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.

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.

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.

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.

A 9-year-old girl under pediatric follow-up since the age of 4 years after diagnosis of neurofibromatosis type 1

To present a case of neurofibromatosis and ADHD comorbidity to raise awareness of the importance of screening for neurodevelopmental disorders.

Case report and literature review

The patient had an adequate control and follow-up of the disorder with periodic check-ups and magnetic resonance imaging during her follow-up. She was referred due to symptoms of inattention with failure to perform exams and impulsivity in interpersonal relationships, affecting her social functioning. In addition, the patient presented simple motor tics of eye contraction and shoulder elevation. The patient was diagnosed with attention deficit hyperactivity disorder together with tic disorder. She was treated with stimulant medication with worsening of tics and marked hyporexia. Therefore, medication with guanfacine was started up to 4 mg per day, adjusted by weight. With this dose there was a control of the tics, with improvement of the symptoms of inattention and impulsivity. In different spheres an improvement in their functionality was observed, with improvement in mood, self-esteem and academic performance.

Neurofibromatosis type 1 is a rare monogenic disorder with a varied presentation (ophthalmologic, dermatologic and predisposition to tumor development). Patientshave been shown to present with symptoms of inattention and executive function impairment, along with other neurodevelopmental disorders such as autism spectrum disorders, learning disabilities or intellectual disability. The literature shows that up to 60% of them has ADHD criteria.

No significant relationships.

To better understand current practices of U.S.-based physicians in the management of Tourette syndrome (TS) and identify gaps that may be addressed by future education.

Two survey instruments were developed to gather data on management of TS and perceptions from physicians and caregivers of children with TS. The clinician survey was developed in consultation with a TS physician expert and utilized clinical vignettes to assess and quantify practice patterns. The caregiver survey was adapted from the clinician survey and other published studies and gathered details on diagnosis, treatment, and perceptions regarding management.

Data included responses from 138 neurologists (including 57 pediatric neurologists), 162 psychiatrists (including 42 pediatric psychiatrists), and 67 caregivers. Most (65%) pediatric neurologists rely solely on clinical findings to make a diagnosis, whereas the majority of other specialists utilize additional testing (eg, neuroimaging, lab testing, and genetics). Most psychiatrists (96%) utilize standardized criteria to make a diagnosis, whereas 22% of neurologists do not. Many physicians (44% of psychiatrists and 20% of neurologists) use pharmacotherapy to treat a patient with “slightly bothersome” tics and no functional impairment, whereas caregivers favored behavioral therapy. Most (76%) caregivers preferred to make the final treatment decision, whereas 80% of physicians preferred equal or physician-directed decision-making.

This study provides insight into practice patterns and perceptions of U.S.-based neurologists and psychiatrists in managing TS. Results highlight the potential value of physician education, including diagnostic approach, tic management and monitoring, involvement of caregivers in decision-making, and updates on TS management.

Tourette syndrome (TS) is a neurodevelopmental disorder characterized by the presence of motor and phonic tics. It is at least three times more common in males compared with females; however, the clinical phenomenology between sexes has not been fully examined. We aimed to contrast the clinical features between males and females with TS and chronic tic disorder.

We studied 201 consecutive patients fulfilling the diagnostic criteria for TS, persistent (or chronic) motor and vocal tic disorder and provisional tic disorder that were considered within the TS spectrum disorder. We performed blinded evaluations of video-recordings and retrospectively reviewed the clinical charts of all patients.

Age ranges between 4 and 65 years. Males represented 77.6% of patients in the cohort. Overall, no differences were observed in the frequency, distribution and complexity of tics between sexes, except for a higher frequency of attention-deficit/hyperactivity disorder (ADHD) (P = .003) among males. Patients younger than 18-years old, in addition to a higher frequency of ADHD (P = .026), males had a statistically higher frequency of complex motor tics (P = .049) and earlier age at onset (P = .072) than females in the multivariate regression analysis. However, these differences were lost in patients older than 18 years, due to increased complexity of tics in females with aging.

A sexual dimorphism was observed between patients with TS mainly before age of 18 years, suggesting an earlier onset of some types of tics and ADHD in males compared to females.

Tourette’s syndrome is a neuropsychiatric disorder marked by motor and phonic tics frequently associated with psychiatric comorbidities, beginning in childhood. While most cases improve or resolve with age, some are refractory.

To review new strategies for the management of Tourette’s Syndrome, following an outpatient clinical vignette.

We performed a review based on the PubMed® database.

A 50-years-old female patient with a long-term outpatient psychiatric follow-up presented with motor tics appearing in adolescence, including winking and facial grimacing, as well as episodes of coprolalia. Over the years, she developed an anxiety disorder and social isolation. In addition to psychological therapy, pharmacological therapy had already been approached with the use alpha-adrenergic agonists and several antipsychotics, such as risperidone and aripiprazole, with the patient showing only partial response to pimozide. In Tourette’s syndrome, the therapy must be adequate to the patient’s individual needs. Emerging treatments for refractory cases, such as anticonvulsants, cannabinoids or antiglutamatergic drugs have been the target of several studies. Botulinum toxin injections are particularly effective in patients with focal motor tics and complex phonic tics. Non-pharmacological treatment options, such as electroconvulsive therapy and deep brain stimulation may prove effective in some cases.

A significant proportion of patients fail to respond to conventional strategies. Thus, new pharmacological and non-pharmacological therapies are on the horizon and may represent an important step in treatment algorithms for refractory cases in the future.

A 15-year-old man who comes to the consultation referred from neurology for a poorly progressive tic disorder that is resistant to treatment. This is a patient with no relevant medical history. He has good academic performance, without symptoms of inattention, hyperactivity or impulsivity. He does not present buccophonatory tics

To Show guanfacine as a efficient treatment fot tics disorder

Case report

He has presented complex motor tics as cervical contractions that have had to receive physiotherapeutic treatment. He also presents simple ocular tics. The patient at our evaluation is on a 4 mg dose of pimozide without response. He was previously on risperidone. It was decided to start treatment with guanfacine up to 5 mg with reduction of pimozide, to 1 mg. The patient with this dose adjustment shows a notable improvement in the frequency and intensity of the tics, both cervical and ocular. The patient also refers to a feeling of being calmer and being able to face stressful situations such as being exposed to social relationships, intervening more in class without increasing their anxiety levels.

Guanfacine is a selective alpha-2a adrenergic receptor agonist that has an indication for attention deficit hyperactivity disorder. Like its precursor, clonidine, there is more and more literature that proposes this medication and others for ADHD as useful drugs in pathologies such as tic disorder. It has a safe use profile, which with progressive adjustment and hardly any side effects is placed as a treatment to take into account in this pathology.

No significant relationships.

Previous studies suggest that adults with Tourette syndrome (TS) can respond unconventionally on tasks involving social cognition. We therefore hypothesized that these patients would exhibit different neural responses to healthy controls in response to emotionally salient expressions of human eyes.

Twenty-five adults with TS and 25 matched healthy controls were scanned using fMRI during the standard version of the Reading the Mind in the Eyes Task which requires mental state judgements, and a novel comparison version requiring judgements about age.

During prompted mental state recognition, greater activity was apparent in TS within left orbitofrontal cortex, posterior cingulate, right amygdala and right temporo-parietal junction (TPJ), while reduced activity was apparent in regions including left inferior parietal cortex. Age judgement elicited greater activity in TS within precuneus, medial prefrontal and temporal regions involved in mentalizing. The interaction between group and task revealed differential activity in areas including right inferior frontal gyrus. Task-related activity in the TPJ covaried with global ratings of the urge to tic.

While recognizing mental states, adults with TS exhibit greater activity than controls in brain areas involved in the processing of negative emotion, in addition to reduced activity in regions associated with the attribution of agency. In addition, increased recruitment of areas involved in mental state reasoning is apparent in these patients when mentalizing is not a task requirement. Our findings highlight differential neural reactivity in response to emotive social cues in TS, which may interact with tic expression.