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Gilles de la Tourette syndrome is a chronic neuropsychiatric disorder that can have a detrimental impact on the health-related quality of life of children with the condition. To date no patient-reported health-related quality of life measures have been developed for children and adolescents in the English language. This study validated the first disease-specific scale for the quantitative assessment of health-related quality of life in 118 children and adolescents with Gilles de la Tourette syndrome (C&A-GTS-QOL) following language adaptation from Italian to English in the United Kingdom. Standard statistical methods were used to test the psychometric properties of the rating scale. Principal component factor analyses led to the identification of six health-related quality of life domains (cognitive, copro-phenomena, psychological, physical, obsessive-compulsive, and activities of daily living), explaining 66.7% of the overall variance. The C&A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability; validity was supported by interscale correlations (range 0.2-0.7), confirmatory factor analysis, and correlation patterns with other rating scales and clinical variables.

Gilles de la Tourette's syndrome is an uncommon illness associated with repetitive un-voluntary abnormal movements and utterance. It is often associated with other psychiatric morbidities. Management requires awareness of this uncommon illness, keen observation, relevant evaluation, and combination of pharmacology and psychotherapy for an optimal outcome. This case is brought out here for florid presentation and nuances of management.

Revisamos a história da síndrome de Tourette, com ênfase a contribuição de Jean-Martin Charcot. We review the history of Tourette syndrome, emphasizing the contribution of Jean-Martin Charcot.

Most people perceive that their actions arise from their own ‘free will’, commonly defined as the ability to choose how to act.1 Neurological disorders, such as epileptic seizures and movement disorders, are often regarded as conditions in which free will is undermined.2 Clinically, an action is considered involuntary when it is automatically performed and cannot be controlled. Our study aim was to explore patients’ views as well as clinicians’ views on ‘free will’ and voluntariness in three hyperkinetic disorders: tics, functional movement disorders (FMD, previously ‘psychogenic’ movement disorder) and myoclonus. We developed a questionnaire to determine to what extent patients in these three groups consider their ‘free will’ to be undermined by the movements induced by their disorder. We compared these findings with clinicians’ views of voluntariness in each of these movement disorders. The current study was part of a larger study on FMD, myoclonus and tics, encompassing a clinical and Bereitschaftspotential study (approved by the local ethics committee).3 4 Thirty-nine expert clinicians participated in a diagnostic study of 60 patients with FMD (n=28), myoclonus (n=15) or tics (n=17). Furthermore, 22 healthy control subjects were included. Clinicians were provided with a 4-item questionnaire. Clinicians were asked to rate the degree of voluntariness on a 100 mm Visual Analogue Scale (VAS) ranging from 0 (completely involuntary) to 100 (completely voluntary) for different movements: (1) raising one’s hand to vote, (2) myoclonus, (3) …

Professor Jean-Martin Charcot is considered the most important professor of Neurology and also the head of the Salpêtrière School of Neurology. In a famous picture painted by André Brouillet and presented at the Salon of 1887, under the title "A clinical lesson at the Salpêtrière", Professor Charcot presents a case of hysteria to a large audience of physicians and renowned intellectuals. Copies of this guided picture are also available for sale at the shop of the Museum of the School of Medicine of Paris and are frequently used in lectures by neurologists worldwide. However, in these reproductions, Gilles de la Tourette's and Charles Féré's positions are inverted. This historical note sheds some light on this little mistake in some of the reproductions of Brouillet's famous painting, so that further confusion can be avoided.

Gilles de la Tourette syndrome (TS) is characterized by motor and vocal tic manifestations, often accompanied by behavioral, cognitive and affective dysfunctions. Electroencephalography of patients with TS has revealed reduced Sensorimotor Rhythm (SMR) and excessive fronto-central Theta activity, that presumably underlie motor and cognitive disturbances in TS. Some evidence exists that neurofeedback (NFB) training aimed at enhancing SMR amplitude is effective for reducing tics. The present report is an uncontrolled single case study where a NFB training protocol, involving combined SMR uptraining/Theta downtraining was delivered to a 17-year-old male with TS. After sixteen SMR-Theta sessions, six additional sessions were administered with SMR uptraining alone. SMR increase was better obtained when SMR uptraining was administered alone, whereas Theta decrease was observed after both trainings. The patient showed a reduction of tics and affective symptoms, and improvement of cognitive performance after both trainings. Overall, these findings suggest that Theta decrease might account for some clinical effects seen in conjunction with SMR uptraining. Future studies should clarify the feasibility of NFB protocols for patients with TS beyond SMR uptraining alone.

International audience; ABSTRACT Objective: Thalamic deep brain stimulation for intractable Tourette Syndrome has been introduced in 1999 by Vandewalle et al. In this follow-up study we report on the long-term (6 and 10 years) outcome in terms of tic reduction, cognition and mood and side-effects of medial thalamic DBS in two previously described Tourette patients. Methods: We compared the outcome of two patients at 6 and 10 years after surgery with their preoperative status and after 8 months and 5 years of treatment, respectively. Standardized video recordings were scored by three independent investigators. Both patients underwent (neuro)psychological assessment at all time points of follow-up. Results: Tic improvement observed at 5 years in patient 1 (90.1%) was maintained at 10 years (92.6%). In patient 2 the tic improvement at 8 months (82%) was slightly decreased at 6 years (78%). During follow-up, case 1 revealed no changes in cognition, but case 2 showed a decrease in verbal fluency and learning which was in line with his subjective reports. Case 2 showed a slight decrease in depression but overall psychopathology was still high at 6 years after surgery with an increase in anger and aggression together with difficulties in social adaptation. Besides temporary hardware related complications no distressing adverse effects were observed. Conclusion: Bilateral thalamic stimulation may provide sustained tic benefit after at least 6 years but overall improvement is not obvious. To maximize overall outcome attention is needed for postoperative psychosocial adaptation, already prior to surgery.