Motor and vocal tics are the core symptom of Tourette syndrome (TS). Tic generation seems to develop throughout the known motor pathways. This review focuses on functional neuroimaging in order to check this assumption. Also it elucidates the alterations and interactions of motor networks in TS depending on different contexts and circumstances like resting state, spontaneous tic movements, suppression of tics and premonitory urges, voluntary goal-oriented movements as well as electrophysiological neuronal stimulation. In general, the primary tic generating motor network uses the basic motor pathways differently, interacts with secondary sensorimotor networks and neuronal systems of cognitive behavioural control in a merely hierarchical manner, changing during neurodevelopment.

Aribert Rothenberger

Veit Roessner

Functional Neuroimaging Investigations of Motor Networks in Tourette Syndrome

The European Society for the Study of Tourette syndrome (ESSTS) was established in Denmark in 2000 by Mary Robertson and Anne Korsgaard. The aims of the organisation are to foster research activity and raise awareness of Tourette syndrome throughout Europe. The organisation went into abeyance in 2002 but was resurrected in 2007 in Bari, Italy. Since that time ESSTS has grown and prospered. We have established elected officers and a constitution. We have successfully applied for three large scale European research grants and have members throughout the European Union. We have held yearly meetings across Europe including two training schools and we have developed successful alliances with patient support groups. ESSTS has developed and published the first European guidelines on assessment, diagnosis and treatment of Tourette syndrome.

Hugh Rickards

Peristera Paschou

Renata Rizzo

Jeremy S. Stern

A Brief History of the European Society for the Study of Tourette Syndrome

Tourette syndrome (TS) is a neurological condition first described by Georges Gilles de la Tourette in 1885. TS was largely thought of as a rare and bizarre condition until the 1960s, when the beneficial effects of neuroleptics on tic symptoms led to an exponential increase in neuroscientific research. Today TS is known to be a relatively common condition that is frequently misdiagnosed due to a combination of its variable manifestation and the waxing and waning of tic frequency and severity. Although there has been a paucity of research on TS compared to other movement disorders, in recent years TS has garnered increasing interest and has shown a number of novel and complex sides, about which much is yet to be learnt. The present article discusses where research has taken us thus far and where it is heading in all the major facets of this fascinating condition.

Andrea E. Cavanna

Conor Kavanagh

Mary M. Robertson

The Future of Research in Tourette Syndrome

Background: Tourette syndrome (TS) is a neuropsychiatric disorder with childhood onset characterized by chronic motor and vocal tics. The typical clinical course of an attenuation of symptoms during adolescence in parallel with the emerging self-regulatory control during development suggests that plastic processes may play an important role in the development of tic symptoms.Methods: We conducted a systematic search to identify existing imaging studies (both anatomical and functional magnetic resonance imaging [fMRI]) in young persons under the age of 19 years with TS.Results: The final search resulted in 13 original studies, which were reviewed with a focus on findings suggesting adaptive processes (using fMRI) and plasticity (using anatomical MRI). Differences in brain activation compared to healthy controls during tasks that require overriding of prepotent responses help to understand compensatory pathways in children with TS. Along with alterations in regions putatively representing the origin of tics, deviations in several other regions most likely represent an activity-dependent neural plasticity that help to modulate tic severity, such as the prefrontal cortex, but also in the corpus callosum and the limbic system.Discussion: Factors that potentially influence the development of adaptive changes in the brain of children with TS are age, comorbidity with other developmental disorders, medication use, IQ along with study-design or MRI techniques for acquisition, and analysis of data. The most prominent limitation of all studies is their cross-sectional design. Longitudinal studies extending to younger age groups and to children at risk for developing TS hopefully will confirm findings of neural plasticity in future investigations.

Heike Eichele

Kerstin J. Plessen

Neural Plasticity in Functional and Anatomical MRI Studies of Children with Tourette Syndrome

Andrea Eugenio Cavanna

Researching Tourette Syndrome in Europe

Tourette syndrome (TS) is often accompanied by other symptoms and syndromes. The two best-known co-morbidities are Attention Deficit Hyperactivity Disorder (ADHD) and Obsessive Compulsive Disorder (OCD), but also other conditions like rage-attacks, depression, and sleeping disturbances are frequent in persons with TS. Both in clinical cohorts and in population-based cohorts the prevalence of co-morbidities is high. The presence of co-morbid ADHD and/or OCD has an impact on psychosocial, educational, and neuropsychological consequences of TS and it is associated with higher rates of other co-morbid disorders, like rage, anxiety, and conduct disorders. The symptoms of a co-morbid disorder might appear prior to the time that tics reach clinical attention. The TS phenotype probably changes during the course of the disease. The exact aetiology of the co-occurrence of co-morbid disorders and TS is not known, but they probably all are neurotransmitter disorders. European guidelines recommend first-choice pharmacological treatment, but randomised double-blinded trials are needed. Professionals need to be aware of the close relationship between TS and co-morbidities in order to give the patients the right treatment and support.

Nanette M. M. Mol Debes

Co-Morbid Disorders in Tourette Syndrome

Gilles de la Tourette syndrome (GTS) is a neurodevelopmental condition characterised by multiple motor and phonic tics and associated behavioural problems, carrying a significant burden on patients' lives. Although the term health related-quality of life (HR-QOL) has only been used in recent years, several studies have long addressed the impact of GTS on physical, psychological and social aspects of wellbeing of both children and adults with GTS. We set out to answer the question &#x22;Is HR-QOL affected by GTS and, if so, in what domains?&#x22; by conducting a systematic literature review of published original studies addressing HR-QOL in both children and adult patients with GTS. This review focuses on the current evidence on the impact of GTS on patients' lives, mainly informed by studies using generic functional impairment and HR-QOL measures from the last decade, and expands on the new opportunities introduced by the recently developed GTS-specific HR-QOL scales (GTS-QOL and GTS-QOL-C&#x26;A). Analysis of the first decade of studies specifically addressing HR-QOL in GTS suggests that co-morbid conditions are key factors in determining HR-QOL in young patients, whilst the picture is more complex in adults with GTS. These findings offer some general directions for both current clinical practice and future research.

Kate David

Valentina Bandera

Cristiano Termine

Umberto Balottin

Anette Schrag

Caroline Selai

Health-Related Quality of Life in Gilles de la Tourette Syndrome: A Decade of Research

Most patients with Tourette syndrome report characteristic sensory experiences (premonitory urges) associated with the expression of tic symptoms. Despite the central role of these experiences to the clinical phenomenology of Tourette syndrome, little is known about their underlying brain processes. In the present article we present the results of a systematic literature review of the published studies addressing the pathophysiological mechanisms of premonitory urges. We identified some preliminary evidence for specific alterations in sensorimotor processing at both cortical and subcortical levels. A better insight into the brain correlates of premonitory urges could lead to the identification of new targets to treat the sensory initiators of tics in patients with Tourette syndrome.

Sangeerthana Rajagopal

Stefano Seri

Premonitory Urges and Sensorimotor Processing in Tourette Syndrome

Background: Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients&#x2019; health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410&#x2013;1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS.Methods: A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&#x26;A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6&#x2013;12 year-olds and a self-report questionnaire for 13&#x2013;18 year-olds.Results: The C&#x26;A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach&#x2019;s alpha &#x3e; 0.7) and validity was supported by interscale correlations (range 0.4&#x2013;0.7), principal-component factor analysis and correlations with other rating scales and clinical variables.Conclusions: The present version of the C&#x26;A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity.

Chiara Luoni

Claudia Selvini

Rosanna Blangiardo

Clare M. Eddy

Paola R. Silvestri

Paola V. Cal&#xec;

Francesco Cardona

The Gilles De La Tourette Syndrome-Quality of Life Scale for Children and Adolescents (C&#x26;A-GTS-QOL): Development and Validation of the Italian Version

Cannabinoids have been used for hundred of years for medical purposes. To day, the cannabinoid delta-9-tetrahydrocannabinol (THC) and the cannabis extract nabiximols are approved for the treatment of nausea, anorexia and spasticity, respectively. In Tourette syndrome (TS) several anecdotal reports provided evidence that marijuana might be effective not only in the suppression of tics, but also in the treatment of associated behavioural problems. At the present time there are only two controlled trials available investigating the effect of THC in the treatment of TS. Using both self and examiner rating scales, in both studies a significant tic reduction could be observed after treatment with THC compared to placebo, without causing significant adverse effects. Available data about the effect of THC on obsessive-compulsive symptoms are inconsistent. According to a recent Cochrane review on the efficacy of cannabinoids in TS, definite conclusions cannot be drawn, because longer trials including a larger number of patients are missing. Notwithstanding this appraisal, by many experts THC is recommended for the treatment of TS in adult patients, when first line treatments failed to improve the tics. In treatment resistant adult patients, therefore, treatment with THC should be taken into consideration.

Kirsten R. M&#xfc;ller-Vahl

Treatment of Tourette Syndrome with Cannabinoids

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Behavioural Neurology

Researching Tourette Syndrome in Europe

Researching Tourette Syndrome in Europe

Articles

Functional Neuroimaging Investigations of Motor Networks in Tourette Syndrome

A Brief History of the European Society for the Study of Tourette Syndrome

The Future of Research in Tourette Syndrome

Neural Plasticity in Functional and Anatomical MRI Studies of Children with Tourette Syndrome

Researching Tourette Syndrome in Europe

Co-Morbid Disorders in Tourette Syndrome

Health-Related Quality of Life in Gilles de la Tourette Syndrome: A Decade of Research

Premonitory Urges and Sensorimotor Processing in Tourette Syndrome

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

Treatment of Tourette Syndrome with Cannabinoids