Gilles de la Tourette syndrome: the complexities of phenotype and treatment

University College London, London, UK.
British journal of hospital medicine (London, England: 2005) (Impact Factor: 0.38). 02/2011; 72(2):100-7. DOI: 10.12968/hmed.2011.72.2.100
Source: PubMed


Tourette syndrome is a chronic motor and vocal tic disorder, which is common (1%). The aetiology is complex (mostly genetic) and 90% of people have co-morbid psychiatric disorders and reduced quality of life. Management includes reassurance, education, behavioural treatments and medications for tics and psychopathology.

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    • "TS does not affect intellectual ability per se but has a high prevalence rate with comorbid learning disabilities[3]. Furthermore, around 90 % of the TS population present with comorbid neuropsychiatric conditions or associated symptoms[4]. Associated conditions frequently co-occurring with TS include Attention Deficit Hyperactivity Disorder (ADHD) and Obsessive Compulsive Disorder (OCD). "
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    ABSTRACT: Background Tourette syndrome (TS) is a neurodevelopmental disorder characterised by motor and vocal tics. These involuntary movements and vocalizations can have a negative impact in the school environment. The paper presents a mixed methods description of the difficulties experienced by UK students with TS in secondary school, drawing on multiple perspectives. Methods Thirty-five young people with TS (11 to 18 years), their parents (n = 35) and key members of school staff (n = 54) took part in semi-structured interviews about TS-related difficulties in secondary school. Theme analysis was used to identify school difficulties reported by the young people, before moving on to analysis of the parents’ and staff members’ transcripts. The most frequently occurring themes from the young people’s accounts were then quantified in order to examine the level of agreement between informants and the association with clinical symptom severity. Results A range of TS-related difficulties with academic work, and social and emotional well-being in school were reported by young people, parents and staff. Three superordinate themes are described: 1) TS makes school work more difficult, 2) Negative response to TS from staff and fellow students and 3) TS makes it more difficult to manage emotions in school. The three difficulties most frequently reported by the young people were problems concentrating in class, unhelpful responses by school staff to tics and difficulties with other students such as name-calling and mimicking tics. Additional difficulties reported by more than a quarter of young people related to homework, examinations, writing, anxiety and managing anger in school. Having more severe motor tics was associated with reporting difficulties with homework and handwriting, whereas having more severe phonic tics was associated with reporting unhelpful responses from staff. Young people and parents agreed more strongly with each other than they did with staff regarding school difficulties faced by individuals, and staff generally reported fewer TS-related difficulties. Conclusions TS can present a barrier to learning in several ways and can also affect interactions with others and emotional experiences in secondary school. Implications for supporting secondary school-aged students with TS are considered.
    Full-text · Article · Jan 2016 · BMC Psychiatry
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    • "For instance, symptom severity correlated negatively with the degree of activation of CSTC circuits during tic suppression (Peterson et al., 1998) and prefrontal cortical thickness (Draganski et al., 2010) and volume of prefrontal CSTC areas was decreased in TS patients compared with healthy controls (Draganski et al., 2010). More than 90% of all patients with TS also have co-morbid psychiatric disorders, most often OCD or ADHD (Robertson, 2011). "
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    ABSTRACT: Over the past 20 years, motor response inhibition and interference control have received considerable scientific effort and attention, due to their important role in behavior and the development of neuropsychiatric disorders. Results of neuroimaging studies indicate that motor response inhibition and interference control are dependent on cortical-striatal-thalamic-cortical (CSTC) circuits. Structural and functional abnormalities within the CSTC circuits have been reported for many neuropsychiatric disorders, including obsessive-compulsive disorder (OCD) and related disorders, such as attention-deficit hyperactivity disorder, Tourette's syndrome, and trichotillomania. These disorders also share impairments in motor response inhibition and interference control, which may underlie some of their behavioral and cognitive symptoms. Results of task-related neuroimaging studies on inhibitory functions in these disorders show that impaired task performance is related to altered recruitment of the CSTC circuits. Previous research has shown that inhibitory performance is dependent upon dopamine, noradrenaline, and serotonin signaling, neurotransmitters that have been implicated in the pathophysiology of these disorders. In this narrative review, we discuss the common and disorder-specific pathophysiological mechanisms of inhibition-related dysfunction in OCD and related disorders.
    Full-text · Article · Jun 2014 · Frontiers in Human Neuroscience
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    • "The population investigated was very homogeneous though, making it more likely that the results can be generalized to other uncomplicated GTS patients despite the sample size. However, approximately 90% of GTS patients suffer from comorbidities [66], hence the findings reported in this study might not be valid for the whole population of GTS patients. Further limitations of this study include the possibility that past and present intake of medication may have influenced the results. "
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    ABSTRACT: Gilles de la Tourette syndrome is a neuropsychiatric disorder characterized by motor and phonic tics that can be considered motor responses to preceding inner urges. It has been shown that Tourette patients have inferior performance in some motor learning tasks and reduced synaptic plasticity induced by transcranial magnetic stimulation. However, it has not been investigated whether altered synaptic plasticity is directly linked to impaired motor skill acquisition in Tourette patients. In this study, cortical plasticity was assessed by measuring motor-evoked potentials before and after paired associative stimulation in 14 Tourette patients (13 male; age 18-39) and 15 healthy controls (12 male; age 18-33). Tic and urge severity were assessed using the Yale Global Tic Severity Scale and the Premonitory Urges for Tics Scale. Motor learning was assessed 45 minutes after inducing synaptic plasticity and 9 months later, using the rotary pursuit task. On average, long-term potentiation-like effects in response to the paired associative stimulation were present in healthy controls but not in patients. In Tourette patients, long-term potentiation-like effects were associated with more and long-term depression-like effects with less severe urges and tics. While motor learning did not differ between patients and healthy controls 45 minutes after inducing synaptic plasticity, the learning curve of the healthy controls started at a significantly higher level than the Tourette patients' 9 months later. Induced synaptic plasticity correlated positively with motor skills in healthy controls 9 months later. The present study confirms previously found long-term improvement in motor performance after paired associative stimulation in healthy controls but not in Tourette patients. Tourette patients did not show long-term potentiation in response to PAS and also showed reduced levels of motor skill consolidation after 9 months compared to healthy controls. Moreover, synaptic plasticity appears to be related to symptom severity.
    Full-text · Article · May 2014 · PLoS ONE
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