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BACKGROUND: The exact etiology of Tourette Syndrome (TS) remains unclear, making the search for impaired neuropsychological functions possibly connected to the underlying cause of TS as important as it is challenging. One neuropsychological domain of interest is fine motor skills.METHOD: This study compared fine motor skill performance on the Purdue Pegboard Task (PPT) in 18 children with TS, 24 unaffected first-degree siblings and 20 controls. A set of screening questionnaires was administered to determine comorbid psychiatric illness.RESULTS: Children with TS, their siblings and controls did not differ significantly in fine motor skills as measured with the PPT. Performance on the PPT was not correlated with tic severity; however, we found an inverse correlation with severity of attention-deficit/hyperactivity disorder (ADHD) symptoms, as assessed by parent reported ADHD symptoms. Children with TS were found to have significantly higher parent reported ADHD symptoms compared to controls, yet only two out of the 18 participants had been diagnosed with ADHD.CONCLUSION: This study suggests that fine motor skill impairment in children with TS may be more strongly correlated with comorbid ADHD than to TS and tics.

Due to the global increase in the number of patients with Functional Tic-Like Behaviors (FTLB), it has become increasingly important to find reliable differences between this patient group and patients with organic tics (OTs), which can be used in differential diagnosis. The purpose of this retrospective study was to critically examine both established and suggested differences between the patient groups. A total of 53 FTLB patients and 200 OT patients were included. Several findings from the current literature were replicated in this study: Compared to patients with OTs, patients with FTLB had significantly more complex tics, were older at symptom onset, were more likely to be female, and were less likely to have family members with tics. Furthermore, the study also revealed new differences between the groups: Patients with FTLB had significantly more family members with a psychiatric disorder, were more likely to have experienced an adverse psychosocial event immediately before symptom onset, and had significantly fewer simple tics. Finally, this study was unable to replicate the previously found differences in comorbidities between patients with OTs and FTLB. These findings could contribute significantly to the understanding of FTLB’s etiology and to improve diagnosis, as including the presence of simple tics and comorbidities in the diagnostic criteria might be discussed in future studies.

Background and purpose: In 2020, health professionals witnessed a dramatic increase in referrals of young people with rapid onset of severe tic-like behaviours. We assembled a working group to develop criteria for the clinical diagnosis of functional tic-like behaviours (FTLBs) to help neurologists, pediatricians, psychiatrists, and psychologists recognize and diagnose this condition. Methods: We used a formal consensus development process, using a multiround, web-based Delphi survey. The survey was based on an in-person discussion at the European Society for the Study of Tourette Syndrome (ESSTS) meeting in Lausanne in June 2022. Members of an invited group with extensive clinical experience working with patients with Tourette syndrome and FTLBs discussed potential clinical criteria for diagnosis of FTLBs. An initial set of criteria were developed based on common clinical experiences and review of the literature on FTLBs and revised through iterative discussions, resulting in the survey items for voting. Results: In total, 24 members of the working group were invited to participate in the Delphi process. We propose that there are three major criteria and two minor criteria to support the clinical diagnosis of FTLBs. A clinically definite diagnosis of FTLBs can be confirmed by the presence of all three major criteria. A clinically probable diagnosis of FTLBs can be confirmed by the presence of two major criteria and one minor criterion. Conclusions: Distinguishing FTLBs from primary tics is important due to the distinct treatment paths required for these two conditions. A limitation of the ESSTS 2022 criteria is that they lack prospective testing of their sensitivity and specificity.

ourette syndrome and chronic tic disorders are characterized by the presence of tics. Different behavioral therapies have shown to be efficacious for treating tics in children and adolescents, but Exposure and Response Prevention (ERP) is a less researched method. However, ERP is a method often used in the clinical setting. Therefore, the present study evaluated the severity of tics over time from beginning of ERP to follow-up approximately 1 year after last training session. In total, 116 patients treated with ERP face to face or ERP via web-based videoconferencing were included. The primary outcome measure was tic severity measured with the Danish version of the Yale Global Tic Severity Scale. The results showed that tic severity decreased during ERP and lasted in the follow-up period, with a statistically higher decrease in the group with patients who completed ERP as planned and the group that stopped earlier than planned because of reduction in tics, compared with those who dropped out due to lack of motivation (p < 0.001). The study concludes that ERP seems to have an immediate and a long-term effect on severity of tics, especially in those who complete the program or those who discontinue earlier due to good results, Tourette Syndrome and Chronic tic disorders are characterized by the presence of tics. Different behavioral therapies have shown to be efficacious for treating tics in children and adolescents, but Exposure and Response Prevention (ERP) is a less researched method. However, ERP is a method often used in the clinical setting. Therefore, the present study evaluated the severity of tics over time from beginning of ERP to follow-up approximately one year after last training session. 116 patients treated with ERP face-To-face or ERP via web-based videoconferencing were included. Primary outcome measure was tic severity measured with the Danish version of the Yale Global Tic Severity Scale. The results showed that tic severity decreased during ERP and lasted in the follow-up period, with a statistically higher decrease in the group with patients who completed ERP as planned and the group that stopped earlier than planned because of reduction in tics, compared to those who dropped out due to lack of motivation (p<0.001). The study concludes that ERP seems to have an immediate and a long-Term effect on severity of tics, especially in those who complete the programme or those who discontinue earlier due to good results.

Background Global increase in functional tics in adolescents has been observed. Differentiating functional from classic tics is important since pathophysiology and treatment differ. We investigated possible triggers for development of functional tics and discuss the possible role of the COVID-19 pandemic and social media exposure in cases of functional tics seen during this period. Moreover, the treatment, and its efficacy is discussed. Methods Medical records of 28 Danish adolescents diagnosed with functional tics at the National Tourette Clinic, Department of Pediatrics, Herlev University Hospital, from May 2020 to June 2021 have been retrospectively reviewed. Descriptive statistical analyses were used to analyze the data. Main findings N=28 patients diagnosed with functional tics were included, 96.4% girls and 3.6% boys, mean age 14.4 years. Tic phenomenology differed from classic tics with more complex tics and no rostrocaudal progression. 69.2% reported harmful tics. 78.6% had trauma/precipitating event and 40% denounced lockdown related to the COVID-19 pandemic as trigger, both prior to onset of functional tics. 60.7% reported psychiatric symptoms/diagnoses. 42.9% had a first-degree family member with psychiatric symptoms/diagnoses. 96.4% were exposed to tics on social media prior to onset. Treatment consisted of psychoeducation, elements from cognitive behavioral therapy, and focus on psychiatric symptoms. All patients responded to the treatment. Conclusion The vulnerability of the adolescents is characteristic. Treatment strategy has shown immediate positive effect. Consequences of the COVID-19 pandemic in combination with exposure to tics on social media could be part of the cause for the increase in number of functional tics.

Chronic tic disorders, such as Tourette syndrome, are characterized by motor and vocal tics. Tics present a considerable burden for some patients, and therefore, effective treatment is important. One evidence-based treatment option is a behavioral therapy called exposure and response prevention (ERP). Despite its effectiveness, access to ERP remains limited due to a lack of treatment sites. Web-based videoconferences can connect patients at home with a therapist located in the hospital, allowing for treatment delivery over a wide geographic area. The primary aim of this study was to compare the development of tics during and 1 year after ERP delivery, respectively, via web-based videoconferences and traditional face-to-face methods in a naturalistic setting. In total, 116 patients treated using either the face-to-face method (n = 72) or web-based videoconferences (n = 44) were included. The primary outcome measure was tic severity. In both training modalities, tic severity decreased during ERP and the effect lasted in the follow-up period. No statistically significant differences in tic severity between the training modalities were found at baseline, last training session, or at follow-up. Our results suggest that ERP delivered via web-based videoconferences is a good alternative to the traditional face-to-face method, Chronic tic disorders, such as Tourette syndrome, are characterized by motor and vocal tics. Tics present a considerable burden for some patients, and therefore, effective treatment is important. One evidence-based treatment option is a behavioral therapy called exposure and response prevention (ERP). Despite its effectiveness, access to ERP remains limited due to a lack of treatment sites. Web-based videoconferences can connect patients at home with a therapist located in the hospital, allowing for treatment delivery over a wide geographic area. The primary aim of this study was to compare the development of tics during and 1 year after ERP delivery, respectively, via web-based videoconferences and traditional face-to-face methods in a naturalistic setting. In total, 116 patients treated using either the face-to-face method ( n = 72) or web-based videoconferences ( n = 44) were included. The primary outcome measure was tic severity. In both training modalities, tic severity decreased during ERP and the effect lasted in the follow-up period. No statistically significant differences in tic severity between the training modalities were found at baseline, last training session, or at follow-up. Our results suggest that ERP delivered via web-based videoconferences is a good alternative to the traditional face-to-face method.

Background Previous studies have shown that Tourette syndrome (TS) has an impact on academic achievements. The aim of this study was to investigate the association between the severity of tics and comorbidities and educational outcomes. Methods From 2005 to 2007, 395 participants were included in a large cohort (314 with TS and 81 controls) and the mean age was 12.60 ± 2.64 years. The cohort was re-examined after 4 to 8 years (median 5.6) where n = 276 participants (223 with TS and 53 controls) were included with a mean age of 18.52 ± 2.73 years. At both time points, severity of tics and the presence and severity of psychiatric comorbidity were assessed. Educational achievements were assessed through structured interviews. Results Children with TS had a lower passing rate at lower secondary and high school compared to healthy controls. More severe vocal tics were associated with fewer passing lower secondary school at a prospective level. At a cross-sectional level, more severe motor tics were associated with fewer passing high school. Tic severity only influenced children with TS without comorbidity. The severity of comorbidity was found to be associated with the educational level at a longitudinal view, but not cross-sectional. Conclusion Overall, children with TS had a lower passing rate at lower secondary school and high school compared to healthy controls. We found that this difference was more likely driven by the severity of comorbidities than tic severity. It is important to be aware of academic achievement in children with TS in order to give them the right support and thereby optimize educational opportunities.

The clinical symptoms in Tourette syndrome (TS) change with age. In order to examine the course of a disease, longitudinal studies are an important tool. Cortico-striato-thalamo-cortical pathways are believed to be involved in the pathophysiology of TS, but knowledge on the influence of the development of the brain during childhood on the development of clinical symptoms is scarce. The patients with remission of tics and the patients with persistent tics might represent two different phenotypical variants of TS and thus represent two different pathophysiological mechanisms in TS. In this chapter, we focus on longitudinal aspects of the development of TS from clinical and neuroradiological perspectives. Firstly, we describe the clinical development and course of tics and comorbidities and secondly the findings of neurodevelopmental brain imaging studies in TS. Brain imaging studies have shown similar areas in the brain involved in TS in both children and adults. Increased volume of posterior prefrontal cortex and thalamus have been found in both children and adults, and microstructural changes in corpus callosum. Differences between pediatric and adult cross-sectional clinical populations have been shown in volume of corpus callosum. Changes in parallel diffusivity in thalamus, caudate nucleus and frontal lobe have been found to be most pronounced in patients with persisting tics compared with patients with remission of tics and healthy controls. Therefore, these areas might be most interesting to examine in further studies in order to explore the possibility of different phenotypes regarding the course of TS.

During 2020, an increase of functional tics in children and adolescents has been observed. In this review, we present phenotypes, differential diagnosis and treatment for functional tics. We discuss potential contributing causes, like the COVID-19 pandemic and the focus on tics on social media. Functional tics are more complex than tics seen in Tourette syndrome and develop more suddenly in relation to stressors mainly in teenage girls. Psychosocial issues and comorbidities must be addressed and treated by a multidisciplinary team through psychoeducation and if necessary, cognitive-behavioural therapy.

Tic disorders have a strong male predominance, with a male-to-female ratio of 4:1 in Tourette syndrome (TS) and 2:1 in persistent tic disorders. In other neurodevelopmental conditions, such as autism spectrum disorder (ASD) and attention-deficit/hyperactivity disorder (ADHD), the disparity in sex distribution has been partially related to differences in symptom presentation between males and females. In tic disorders, however, little research has been conducted on this topic, probably due to the limited access to large samples with a significant proportion of females. The aim of this study was to describe sex differences in the clinical presentation of tic disorders in children and adolescents in one of the largest pediatric samples with TS/persistent tic disorders (n = 709, 23.3% females) recruited as part of the European Multicenter Tics in Children Study (EMTICS). Validated measures assessed the severity of tics and comorbid psychiatric symptoms. Using mixed-effect models, we found that sex had a significant influence on the severity of tics, ADHD symptoms, ASD symptoms, and emotional problems. Males had more severe symptoms than females, except for emotional problems. We also observed a statistically significant interaction between sex and age on the severity of tics and compulsions, with females showing higher symptom severity with increasing age than males. These findings indicate that the clinical presentation of TS/persistent tic disorders varies with sex. Males seem to exhibit a more noticeable pattern of clinical symptoms at a younger age that may contribute to their earlier detection in comparison to females.

In 2011 a working group of the European Society for the Study of Tourette Syndrome (ESSTS) has developed the first European assessment guidelines for Tourette syndrome (TS). Now, we present an updated version 2.0 of these European clinical guidelines for Tourette syndrome and other tic disorders, part I: assessment. Therefore, the available literature has been thoroughly screened, supplemented with national guidelines across countries and discussions among ESSTS experts. Diagnostic changes between DSM-IV and DSM-5 classifications were taken into account and new information has been added regarding differential diagnoses, with an emphasis on functional movement disorders in both children and adults. Further, recommendations regarding rating scales to evaluate tics, comorbidities, and neuropsychological status are provided. Finally, results from a recently performed survey among ESSTS members on assessment in TS are described. We acknowledge that the Yale Global Tic Severity Scale (YGTSS) is still the gold standard for assessing tics. Recommendations are provided for scales for the assessment of tics and psychiatric comorbidities in patients with TS not only in routine clinical practice, but also in the context of clinical research. Furthermore, assessments supporting the differential diagnosis process are given as well as tests to analyse cognitive abilities, emotional functions and motor skills.

In 2011, the European Society for the Study of Tourette Syndrome (ESSTS) published the first European guidelines for Tourette Syndrome (TS). We now present an update of the part on pharmacological treatment, based on a review of new literature with special attention to other evidence-based guidelines, meta-analyses, and randomized double-blinded studies. Moreover, our revision took into consideration results of a recent survey on treatment preferences conducted among ESSTS experts. The first preference should be given to psychoeducation and to behavioral approaches, as it strengthens the patients' self-regulatory control and thus his/her autonomy. Because behavioral approaches are not effective, available, or feasible in all patients, in a substantial number of patients pharmacological treatment is indicated, alone or in combination with behavioral therapy. The largest amount of evidence supports the use of dopamine blocking agents, preferably aripiprazole because of a more favorable profile of adverse events than first- and second-generation antipsychotics. Other agents that can be considered include tiapride, risperidone, and especially in case of co-existing attention deficit hyperactivity disorder (ADHD), clonidine and guanfacine. This view is supported by the results of our survey on medication preference among members of ESSTS, in which aripiprazole was indicated as the drug of first choice both in children and adults. In treatment resistant cases, treatment with agents with either a limited evidence base or risk of extrapyramidal adverse effects might be considered, including pimozide, haloperidol, topiramate, cannabis-based agents, and botulinum toxin injections. Overall, treatment of TS should be individualized, and decisions based on the patient's needs and preferences, presence of co-existing conditions, latest scientific findings as well as on the physician's preferences, experience, and local regulatory requirements.

Pediatric acute-onset neuropsychiatric syndrome is a clinical concept used to describe a subgroup of children with sudden onset of psychiatric and somatic symptoms. The diagnostic term and especially management of children differs depending on the clinical setting to which they present, and the diagnosis and management is controversial. The aim of this paper is to propose a clinical guidance including homogenous diagnostic work-up and management of paediatric acute onset neuropsychiatric syndrome within the Nordic countries. The guidance is authored by a Nordic-UK working group consisting of paediatric neurologist, child psychiatrists and psychologists from Denmark, Norway, Sweden and Great Britain, and is the result of broad consensus. Conclusion: Consensus was achieved in the collaboration on work-up and treatment of patients with paediatric acute-onset neuropsychiatric syndrome, which we hope will improve and homogenise patient care and enable future collaborative research in the field.

Background: Explosive outbursts occur in 25%–70% of children with Tourette syndrome (TS) and may cause more distress than the tics themselves. Previous studies have indicated that a comorbid diagnosis of attention-deficit/hyperactivity disorder (ADHD) is associated with emotional dysregulation in TS; however, this relationship has almost exclusively been studied using parent-reported questionnaires. Methods: We examined emotion regulation (ER) with an observational measure in 150 medication-naïve children aged 7–12 allocated to four groups: Forty-nine children with TS, 23 children with ADHD, 16 children with TS + ADHD, and 62 typically developing controls. We assessed participants’ ER ability, as well as parent–child interactions in the context of a complex puzzle task, and coded the observed behavior with the Tangram Emotion Coding Manual (TEC-M). We examined group differences in ER, as well as associations between ER and severity of symptoms pertaining to TS and ADHD. Results: Children with TS did not differ from controls in their ER ability. However, children with ADHD and TS + ADHD had more problems with ER than those with TS only and controls. Finally, parents of children with ADHD displayed more tension during the experimental task. ER ability was not associated with tic severity nor premonitory urges; however, better ER ability was associated with less severe symptoms of ADHD. Conclusions: This study is the first to evaluate ER with an observational, clinician-rated measure in a controlled social setting in children with TS. Our findings support earlier questionnaire-based studies by showing impaired ER in children with TS + ADHD, but not in children with TS without comorbidity. These findings inform our understanding of the phenomenology of emotional dysregulation in TS and the role of comorbid disorders.