Screening for Quality of Life in a Neurology Tic Clinic Using Quality Improvement Methodology.

BACKGROUND: Tic disorders in children often co-occur with other disorders that can significantly impact functioning. Screening for quality of life (QoL) can help identify optimal treatment paths. This quality improvement (QI) study describes implementation of a QoL measure in a busy neurology clinic to help guide psychological intervention for patients with tics. METHODS: Using QI methodology outlined by the Institute for Healthcare Improvement, this study implemented the PedsQL Generic Core (4.0) in an outpatient medical clinic specializing in the diagnosis and treatment of tic disorders. Assembling a research team to design process maps and key driver diagrams helped identify gaps in the screening process. Conducting several plan-do-study-act cycles refined identification of patients appropriate to receive the measure. Over the three-year study, electronic health record notification tools and data collection were increasingly utilized to capture patients' information during their visit. RESULTS: Over 350 unique patients were screened during the assessment period. Electronic means replaced paper measures as time progressed. The percentage of patients completing the measure increased from 0% to 51.9% after the initial implementation of process improvement, advancing to 91.6% after the introduction of electronic measures. This average completion rate was sustained for 15 months. CONCLUSIONS: Using QI methodology helped identify the pragmatics of implementing a QoL assessment to enhance screening practices in a busy medical clinic. Assessment review at the time of appointment helped inform treatment and referral decisions.

Treatment failure in persistent tic disorders: an expert clinicians' consensus-based definition.

A standardized definition of treatment failure in the management of tics is currently lacking. Such definition would prevent persistent use of unnecessary interventions and help clinicians to determine when to offer less established treatments (e.g., deep brain stimulation surgery). To achieve an expert consensus-based definition of failure of medical treatments for tics, we used a multi-step, multi-round, web-based Delphi approach involving international specialist clinicians with specific expertise in tic disorders. These experts were identified through professional networks or consortia related to chronic tic disorders. We created a survey and reviewed the questions with stakeholders prior to two rounds of Delphi surveys, followed by a final review and discussion among research team members. Both survey rounds were completed using a sample of 36 expert stakeholders from 14 countries, including neurologists, psychiatrists, and clinical psychologists. The Delphi process led to consensus on 10 statements which formed the final definition of treatment failure. The definition was structured and operationalized according to two separate sections, one for behavioral and one for pharmacological treatments. Core components of the definition and its operationalization included lack of efficacy, adherence, and tolerability, as well as a definition of failure of behavioral therapies as a whole, and of pharmacological therapies as a whole. The group concluded that the components of this specific definition reflect the range and complexity of characteristics to consider in establishing tic-related treatment failure. Future research should assess the feasibility of this operational definition and whether it will change clinical decision-making and improve management outcomes.

Treatments for Tourette syndrome in children and young adults: a systematic review / Tratamento da síndrome de Tourette em crianças e jovens adultos: uma revisão sistemática

INTRODUCTION: Tourette's Syndrome (TS) is a neurodevelopmental disorder characterized by motor and / or vocal tics for more than 12 months. TS affects about 0.8% of pediatric patients and is associated with great functional impairment and psychological distress. The present study aims to list and compare the effectiveness of therapies used in children and young people with TS. METHODS: PubMed / MEDLINE, Cochrane Library, ScienceDirect, SciELO and Lilacs were used from September 2020 to April 2021 to search for randomized clinical trials with pharmacological, behavioral, physical or alternative interventions for tics in children and young people with ST. RESULTS: 13 clinical trials were included, of which six pharmacological, six behavioral and one of other conformation. The global score on the Yale Global Tic Severity Scale showed evidence in favor of Habit Reversal Training (HRT) and Comprehensive Behavioral Intervention for Tics (CBIT). Evidence from two studies suggests that antipsychotic medications improve tic scores. Evidence from other interventions has shown no conclusive benefit. CONCLUSIONS: The present study identified benefits with the use of antipsychotics. The study also found that HRT and CBIT showed improvement in reducing the severity of tics, in addition to not having any adverse effects. These therapies showed significant clinical improvement, but there is no comparison between the use of these isolated approaches in relation to their use associated with medications. In view of the different forms of therapy, further studies are needed to identify the effectiveness and the profile of adverse effects of these interventions.

INTRODUÇÃO: A Síndrome de Tourette (ST) é um distúrbio do neurodesenvolvimento caracterizado por tiques motores e/ou vocais por mais de 12 meses. A ST afeta cerca de 0,8% dos pacientes pediátricos e associa-se a grande comprometimento funcional e sofrimento psíquico. O presente estudo tem como objetivo listar e comparar a eficácia das terapias utilizadas em crianças e jovens com ST. MÉTODOS: PubMed/MEDLINE, Cochrane Library, ScienceDirect, SciELO e Lilacs foram usados desde setembro de 2020 até abril de 2021 para a busca de ensaios clínicos randomizados com intervenções farmacológicas, comportamentais, físicas ou alternativas para tiques em crianças e jovens com ST. RESULTADOS: 13 ensaios clínicos foram incluídos, dos quais seis farmacológicos, seis comportamentais e um de outra conformação. A pontuação global na Yale Global Tic Severity Scale, apresentou evidências a favor do Treinamento de Reversão de Hábito (TRH) e Intervenção Comportamental Abrangente para Tiques (ICAT). As evidências de dois estudos sugerem que medicamentos antipsicóticos melhoram os escores de tiques. Evidências de outras intervenções não mostraram nenhum benefício conclusivo. CONCLUSÕES: O presente estudo identificou benefícios com o uso do antipsicóticos. O estudo também identificou que a TRH e a ICAT apresentaram melhora na redução da gravidade dos tiques, além de não apresentarem efeitos adversos. Essas terapias mostraram importante melhora clínica, mas não há comparação entre o uso dessas abordagens isoladas em relação ao seu uso associado com medicamentos. Diante das diferentes formas de terapia, mais estudos são necessários para identificar a eficácia e o perfil de efeitos adversos dessas intervenções.

Stereotactic radiofrequency thermocoagulation application in the anterior limbs of patients' internal capsules in treating intractable tic disorders.

Purpose: To investigate the effect of applying stereotactic radiofrequency thermocoagulation in the anterior limbs of patients' internal capsules in treating intractable tic disorders.Materials and methods: Patients diagnosed with intractable tic disorders were prospectively enrolled and treated using stereotactic radiofrequency thermocoagulation in the anterior limbs of the internal capsules. Periprocedural complications, effects, and follow-up outcomes were then analyzed.Results: Fifty patients were enrolled, including 38 with Tourette syndrome and 12 with persistent refractory vocal or motor tic disorders. The radiofrequency thermocoagulation procedure was performed successfully in all patients. Five participants (10%) experienced periprocedural complications, including one having a slight hemiplegia, two developing fevers (4%), and two developing urination disorders (4%). The participants underwent a follow-up for 12 months, with excellent effects being achieved in 23 patients (46%), prominent results in 13 (26%), good results in 10 (20%), and invalid results in 4 (8%), reaching an efficacy rate of 92% (46/50). Thirty-six patients experienced excellent and prominent effects, with no additional management after the radiofrequency ablation being needed, achieving a success rate of 72%. After radiofrequency thermocoagulation, the Yale Global Tic Severity Scale (YGTSS) scores were significantly reduced (p < .01) when compared with those before the procedure. Following this procedure, participants' serum dopamine levels (SDA) significantly decreased (p < .05), while their serotonin levels were significantly elevated (p < .05) when compared to the measurements taken before the procedure.Conclusion: Stereotactic radiofrequency thermocoagulation applied to the anterior limbs of patients' internal capsules may be effective for treating intractable tic disorders, without risk of serious complications.

Tics and stereotypies: A comparative clinical review.

Tics and stereotypies are the most common pathological repetitive complex motor behaviors occurring during the neurodevelopmental period. Although they may appear transiently during development without acquiring a pathological status, when they become chronic they may be distressing, socially impairing, or even, in the case of malignant tics, potentially physically harmful. Despite a certain similarity in their phenomenology, physicians should be able to distinguish them for their different variability over time, topographical distribution, association with sensory manifestations, and relationship with environmental triggers. The complex phenomenology of tics and stereotypies is constantly enriched by the characterization of novel variants, e.g. tics triggered by auditory stimuli in association with misophonia and stereotypies associated with intense imagery activity. Their pathophysiology remains partially elusive, but both animal model and brain imaging studies confirm the involvement of all the three major loops (sensorimotor, associative and limbic) within the cortico-basal ganglia circuitry. From a management perspective, the greatest advances witnessed in the last decade involve the diffusion of behavioral strategies (e.g. habit reversal training or response interruption and redirection), including the development of protocols for telehealth on online training in order to optimise access. In the context of severe tics, e.g. in refractory Tourette syndrome, there is increasing experience with deep brain stimulation of the intralaminar thalamic nuclei or the globus pallidus internus, although more research is needed to fine tune target choice and stimulation setting definition.

Tourette syndrome and other chronic tic disorders: an update on clinical management.

INTRODUCTION: The management of Tourette syndrome (TS) and other chronic tic disorders occurs in multiple stages and begins with comprehensive assessment and complex psychoeducation. Behavioral and pharmacological interventions (second stage) are needed when tics cause physical or psychosocial impairment. Deep brain stimulation surgery or experimental therapies represent the third stage. Areas covered: Discussed are recent advances in assessment and therapy of chronic tic disorders, encompassing the three stages of intervention, with the addition of experimental, non-invasive brain stimulation strategies. A PubMed search was performed using as keywords: 'tic disorders', 'Tourette syndrome', 'assessment', 'rating scales', 'behavioral treatment', 'pharmacological treatment', 'deep brain stimulation', 'transcranial magnetic (or current) stimulation', and 'transcranial current stimulation'. More than 300 peer-reviewed articles were evaluated. The studies discussed have been selected on the basis of novelty and impact. Expert commentary: Comprehensive assessment of tic disorders and psychoeducation are crucial to a correct active management approach. Behavioral treatments represent first line of active interventions, with increasing potential offered by telehealth. Antipsychotics and alpha agonists remain first line pharmacological interventions for tics, although VMAT-2 inhibitors appear promising. Deep brain stimulation is a potential option for medically refractory, severely disabled patients with tics, but age and target selection require further investigation.

Motor Improvement and Emotional Stabilization in Patients With Tourette Syndrome After Deep Brain Stimulation of the Ventral Anterior and Ventrolateral Motor Part of the Thalamus.

BACKGROUND: Since its first application in 1999, the potential benefit of deep brain stimulation (DBS) in reducing symptoms of otherwise treatment-refractory Tourette syndrome (TS) has been documented in several publications. However, uncertainty regarding the ideal neural targets remains, and the eventuality of so far undocumented but possible negative long-term effects on personality fuels the debate about the ethical implications of DBS. METHODS: In this prospective open-label trial, eight patients (three female, five male) 19-56 years old with severe and medically intractable TS were treated with high-frequency DBS of the ventral anterior and ventrolateral motor part of the thalamus. To assess the course of TS, its clinical comorbidities, personality parameters, and self-perceived quality of life, patients underwent repeated psychiatric assessments at baseline and 6 and 12 months after DBS onset. RESULTS: Analysis indicated a strongly significant and beneficial effect of DBS on TS symptoms, trait anxiety, quality of life, and global functioning with an apparently low side-effect profile. In addition, presurgical compulsivity, anxiety, emotional dysregulation, and inhibition appeared to be significant predictors of surgery outcome. CONCLUSIONS: Trading off motor effects and desirable side effects against surgery-related risks and negative implications, stimulation of the ventral anterior and ventrolateral motor part of the thalamus seems to be a valuable option when considering DBS for TS.

The role of tonsillectomy in the treatment of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS).

IMPORTANCE: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) in children describes neuropsychiatric symptom exacerbations that relate temporally to streptococcal infections. Recent case reports suggest tonsillectomy may effectively reduce these symptoms; however, no consensus treatment guidelines exist. This study examines whether tonsillectomy improves neuropsychiatric symptoms in children with PANDAS who have incomplete response to antibiotic therapy. OBSERVATIONS: Ten patients met strict diagnostic criteria for PANDAS. Comparisons were made between parental reports of symptom severity at diagnosis, after antibiotic treatment (in 10 patients), and after tonsillectomy (in 9). From a baseline severity score of 10, antibiotics alone improved symptoms to a median (interquartile range [IQR]) score of 8 (6.5-10.0) (P = .03). Nine children who subsequently underwent tonsillectomy reported symptom improvement in comparison with treatment with antibiotics alone, including those with no response to antibiotics. Symptom severity improved at all periods after tonsillectomy compared with antibiotics alone. The median score [IQR] 3 months postoperatively was 3 (0.0-6.5) (P = .01); 6 months postoperatively, 3 (0.0-5.0) (P = .02); 1 year postoperatively, 3 (0.0-5.0) (P = .02); and 3 years postoperatively, 0.5 (0.0-2.3) (P = .03). Four of the 9 had complete resolution after tonsillectomy. CONCLUSIONS AND RELEVANCE: This PANDAS cohort whose neuropsychiatric symptoms did not respond sufficiently to antibiotics may have gained benefit from tonsillectomy.

Phenomenology and natural history of Tourette syndrome: brief summary or research / Fenomenologia e história natural da síndrome de Tourette: breve resumo da pesquisa

The phenomenology of Tourette syndrome is complex. Although overt motor and vocal tics are the defining features of Tourette syndrome, many individuals report experiencing sensory "urges," which are often difficult to describe. The natural history of this condition is also variable, with some individuals experiencing a marked reduction in tics by the end of the second decade of life while others go on to have a lifelong condition. The aim of this thesis was three-fold: (1) to develop a valid and reliable clinical rating instrument; (2) to investigate the sensory phenomena associated with Tourette syndrome; and (3) to document the course of tic severity over the course of the first two decades of life. Each of these three studies involved groups of patients with Tourette syndrome or a chronic tic disorder and each of these studies has been published in a peer-reviewed journal. The Yale Global Tic Severity Scale (YGTSS) has excellent psychometric properties that have been independently replicated. It has also emerged as the most widely used clinician-rated tic severity scale in randomized clinical trials around the world. Sensory phenomena, particularly premonitory urges, are commonly reported among individuals with Tourette syndrome by the age of 10 years. There is considerable overlap with the sensory phenomena described by individuals with Obsessive-Compulsive Disorder. Tics usually have their onset in the first decade of life. They then follow a waxing and waning course and a changing repertoire of tics. As documented in the third study, for a majority of patients the period of worst tic severity usually falls between the ages of 7 and 15 years of age, after which tic severity gradually declines. This falloff in tic symptoms is consistent with available epidemiological data that indicate a much lower prevalence of Tourette syndrome among adults than children. This decline in tic severity has been confirmed in subsequent studies...

A fenomenologia da síndrome de Tourette (ST) é complexa. Apesar de tiques motores e vocais serem as características definidoras da síndrome, muitas pessoas relatam ter urgências premonitórias (fenômenos sensoriais) de difícil descrição. A história natural da ST também é variável, com alguns indivíduos que experimentam uma redução acentuada nos tiques até o final da segunda década de vida, enquanto outros permanecem com sintomas ao longo de toda a vida adulta. Os objetivos principais desta tese são três: (1) desenvolver um instrumento de avaliação clínica com boa validade e confiabilidade para ST; (2) investigar os fenômenos sensoriais (FS) associados a ST; e (3) documentar o curso da gravidade dos tiques durante as duas primeiras décadas de vida. Para atingir esses objetivos incluíram-se grupos de pacientes clinicamente bem caracterizados e de artigos científicos publicados em periódicos internacionais de alto impacto. A Escala de Gravidade Global de tiques de Yale (YGTSS) apresentou excelentes propriedades psicométricas, o que foi replicado em estudos independentes. Também emergiu como a escala de gravidade mais utilizada em ensaios clínicos randomizados para ST em todo o mundo. Os FS, particularmente urgências premonitórias, são comumente relatados entre os indivíduos com ST com a partir da idade de 10 anos. Há uma sobreposição considerável com os FS descritos por indivíduos com Transtorno Obsessivo- Compulsivo (TOC). Os tiques costumam ter seu início na primeira década de vida e, então, seguem um curso flutuante com mudança do seu repertório. Conforme documentado no terceiro estudo, para a maioria dos pacientes, o período de pior gravidade dos tiques ocorre geralmente entre 7 e 15 anos de idade, após o qual a gravidade declina gradualmente. Esta queda dos sintomas de tiques é consistente com os dados epidemiológicos disponíveis que indicam uma prevalência muito menor de ST entre adultos do que crianças. Em resumo, há um esforço para incremento...

Tic disorders.

PURPOSE OF REVIEW: Primary tic disorders are complex, multifactorial disorders in which tics are accompanied by other sensory features and an array of comorbid behavioral disorders. Secondary tics are proportionally much less frequent, but their etiology is diverse. This review aims to guide clinicians in the recognition of the phenomenology, pathophysiology, and treatment of these disorders. RECENT FINDINGS: Advances include greater phenomenologic insights, particularly of nonmotor (sensory) features; increased knowledge of disease mechanisms, particularly coming from neuropsychological, functional imaging, pathologic, and animal model studies; growing evidence on the efficacy of alpha-2 agonists and the newer generation of dopamine-modulating agents; and recent strides in the evaluation of cognitive-behavioral therapy and deep brain stimulation surgery. SUMMARY: The correct diagnostic approach to tic disorders requires accurate historical gathering, a thorough neurologic examination, and detailed definition of the patient's psychopathologic profile. Treatment should always begin with individualized psychoeducational strategies. Although pharmacologic treatments remain beneficial for most patients, cognitive-behavioral treatments have thus far shown promising efficacy. Deep brain stimulation surgery should still be limited to adult patients refractory to pharmacotherapy and cognitive-behavioral therapy.

European clinical guidelines for Tourette syndrome and other tic disorders. Part IV: deep brain stimulation.

Ten years ago deep brain stimulation (DBS) has been introduced as an alternative and promising treatment option for patients suffering from severe Tourette syndrome (TS). It seemed timely to develop a European guideline on DBS by a working group of the European Society for the Study of Tourette Syndrome (ESSTS). For a narrative review a systematic literature search was conducted and expert opinions of the guidelines group contributed also to the suggestions. Of 63 patients reported so far in the literature 59 had a beneficial outcome following DBS with moderate to marked tic improvement. However, randomized controlled studies including a larger number of patients are still lacking. Although persistent serious adverse effects (AEs) have hardly been reported, surgery-related (e.g., bleeding, infection) as well as stimulation-related AEs (e.g., sedation, anxiety, altered mood, changes in sexual function) may occur. At present time, DBS in TS is still in its infancy. Due to both different legality and practical facilities in different European countries these guidelines, therefore, have to be understood as recommendations of experts. However, among the ESSTS working group on DBS in TS there is general agreement that, at present time, DBS should only be used in adult, treatment resistant, and severely affected patients. It is highly recommended to perform DBS in the context of controlled trials.

Tics y síndrome de Tourette: ¿son psicogénicos los tics? ¿Cuál es su fisiopatología y qué opciones terapéuticas existen? / Tics and Tourette syndrome: are tics of psychogenic origin? what is its pathophysiology and what are the therapeutic options

El Síndrome de Tourette es un trastorno frecuente en niños y adolescentes, y cada vez más reconocido en nuestro medio. Tiene una asociación establecida con otras comorbilidades como el síndrome de déficit atencional y el trastorno obsesivo compulsivo. En esta revisión se analizan nuevos avances en la comprensión de su fisiopatología y las nuevas alternativas terapéuticas disponibles.

Tic convulsivo doloroso: tratamiento por microcraniectomía / Painful tic convulsive: treatment by microcraniectomy

Se presentan cinco casos de espasmo hemifacial y neuralgia trigeminal, ipsilateral y concomitante conocido como tic convulsivo doloroso. Todos los pacientes fueron tratados por intervención quirúrgica de invasión mínima, la microcraniectomía asterional. Esta solucionó ambos problemas en el mismo procedimiento. La rareza de esta entidad justifica su plublicación

Los tics en la infancia / Tics in childhood

Los tics en la infancia constituyen una manifestación patológica muy frecuente. Son movimientos involuntarios, sin finalidad concreta y favorecidos pro factores emocionales. Existen tres grupos importantes que deben ser conocidos: tics transitorios, tics motores crónicos y tics múltiples o síndrome de Gilles de la Tourette. Los criterioe diagnósticos de los tics crónicos múltiples han variado con el transcurso de los años, hasta llegar a simplificarse por motivos de mayor conocimiento etiopatogénico. Es un síndrome con una incidencia relativamente frecuente. En este artículo se hace hincapié en el núcleo patológico obsesión-compulsivo, formas monosintomáticas y en la etiopatogenia orgánica del síndrome. Se citan las nuevas investigaciones con unas perspectivas de futuro apasionantes

Aspectos diagnósticos y terapéuticos de los tics / Diagnostic and therapeutic aspects of tics

El diagnóstico diferencial de los tics se plantea en las etapas primeras del tic transitorio del niño y de la Enfermedad de Gilles de la Tourette dada su similitud clínica al inicio. La evolución con la remisión habitual y en definitiva total de las primeras y la asociación de nuevos síntomas algunos de ellos específicos como la coprolalia en las segundas permite aclarar el diagnóstico. A pesar de la preocupación familiar el tic transitorio benigno del niño no debe ser tratado a menos que sea intenso. En la enfermedad de los tics cuando la disquinesia es de grado leve no debe medicarse; si la intensidad es moderada puede iniciarse tratamento con clonazepam pero en los grados mediano e intenso es imperativo emplear tratamiento específico para evitar la importante repercusión social tanto del tic como de la coprolalia, que ocasionarán trastornos emocionales importantes con depresión psíquica y aislamiento. Si bien el haloperidol ha sido empleado como primera opción dada su eficacia, consideramos que debido a sus frecuentes, variados e importantes efectos adversos, deben utilizarse inicialmente otros fármacos de similar potencia antidisquinética y de mejor tolerancia. Proponemos en primera instancia el uso del triapride, antidisquinético específico con escasa capacidad parkinsonígena y que no induce disquinesias; si su efecto terapéutico no es suficiente creemos conveniente la administración de pimozide o flufenazina que poseen similar eficacia a la del haloperidol con mejor tolerancia. Además del uso de...

[Pleiotropic tics and early disorders of ego development]. / Polyphäne Tics und frühe Störung der Ich-Entwicklung.

The long-term inpatient psychotherapeutic treatment of a 10-year-old girl with pleiotropic tics, aggressive behavior and a conduct disorder is described. The symptoms were present in the context of a compulsive-depressive personality development. The positive course of treatment is interpreted from a psychoanalytic perspective.

Behavioural psychotherapy of uncommon referrals.

In a behavioural clinic, over a period of nine years, trainee nurse-therapists treated 65 unusual referrals (8 per cent) out of a total of 800 patients. The remainder had phobic, obsessive-compulsive, sexual and social disorders, which responded encouragingly to behavioural treatment. Of the unusual referrals, useful results were obtained by behavioural treatment for stuttering, hairpulling, tics, and writer's cramp; bulimia is worth further study. Unresponsive conditions included compulsive gambling and obesity.