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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    • "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.
    Frontiers in Human Neuroscience 06/2014; 8:419. DOI:10.3389/fnhum.2014.00419 · 3.63 Impact Factor
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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.
    PLoS ONE 05/2014; 9(5):e98417. DOI:10.1371/journal.pone.0098417 · 3.23 Impact Factor
    • "of TS and absence of comorbid disorders was confirmed in writing by the participants' general practitioner, pediatrician, or psychiatrist. Guided by the evidence that TS symptoms peak early in the second decade of life (Leckman, 2002; Robertson, 2011), age exclusion criteria included a lower cutoff of 10 years and a higher cutoff of 20 years (oldest participant included in the study was 20.1 years; see Table 1) to include any late-onset cases with TS. Other exclusion criteria included the following: a clinical diagnosis of a comorbid psychiatric or mental disorder (e.g., ADHD or OCD), serious physical illness or handicaps, major neurological disease, and presence of severe learning disabilities. "
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    ABSTRACT: In light of descriptive accounts of attenuating effects of physical activity on tics, we used an experimental design to assess the impact of an acute bout of aerobic exercise on tic expression in young people (N = 18) with Tourette Syndrome (TS). We compared video-based tic frequency estimates obtained during an exercise session with tic rates obtained during pre-exercise (baseline) and post-exercise interview-based sessions. Results showed significantly reduced tic rates during the exercise session compared with baseline, suggesting that acute exercise has an attenuating effect on tics. Tic rates also remained reduced relative to baseline during the post-exercise session, likely reflecting a sustained effect of exercise on tic reduction. Parallel to the observed tic attenuation, exercise also had a beneficial impact on self-reported anxiety and mood levels. The present findings provide novel empirical evidence for the beneficial effect of exercise on TS symptomatology bearing important research and clinical implications.
    Behavior modification 04/2014; 38(2). DOI:10.1177/0145445514532127 · 2.23 Impact Factor
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