observed in the frequency, distribution and complexity of tics between sexes, except for a higher frequency of attention-deficit/hyperactivity disorder (ADHD) (P = .003) among males. Patients younger than 18-years old, in addition to a higher frequency of ADHD (P = .026), males had a statistically higher frequency of complex motor tics (P = .049) and earlier age at onset (P = .072) than females in the multivariate regression analysis. However, these differences were lost in patients older than 18 years, due to increased complexity of tics in females with aging.
Conclusions
A sexual dimorphism was observed between patients with TS mainly before age of 18 years, suggesting an earlier onset of some types of tics and ADHD in males compared to females.
Tourette’s syndrome is a neuropsychiatric disorder marked by motor and phonic tics frequently associated with psychiatric comorbidities, beginning in childhood. While most cases improve or resolve with age, some are refractory.
Objectives
To review new strategies for the management of Tourette’s Syndrome, following an outpatient clinical vignette.
Methods
We performed a review based on the PubMed® database.
Results
A 50-years-old female patient with a long-term outpatient psychiatric follow-up presented with motor tics appearing in adolescence, including winking and facial grimacing, as well as episodes of coprolalia. Over the years, she developed an anxiety disorder and social isolation. In addition to psychological therapy, pharmacological therapy had already been approached with the use alpha-adrenergic agonists and several antipsychotics, such as risperidone and aripiprazole, with the patient showing only partial response to pimozide. In Tourette’s syndrome, the therapy must be adequate to the patient’s individual needs. Emerging treatments for refractory cases, such as anticonvulsants, cannabinoids or antiglutamatergic drugs have been the target of several studies. Botulinum toxin injections are particularly effective in patients with focal motor tics and complex phonic tics. Non-pharmacological treatment options, such as electroconvulsive therapy and deep brain stimulation may prove effective in some cases.
Conclusions
A significant proportion of patients fail to respond to conventional strategies. Thus, new pharmacological and non-pharmacological therapies are on the horizon and may represent an important step in treatment algorithms for refractory cases in the future.
Tourette syndrome (TS) is a challenging and poorly understood condition that can have a considerable negative effect on an individual’s ability to learn, despite there being little to no impact on their intelligence. In this paper, we detail the experiences of 2 higher education staff who supported a student with severe TS to undertake studies in a university bridging program. We make suggestions and recommendations for teachers who have students with TS. Over the course of 5 semesters, the teaching team researched TS in order to understand what the student was facing and adjusted their teaching strategies and the learning environment to overcome the complications that the condition presented. The design of the learning environment and the embedded accessible pedagogy that we found helpful are framed and discussed using the 3 primary principles of universal design for learning: engagement, representation, and action and expression. The authors utilise the minimal model of Rolfe, Freshwater, and Jasper (2001) to reflect upon and share their practice.
Although boys are disproportionately affected by tics in Tourette syndrome (TS), this gender bias is attenuated in adulthood and a recent study has suggested that women may experience greater functional interference from tics than men. The authors assessed the gender distribution of adults in a tertiary University-based TS clinic population and the relative influence of gender and other variables on adult tic severity (YGTSS score) and psychosocial functioning (GAF score). We also determined retrospectively the influence of gender on change in global tic severity and overall TS impairment (YGTSS) since adolescence. Females were over-represented in relation to previously published epidemiologic surveys of both TS children and adults. Female gender was associated with a greater likelihood of tic worsening as opposed to tic improvement in adulthood; a greater likelihood of expansion as opposed to contraction of motor tic distribution; and with increased current motor tic severity and tic-related impairment. However, gender explained only a small percentage of the variance of the YGTSS global severity score and none of the variance of the GAF scale score. Psychosocial functioning was influenced most strongly by tic severity but also by a variety of comorbid neuropsychiatric disorders.
There is irrefutable evidence that routine physical activity or exercise can offer considerable health benefits to individuals living with various mental disorders. However, it is not clear what effect physical activity has on the symptoms of Tourette syndrome. Despite a paucity of evidence, physical activity or exercise has already been recommended by various health organizations for the management of tics. We provide a systematic review of the effects of physical activity or exercise on tic symptomology in individuals with Tourette syndrome. Major electronic databases were searched for all available publications before August 2017. Keywords and MeSH terms included “physical activity” or “exercise” or “exercise therapy” or “physical exertion” or “sports” and “tics” or “tic disorders” or “Tourette.” Eight studies were included, the majority of which were case reports. Despite a number of methodological limitations of the included studies, the review points to a trend that the effects of acute physical activity are intensity-dependent, where light intensity may alleviate and vigorous intensity may exacerbate tics. Chronic physical activity, however, appears to reduce the severity of tics even at higher intensity. Several physiological mechanisms may explain the differential effects of acute and chronic physical activity in Tourette syndrome. Future randomized controlled studies should better characterize the effects of different intensities and types of physical activity in Tourette syndrome.
Chronic tic disorders may have a major impact on a child's function. A significant effect has been shown for combined habit reversal training (HRT) and exposure response prevention (ERP) treatment delivered in an individual and group setting.
Aims
The present study examines predictors and moderators of treatment outcome after an acute therapeutic intervention.
Method
Fifty-nine children and adolescents were randomised to manualised treatment combining HRT and ERP as individual or group training. Age, gender, baseline tic severity, Premonitory Urge for Tics Scale (PUTS) scores, Beliefs about Tic Scale (BATS) scores, hypersensitivity and comorbid psychiatric symptoms were analysed as predictors of outcome. The same characteristics were examined as moderators for individual versus group treatment. Outcome measures included the change in total tic severity (TTS) score and functional impairment score (as measured by the Yale Global Tic Severity Scale (YGTSS)).
Results
Internalising symptoms predicted a lesser decrease in functional impairment. The occurrence of obsessive–compulsive symptoms predicted a larger decrease in TTS. Baseline hypersensitivity and high scores on depressive symptoms favoured individual treatment. High baseline PUTS scores favoured group therapy.
Conclusions
This is the first study examining factors predicting and moderating perceived functional impairment following a therapeutic intervention. The study adds to the knowledge on predictors and moderators of TTS. Furthermore, this is the first study examining the effect of the BATS score. The study points towards factors that may influence treatment outcome and that require consideration when choosing supplemental treatment. This applies to comorbid anxiety and depressive symptoms, and to the child's belief about their tics and premonitory urge.
The unique phenotypic and genetic aspects of obsessive-compulsive (OCD) and attention-deficit/hyperactivity disorder (ADHD) among individuals with Tourette syndrome (TS) are not well characterized. Here, we examine symptom patterns and heritability of OCD and ADHD in TS families.
Method
OCD and ADHD symptom patterns were examined in TS patients and their family members (N = 3494) using exploratory factor analyses (EFA) for OCD and ADHD symptoms separately, followed by latent class analyses (LCA) of the resulting OCD and ADHD factor sum scores jointly; heritability and clinical relevance of the resulting factors and classes were assessed.
Results
EFA yielded a 2-factor model for ADHD and an 8-factor model for OCD. Both ADHD factors (inattentive and hyperactive/impulsive symptoms) were genetically related to TS, ADHD, and OCD. The doubts, contamination, need for sameness, and superstitions factors were genetically related to OCD, but not ADHD or TS; symmetry/exactness and fear-of-harm were associated with TS and OCD while hoarding was associated with ADHD and OCD. In contrast, aggressive urges were genetically associated with TS, OCD, and ADHD. LCA revealed a three-class solution: few OCD/ADHD symptoms (LC1), OCD & ADHD symptoms (LC2), and symmetry/exactness, hoarding, and ADHD symptoms (LC3). LC2 had the highest psychiatric comorbidity rates (⩾50% for all disorders).
Conclusions
Symmetry/exactness, aggressive urges, fear-of-harm, and hoarding show complex genetic relationships with TS, OCD, and ADHD, and, rather than being specific subtypes of OCD, transcend traditional diagnostic boundaries, perhaps representing an underlying vulnerability (e.g. failure of top-down cognitive control) common to all three disorders.
Previous studies suggest that adults with Tourette syndrome (TS) can respond unconventionally on tasks involving social cognition. We therefore hypothesized that these patients would exhibit different neural responses to healthy controls in response to emotionally salient expressions of human eyes.
Method
Twenty-five adults with TS and 25 matched healthy controls were scanned using fMRI during the standard version of the Reading the Mind in the Eyes Task which requires mental state judgements, and a novel comparison version requiring judgements about age.
Results
During prompted mental state recognition, greater activity was apparent in TS within left orbitofrontal cortex, posterior cingulate, right amygdala and right temporo-parietal junction (TPJ), while reduced activity was apparent in regions including left inferior parietal cortex. Age judgement elicited greater activity in TS within precuneus, medial prefrontal and temporal regions involved in mentalizing. The interaction between group and task revealed differential activity in areas including right inferior frontal gyrus. Task-related activity in the TPJ covaried with global ratings of the urge to tic.
Conclusions
While recognizing mental states, adults with TS exhibit greater activity than controls in brain areas involved in the processing of negative emotion, in addition to reduced activity in regions associated with the attribution of agency. In addition, increased recruitment of areas involved in mental state reasoning is apparent in these patients when mentalizing is not a task requirement. Our findings highlight differential neural reactivity in response to emotive social cues in TS, which may interact with tic expression.
Tic disorders are moderately heritable common psychiatric disorders that can be highly troubling, both in childhood and in adulthood. In this study, we report results obtained in the first epigenome-wide association study (EWAS) of tic disorders. The subjects are participants in surveys at the Netherlands Twin Register (NTR) and the NTR biobank project. Tic disorders were measured with a self-report version of the Yale Global Tic Severity Scale Abbreviated version (YGTSS-ABBR), included in the 8th wave NTR data collection (2008). DNA methylation data consisted of 411,169 autosomal methylation sites assessed by the Illumina Infinium HumanMethylation450 BeadChip Kit (HM450k array). Phenotype and DNA methylation data were available in 1,678 subjects (mean age = 41.5). No probes reached genome-wide significance (p < 1.2 × 10−7). The strongest associated probe was cg15583738, located in an intergenic region on chromosome 8 (p = 1.98 × 10−6). Several of the top ranking probes (p < 1 × 10−4) were in or nearby genes previously associated with neurological disorders (e.g., GABBRI, BLM, and ADAM10), warranting their further investigation in relation to tic disorders. The top significantly enriched gene ontology (GO) terms among higher ranking methylation sites included anatomical structure morphogenesis (GO:0009653, p = 4.6 × 10−15) developmental process (GO:0032502, p = 2.96 × 10−12), and cellular developmental process (GO:0048869, p = 1.96 × 10−12). Overall, these results provide a first insight into the epigenetic mechanisms of tic disorders. This first study assesses the role of DNA methylation in tic disorders, and it lays the foundations for future work aiming to unravel the biological mechanisms underlying the architecture of this disorder.
Pediatric obsessive-compulsive disorder (OCD) and tic disorders (TD) are often associated with attention-deficit hyperactivity disorder (ADHD). In order to clarify the role of attention and inhibitory control in pediatric OCD and TD, a continuous performance test (CPT) was administered to a cohort of children and adolescents with OCD alone, TD alone, and OCD+TD.
Methods
A clinical cohort of 48 children and adolescents with OCD alone (n=20), TD alone (n=15), or OCD+TD (n=13) was interviewed clinically and administered the Conners Continuous Performance Test II (CPT-II). The Conners CPT-II is a 14-minute normed computerized test consisting of 6 blocks. It taps into attention, inhibitory control, and sustained attention cognitive domains. Key parameters include errors of omission (distractability), commission (inhibitory control), and variable responding over time (sustained attention). Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) criteria were applied in a best-estimate process to diagnose OCD, TD, ADHD, and anxiety disorders.
Results
Children with OCD+TD had more errors of omission (p=0.03), and more hit RT block change (p=0.003) and hit SE block change (p=0.02) than subjects with OCD alone and TD alone. These deficits in sustained attention were associated with younger age and hoarding tendencies. A clinical diagnosis of ADHD in the OCD+TD group also determined worse sustained attention.
Conclusions
A deficit in sustained attention, a core marker of ADHD, is also a marker of OCD+TD, compared to OCD alone and TD alone. Biological correlates of sustained attention may serve to uncover the pathophysiology of OCD and TD through genetic and imaging studies.
Tics are involuntary movements or sounds. Tourette syndrome is one of a family of tic disorders that affect around 1% of the population but which remains underrecognised in the community. In paediatric special education learning disability classes, the prevalence of individuals with tic disorders is around 20–45% — higher still in special education emotional/behavioural classes. Given the high rates of individuals with tic disorders in special education settings, as well as the unique challenges of working in an educational setting with a person with a tic disorder, it is incumbent upon professionals working in these settings to be cognisant of the possibility of tic disorders in this population. This review seeks to provide an overview of tic disorders and their association with learning and mental health difficulties. The review focuses on an exploration of factors underpinning the association between tic disorders and learning disabilities, including neurocognitive corollaries of tic disorders and the influence of common comorbidities, such as ADHD, as well as upon strategies to support individuals with tic disorders in the classroom.
To evaluate the clinical features of obsessive-compulsive disorder (OCD) patients with comorbid tic disorders (TD) in a large, multicenter, clinical sample.
Method
A cross-sectional study was conducted that included 813 consecutive OCD outpatients from the Brazilian OCD Research Consortium and used several instruments of assessment, including the Yale-Brown Obsessive-Compulsive Scale, the Dimensional Yale-Brown Obsessive-Compulsive Scale, the Yale Global Tic Severity Scale (YGTSS), the USP Sensory Phenomena Scale, and the Structured Clinical Interview for DSM-IV Axis I Disorders.
Results
The sample mean current age was 34.9 years old (SE 0.54), and the mean age at obsessive-compulsive symptoms (OCS) onset was 12.8 years old (SE 0.27). Sensory phenomena were reported by 585 individuals (72% of the sample). The general lifetime prevalence of TD was 29.0% (n = 236), with 8.9% (n = 72) presenting Tourette syndrome, 17.3% (n = 141) chronic motor tic disorder, and 2.8% (n = 23) chronic vocal tic disorder. The mean tic severity score, according to the YGTSS, was 27.2 (SE 1.4) in the OCD + TD group. Compared to OCD patients without comorbid TD, those with TD (OCD + TD group, n = 236) were more likely to be males (49.2% vs. 38.5%, p < .005) and to present sensory phenomena and comorbidity with anxiety disorders in general: separation anxiety disorder, social phobia, specific phobia, generalized anxiety disorder, post-traumatic stress disorder, attention-deficit hyperactivity disorder, impulse control disorders in general, and skin picking. Also, the “aggressive,” “sexual/religious,” and “hoarding” symptom dimensions were more severe in the OCD + TD group.
Conclusion
Tic-related OCD may constitute a particular subgroup of the disorder with specific phenotypical characteristics, but its neurobiological underpinnings remain to be fully disentangled.
The causal conditions for psychiatric disorders include: stress, depression, illicit psychoactive drug misuse, psychotropic medication (antidepressants and antipsychotics), autistic spectrum disorders, Tourette syndrome and attention deficit hyperactivity disorder (ADHD). This chapter discusses the treatment and diagnostic options for these conditions. A variety of commonly used recreational drugs can act either directly or indirectly to cause seizures. In the UK these include opioids, stimulants (cocaine, amphetamine, methylenedioxymethamphetamine), marijuana, benzodiazepines, barbiturates, club drugs (phencyclidine and ketamine), hallucinogens, and inhalants. The core features of autism and related disorders (ASD) are qualitative abnormalities in reciprocal social interactions, patterns of communication, and a restricted stereotyped repetitive repertoire of interests and activities. Attention deficit hyperactivity disorder is a childhood-onset illness characterized by severe inattention, hyperactivity, and impulsivity. Diagnosis of ADHD is clinical and based upon fulfilling the DSM-IV criteria.
Tourette Syndrome (TS) in children is associated with various neurobehavioral disorders including attention deficit hyperactivity disorder (ADHD). Children with TS and ADHD show some difficulties with neuropsychological tasks, but we do not know if children with TS alone have neuropsychological deficits. To assess specific cognitive differences among children with TS and/or ADHD, we administered a battery of neuropsychological tests, including 10 tasks related to executive function (EF), to 10 children with TS-only, 48 with ADHD-only, and 32 with TS+ADHD. Children in all groups could not efficiently produce output on a timed continuous performance task [Test of Variables of Attention (TOVA) mean reaction time and reaction time variability]. Children with TS-only appeared to have fewer EF impairments and significantly higher perceptual organization scores than children with TS+ADHD or ADHD-only. These findings suggest that deficiencies in choice reaction time and consistency of timed responses are common to all three groups, but children with TS-only have relatively less EF impairment than children with TS+ADHD or ADHD-only. (JINS, 1995, 1, 511–516.)