Tics emergencies and malignant tourette syndrome: Assessment and management.

Tourette syndrome (TS) is a complex neurodevelopmental disorder characterized by the presence of tics, frequently accompanied by a variety of neuropsychiatric comorbidities. A subset of patients with TS present with severe and disabling symptoms, requiring prompt therapeutic intervention. Some of these manifestations may result in medical emergencies when severe motor or phonic tics lead to damage of anatomical structures closely related to the tic. Examples include myelopathy or radiculopathy following severe neck ("whiplash") jerks or a variety of self-inflicted injuries. In addition to self-aggression or, less commonly, allo-aggression, some patients exhibit highly inappropriate behavior, suicidal tendencies, and rage attacks which increase the burden of the disease and are important components of "malignant TS". This subset of TS is frequently associated with comorbid obsessive-compulsive disorder. Therapeutic measures include intensive behavioral therapy, optimization of oral pharmacotherapy, botulinum toxin injections, and deep brain stimulation.

Premonitory Urge in Patients with Tics and Functional Tic-like Behaviors.

BACKGROUND: Premonitory urges (PU) are well described in primary tics, but their frequency and intensity in functional tic-like behaviors (FTLB) are unclear. OBJECTIVE: To study the experience of PU in patients with FTLB. METHODS: We compared the results of the premonitory urge for tics scale (PUTS) in adults with tics and FTLB in the University of Calgary Adult Tic Registry. RESULTS: We included 83 patients with tics and 40 with FTLB. When comparing patients with tics, FTLB with tics and FTLB only, we did not detect significant differences either in the total PUTS score (P = 0.39), or in any of the individual PUTS item sub-scores (P values ranging between 0.11 and 0.99). CONCLUSIONS: Patients with FTLB report PU at similar frequency and intensity to patients with tics. This finding confirms that PU are not a useful feature to discriminate FTLB from tics.

Functional Tic-Like Behaviors: A Common Comorbidity in Patients with Tourette Syndrome.

BACKGROUND: Comorbid functional tic-like behaviors (FTB) have been described only rarely in patients with Tourette syndrome (TS). OBJECTIVES: We present the first large sample of patients suffering from TS and FTB to raise awareness of this clinical presentation and to guide how to differentiate one from the other. METHODS: We analyzed clinical data of 71 patients (n = 27 [38.0%] female, mean age: 21.5, range: 11-55) with TS + FTB. RESULTS: In the majority of patients, FTB started abruptly on average 15 years after tic onset with "treatment-resistant" complex movements and ("coprophenomena-like") vocalizations preceded by timely related psychological stressors. Psychological evaluation revealed evidence for internal conflicts (79%), emotional dysregulation (56%), and maintaining factors (70%). About one third of patients had a positive history for further medically unexplained symptoms. Compared to a large TS sample (n = 1032), patients with TS + FTB were more likely to be female, and presented significantly more common with "coprophenomena-like" symptoms, atypical influential factors, atypical descriptions of premonitory sensations, and higher rates of comorbid obsessive-compulsive disorder and "self-injurious" behavior. CONCLUSIONS: Based on our data it can be assumed that FTB is a common comorbidity in TS, similar to functional overlay in other movement disorders and epilepsy. Before classifying a patient as suffering from treatment-resistant TS, FTB should be ruled out.

[Attention deficit/hyperactivity disorder (ADHD) and Tourette disorder (TD): between comorbidity and differential diagnosis]. / Trastorno por déficit de atención/hiperactividad (TDAH) y trastorno de Tourette (TT): entre la comorbilidad y el diagnóstico diferencial.

Tourette Disorder (TD) and attention deficit hyperactivity disorder (ADHD) are both major neuropsychiatric conditions that usually begin during infancy This revision aims to collaborate with pediatricians, who are often confronted with the question of co-morbidity or differential diagnosis between ADHD and TD. The question becomes urgent when the clinician must decide if he/she can start ADHD or TD treatment. We encourage our colleagues to revise our findings, based in bimolecular and neuroanatomic shared issues in addition to updated epidemiological findings. The clinician will find an original proposed algorithm that they can use when the shared symptoms are present in a little patient. TD and ADHD must be intervened early, so we can get better outcomes. The consequences of letting the symptoms increase can generate sequels and handicaps, that can interfere with the quality of life and functionality not only during infancy and adolescence but also in adult life.

El Trastorno de Tourette (TT) y el Trastorno por déficit de atención / hiperactividad (TDAH) son entidades neuropsiquiátricas que usualmente inician en la infancia. Esta revisión busca colaborar con los clínicos, quienes suelen confrontarse al dilema de saber si existe una comorbilidad o un diagnóstico diferencial, ya que esta pregunta cobra vital importancia en el momento de decidir el tratamiento. Invitamos al colega a revisar nuestros hallazgos, soportados por bases moleculares, fisiológicas y neuroanatómicas, además de los datos epidemiológicos. Al final, brindamos una propuesta de algoritmo diagnóstico que podrá utilizar cuando se encuentre ante síntomas compartidos entre los dos diagnósticos. El TDAH y el TT deben ser intervenidos tempranamente, para mejorar la calidad de vida y funcionalidad del paciente y prevenir secuelas, no solo en niños, niñas y adolescentes (NNA), también a lo largo de la vida.

Advancements in the phenomenology, assessment, and treatment of Tourette syndrome.

PURPOSE OF REVIEW: Tourette syndrome (TS) and chronic tic disorders, collectively referred to as TS, are relatively common childhood onset neuropsychiatric conditions associated with functional impairment and distress. Over the past several years, clinical research has contributed to the advancement of the field's understanding of mechanisms and clinical correlates of TS. This progress has led to the development of key assessment tools and the implementation of novel interventions for individuals with TS. RECENT FINDINGS: This article provides a review of innovative TS research focusing on four key themes: investigation of clinical phenomenology of TS; validation of assessment tools for TS; dissemination of current evidence-based treatments for TS; and exploration of new intervention programs. SUMMARY: Cumulatively, this growing body of work presents considerable progress and provides a path forward to improve the assessment and treatment of TS.

Pharmacological treatment of Tourette's syndrome: from the past to the future.

Tourette's syndrome (TS) is a neuropsychiatric disease featuring tics and vocal tics, with a prevalence of approximately 1%, including 75% of the total number of male patients. TS seriously disturbs the patients' career, education, and life and brings a serious and unbearable psychological burden to the patients themselves and their families. At present, there are no specific clinical medications recommended for treating TS. Therefore, it is necessary to select the appropriate medication for symptomatic treatment based on the doctor's personal experience and the patient's symptoms, with the main goal of relieving symptoms, thus improving the patient's social skills and psychological problems. Here we conducted a comprehensive search on PubMed to review and organize the history and current status of the development of drug therapy for TS through a timeline format. We also systematically evaluated the effects of each drug for TS treatment to summarize the current problems and new research directions and to provide some ideas for clinical treatment.

Co-morbid tics and stereotypies: a systematic literature review.

BACKGROUND: Tics and stereotypies are childhood-onset repetitive behaviours that can pose significant diagnostic challenges in clinical practice. Both tics and stereotypies are characterised by a complex co-morbidity profile, however little is known about the co-occurrence of these hyperkinetic disorders in the same patient population. OBJECTIVE: This review aimed to assess the relationship between tics and stereotypies when these conditions present in co-morbidity. METHODS: We conducted a systematic literature review of original studies on co-morbid tics and stereotypies, according to the standards outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS: Our literature search identified six studies of suitable sample size (n ≥ 40) presenting data on the association between tics and stereotypies in otherwise typically developing patients. A considerable proportion (23%) of patients diagnosed with stereotypic movement disorder present with co-morbid tics (range 18-43%). Likewise, the prevalence of primary stereotypies is increased in patients with tic disorders such as Tourette syndrome (8%, range 6-12%). DISCUSSION: Tics and stereotypies can often develop in co-morbidity. The association of tics and stereotypies in the same patient has practical implications, in consideration of the different treatment approaches. Future research should focus on the assessment and management of both conditions, particularly in special populations (e.g. patients with pervasive developmental disorders).

What distinguishes patients with mass social media-induced illness presenting with Tourette-like behavior from those with Tourette syndrome? Results of a prospective cohort study.

Since 2019, a global increase in patients presenting with functional Tourette-like behaviors (FTB) has been observed. This has been related to the exposure of tic-related content in social media, although other factors seem to further fuel this phenomenon. Recently, we, therefore, proposed the term mass social media-induced illness (MSMI) as, in our opinion, this phenomenon constitutes a new type of mass sociogenic illness (MSI) that is in contrast to all recent outbreaks spread solely via social media. In accordance with this hypothesis, we were able to identify the host of the German YouTube channel "Gewitter im Kopf" ("Thunderstorm in the brain") as the initial virtual index case. The purpose of this paper is to present clinical characteristics of a sample of 32 patients diagnosed with MSMI-FTB compared to a large sample of patients with Tourette syndrome (TS) and other chronic tic disorders (CTD) (n = 1032) from the same center in Germany indicating clinical factors helpful to distinguish between tics in TS/CTD and MSMI-FTB. Our main findings were: in patients with MSMI-FTB compared to those with TS/CTD we found (i) a significantly higher age at onset, (ii) a significantly higher rate of females, (iii) a significantly higher rate of obscene and socially inappropriate symptoms, (iv) a significantly lower rate of comorbid ADHD, and (v) a significantly lower rate of OCD/OCB. In contrast, rates of comorbid anxiety and depression as well as reported frequencies of premonitory urges/sensations and suppressibility of symptoms did not differ between groups.

Automated Quantification of Eye Tics Using Computer Vision and Deep Learning Techniques.

BACKGROUND: Tourette syndrome (TS) tics are typically quantified using "paper and pencil" rating scales that are susceptible to factors that adversely impact validity. Video-based methods to more objectively quantify tics have been developed but are challenged by reliance on human raters and procedures that are resource intensive. Computer vision approaches that automate detection of atypical movements may be useful to apply to tic quantification. OBJECTIVE: The current proof-of-concept study applied a computer vision approach to train a supervised deep learning algorithm to detect eye tics in video, the most common tic type in patients with TS. METHODS: Videos (N = 54) of 11 adolescent patients with TS were rigorously coded by trained human raters to identify 1.5-second clips depicting "eye tic events" (N = 1775) and "non-tic events" (N = 3680). Clips were encoded into three-dimensional facial landmarks. Supervised deep learning was applied to processed data using random split and disjoint split regimens to simulate model validity under different conditions. RESULTS: Area under receiver operating characteristic curve was 0.89 for the random split regimen, indicating high accuracy in the algorithm's ability to properly classify eye tic vs. non-eye tic movements. Area under receiver operating characteristic curve was 0.74 for the disjoint split regimen, suggesting that algorithm generalizability is more limited when trained on a small patient sample. CONCLUSIONS: The algorithm was successful in detecting eye tics in unseen validation sets. Automated tic detection from video is a promising approach for tic quantification that may have future utility in TS screening, diagnostics, and treatment outcome measurement. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Prospective follow-up study of youth and adults with onset of functional tic-like behaviours during the COVID-19 pandemic.

BACKGROUND AND PURPOSE: Very little is known about the long-term prognosis of patients with functional tic-like behaviours (FTLBs). We sought to characterize the trajectory of symptom severity over a 12-month period. METHODS: Patients with FTLBs were included in our prospective longitudinal child and adult clinical tic disorder registries at the University of Calgary. Patients were prospectively evaluated 6 and 12 months after their first clinical visit. Tic inventories and severity were measured with the Yale Global Tic Severity Scale (YGTSS). RESULTS: Eighty-three youths and adults with FTLBs were evaluated prospectively until April 2023. Mean YGTSS total tic severity scores were high at baseline, with a mean score of 29.8 points (95% confidence interval [CI] = 27.6-32.1). Fifty-eight participants were reevaluated at 6 months, and 32 participants were reevaluated at 12 months. The YGTSS total tic severity score decreased significantly from the first clinical visit to 6 months (raw mean difference = 8.9 points, 95% CI = 5.1-12.7, p < 0.0001), and from 6 to 12 months (raw mean difference = 6.4 points, 95% CI = 0.8-12.0, p = 0.01). Multivariable linear regression demonstrated that tic severity at initial presentation and the presence of other functional neurological symptoms were associated with higher YGTSS total tic scores at 6 months, whereas younger age at baseline, receiving cognitive behavioural therapy for anxiety and/or depression, and prescription of selective serotonin reuptake inhibitors were associated with lower YGTSS total tic scores at 6 months. CONCLUSIONS: We observed a meaningful improvement in tic severity scores in youth and adults with FTLBs over a period of 6-12 months.

A Family Genetic Study of Obsessive Compulsive Disorder in Youth.

OBJECTIVE: To use a family genetic study to evaluate familial risk of obsessive compulsive disorder (OCD) and common comorbid illnesses in first-degree relatives of pediatric-onset probands with primary OCD. METHOD: One hundred and thirty youth with OCD and their 133 siblings and 241 parents and 49 pediatric controls were directly evaluated along multiple domains including psychopathology using structured diagnostic interviews and clinical corroboration. RESULTS: Rates of anxiety, mood, disruptive behavior, and tic disorders were markedly elevated in the probands while rates in siblings were elevated at rates between the probands and controls. Twenty six percent of first-degree relatives had clinical OCD, 9% had chronic tics or Tourette's disorder, and 21% met criteria for ADHD. CONCLUSION: Rates of familial transmission of OCD and common comorbid illnesses were significantly higher in our pediatric-onset probands than rates reported in the literature in relatives of those with adult-onset OCD.

Cytokine levels reflect tic symptoms more prominently during mild phases.

Tic disorder is a neuropsychiatric condition that affects 3% of all children and can have a significant impact on their quality of life. Cytokines, interferons, interleukins, lymphokines, and tumor necrosis factors are involved in the neuroinflammatory circuitry of tic disorders. This study aimed to identify the cytokines involved in the pathogenesis of tic disorders. We enrolled 44 patients with tic disorder and 38 healthy controls. Patients were free of psychotropic medications for at least 3 weeks. Whole blood samples were analyzed using a Luminex® human cytokine multiplex assay kit. Patients were divided into groups with "mild tics" and "above moderate tics" based on Yale Global Tic Severity Scale (YGTSS) scores for comparison. The final analysis included 35 patients (28 male and 7 female) and 31 controls (20 male and 11 female). In the mild tic group, interleukin (IL)-12 p70 negatively correlated with motor tic scores. Granulocyte-macrophage colony-stimulating factor, IL-4, IL-8, and tumor necrosis factor (TNF)-α were positively correlated to phonic tic scores. IL-12 p40 and TNF-α were positively correlated to total tic scores. IL-12 p70 and IL-17a negatively correlated to impairment scores and total YGTSS scores. Tic disorder patients and healthy controls exhibit different cytokine profiles. Only patients with mild symptoms exhibit significant correlations, suggesting that the correlations between cytokine levels and tic symptoms are more relevant during the mild or remission phases. Our results present the importance of IL-1ß and TNF-α, among others, but the identification of key cytokines are still necessary.

Non-just-right experiences are more closely related to OCD than tics in Tourette patients.

Complex tics and obsessive or compulsive behaviour can be difficult to differentiate diagnostically. The majority of adult patients with Tourette syndrome report experiencing premonitory urges before tics. Some of these experiences have been linked to non-just-right experiences (NJRE), which are frequently reported by patients with obsessive-compulsive disorder or behaviours (OCD/OCB). We aimed to assess whether NJRE are more closely related to tics and tic-associated premonitory urges or whether they are more closely associated with OCD. A total of N = 111 patients (mean age = 34.77 + /-12.93; N = 37 female) with a confirmed diagnosis of Tourette syndrome completed the premonitory urges for tic disorders scale (PUTS), the revised non-just-right experiences scale (NJRE-QR), and questionnaires regarding their tic severity, and comorbid OCD/OCB. A multi-trait-multi-methods matrix was calculated to examine associations amongst scales measuring tic-related and OCB-related phenomena. The PUTS correlated overall higher with tic questionnaires than with OCD/OCB questionnaires. The NJRE correlated higher with OCD symptoms than with tic severity. The results indicate that non-just-right experiences are more closely associated with comorbid OCB than with tics in patients with Tourette syndrome.

Creative thinking in Tourette's syndrome: A comparative study of patients and healthy controls.

INTRODUCTION: Tourette's syndrome is a neurological disorder characterized by tics, that may interfere with patients' everyday life. Research suggested that creative thinking (namely, divergent and convergent thinking) could help patients cope with their symptoms, and therefore it can be a resource in non-pharmacological interventions. The present study aimed at investigating (i) possible differences in creative thinking between Tourette's syndrome patients and healthy controls and (ii) whether creative thinking can support patients in coping with their symptomatology. METHODS: A group of 25 Tourette's syndrome patients and 25 matched healthy controls underwent an assessment of creative thinking, fluid intelligence, and depressive symptoms. Creative thinking was compared between patients and healthy controls after controlling for fluid intelligence and depressive symptoms. Moreover, the moderating role of divergent and convergent thinking on the subjective impact of tics was tested in a group of 30 patients. RESULTS: Tourette's syndrome patients outperformed healthy controls in convergent thinking. Moreover, divergent thinking was found as a significant moderator of the relationship between tics severity and the subjective impact in Tourette's syndrome patients. CONCLUSIONS: Findings highlighted the specific impact of convergent and divergent thinking on Tourette's syndrome patients. Considering the supportive role of creative thinking in Tourette's syndrome, our results confirm that higher levels of divergent thinking may reduce the tic-related discomfort. These findings suggest the potential positive implications of creative thinking in non-pharmacological interventions for Tourette's syndrome.

Tourette syndrome and other tic disorders of childhood.

Tics are repetitive, patterned, and nonrhythmic movements or vocalizations/audible sounds that are misplaced in context. Phenomenology and characteristics of tics (e.g., premonitory urge, suppressibility) differentiate them from compulsions, stereotypies, functional tic-like behaviors, and other types of hyperkinetic movement disorders. With a prevalence of approximately 1% in school-aged boys, Tourette syndrome (TS) is considered a common childhood-onset neurodevelopmental disorder, defined by the combination of at least two motor tics and at least one phonic tic lasting more than 1 year. TS is a highly heritable disorder, with a wide spectrum of severity. In some individuals, tics can cause pain, distress, functional impairment, or stigmatization. About 90% of individuals with TS have at least one mental health comorbidity (attention-deficit/hyperactivity disorder, obsessive-compulsive disorder, anxiety/depressive disorders). These comorbidities significantly impact patients' quality of life and must therefore be screened and managed accordingly in this population. Treatment of tics is based on behavioral therapies targeting tics (habit reversal training included in the comprehensive behavioral intervention for tics, and exposure and response prevention for tics), in association with medication if needed (e.g., alpha-2-agonists, second-generation antipsychotics). Deep brain stimulation is considered an experimental option in the most severe, treatment-resistant patients. In adulthood, less than 25% of individuals still have moderate or severe tics.

Study protocol: A cross-sectional survey of clinicians to identify barriers to clinical practice guideline implementation in the assessment and treatment of persistent tic disorders.

INTRODUCTION: Eight members of the International Parkinson's Disease and Movement Disorders Society Tic and Tourette Syndrome Study Group formed a subcommittee to discuss further barriers to practice guideline implementation. Based on expert opinion and literature review, the consensus was that practice variations continue to be quite broad and that many barriers in different clinical settings might negatively influence the adoption of the American Academy of Neurology and the European Society for the Study of Tourette Syndrome published guidelines. OBJECTIVES: 1) To identify how clinical practices diverge from the existing American Academy of Neurology and European Society for the Study of Tourette Syndrome guidelines, and 2) to identify categories of barriers leading to these clinical care gaps. METHODS AND ANALYSIS: This article presents the methodology of a planned cross-sectional survey amongst healthcare professionals routinely involved in the clinical care of patients with persistent tic disorders, aimed at 1) identifying how practices diverge from the published guidelines; and 2) identifying categories of barriers leading to these clinical care gaps. Purposeful sampling methods are used to identify and recruit critical persistent tic disorders stakeholders. The analysis will use descriptive statistics.

Functional tics: Expanding the phenotypes of functional movement disorders?

BACKGROUND AND PURPOSE: Until the outbreak reported during the COVID-19 pandemic, functional tics were considered to be a relatively rare clinical phenotype, as opposed to other functional movement disorders such as functional tremor and dystonia. To better characterize this phenotype, we compared the demographic and clinical characteristics of patients who developed functional tics during the pandemic and those of patients with other functional movement disorders. METHODS: Data from 110 patients were collected at the same neuropsychiatry centre: 66 consecutive patients who developed functional tics without other functional motor symptoms or neurodevelopmental tics and 44 patients with a mix of functional dystonia, tremor, gait, and myoclonus. RESULTS: Both groups were characterized by female sex preponderance (70%-80%) and (sub)acute onset of functional symptoms (~80%). However, patients with functional tics had a significantly earlier age at onset of functional symptoms (21 vs. 39 years). Exposure to relevant social media content was reported by almost half of the patients with functional tics, but by none of the patients with other functional movement disorders. Comorbidity profiles were similar, with relatively high rates of anxiety/affective symptoms and other functional neurological symptoms (nonepileptic attacks). CONCLUSIONS: Patients who developed functional tics during the pandemic represent a phenotypic variant of the wider group of patients with functional movement disorders, associated with younger age at onset and influenced by pandemic-related factors, including increased exposure to specific social media content. Diagnostic protocols and treatment interventions should be tailored to address the specific features of this newly defined phenotype.

Decoupling: adaptation of a treatment for body-focused repetitive behaviour to Tourette syndrome. A case report.

AIMS: Tourette syndrome (TS) is a neurological condition; its etiology is not yet fully understood. Cognitive behavioural therapy with habit reversal training is the recommended first-line treatment, but is not effective in all patients. This is the first report examining the usefulness of decoupling, a behavioural self-help treatment originally developed for patients with body-focused repetitive behaviours, in a patient with TS. METHOD: Patient P.Z. showed 10 motor and three vocal tics on the Adult Tic Questionnaire (ATQ) before treatment. He was taught decoupling by the first author. RESULTS: The application of decoupling led to a reduction of P.Z.'s eye tics, which was one of his first and most enduring and severe tics. It was not effective for other areas. Quality of life and depression improved, which P.Z. attributed to the improvement of his tics. CONCLUSION: Decoupling may be adopted as an alternative, when habit reversal training is not feasible. Future research, preferably using a controlled design with a large sample, may elucidate whether decoupling is only effective for tics relating to the eyes, the most common symptom in tic disorder/TS, or whether its effects extend to other symptoms.