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Open AccessJournal ArticleDOI

Tourette Syndrome: Update

Mark Hallett
- 01 Aug 2015 - 
- Vol. 37, Iss: 7, pp 651-655
TLDR
There is a loss of inhibition in these patients and recent studies show abnormalities in brain GABA and dopamine function and dopamine blocking agents are effective therapy, and in severe drug-refractory patients, deep brain stimulation can be effective.
Abstract
Tourette Syndrome is a disorder characterized by tics. It typically begins in childhood and often improves in adult life. Tics are best described as voluntary movements made automatically so that volition is not ordinarily appreciated. There is frequently an urge, sometimes in the form of a specific sensory feeling (sensory tic), that precedes the tic. Patients say that they make the tic in order to reduce the urge, although shortly after the tic, the urge recurs. The sensory feeling may arise due to defective sensory habituation. Since tics relieve the urge, this can be considered rewarding, and repetition of this behavior may perpetuate the tic as a habit. Tourette Syndrome affects boys more than girls and is associated with attention deficit hyperactivity disorder and obsessive compulsive disorder. Although Tourette Syndrome often appears to be autosomal recessive in inheritance, it has been difficult to find any abnormal genes. There is a loss of inhibition in these patients and recent studies show abnormalities in brain GABA. Certainly there is also an abnormality in dopamine function and dopamine blocking agents are effective therapy. In severe drug-refractory patients, deep brain stimulation can be effective.

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The GABAA Receptor as a Therapeutic Target for Neurodevelopmental Disorders

TL;DR: It is argued that the GABAergic system is disturbed in many neurodevelopmental disorders, including fragile X syndrome, Rett Syndrome, and Dravet syndrome, and is a key candidate target for therapeutic intervention.
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Enhanced habit formation in Gilles de la Tourette syndrome

TL;DR: Enhanced habit formation in unmedicated patients with Gilles de la Tourette syndrome is indicated, indicating Aberrant reinforcement signals to the sensorimotor striatum may be fundamental for the formation of stimulus-response associations and may contribute to the habitual behaviour and tics of this syndrome.
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Neurobiology of the Premonitory Urge in Tourette’s Syndrome: Pathophysiology and Treatment Implications

TL;DR: This article reviews the existing literature on the clinical and neurobiological aspects of the premonitory urge in patients with TS, with focus on its pathophysiology and possible treatment implications.
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TL;DR: It is vital to transfer the expanding knowledge of the movement disorders into the development of novel symptomatic or disease-modifying treatments, and to assess these therapeutic strategies in appropriately designed and well done trials.
References
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Journal ArticleDOI

Habits, Rituals, and the Evaluative Brain

TL;DR: This review suggests that many of these behaviors could emerge as a result of experience-dependent plasticity in basal ganglia-based circuits that can influence not only overt behaviors but also cognitive activity.
Journal ArticleDOI

What is the Bereitschaftspotential

TL;DR: BP is used for detecting the participation of the 'voluntary motor system' in the generation of apparently involuntary movements in patients with psychogenic movement disorders and event-related desynchronization of beta frequency EEG band before self-initiated movements shows a different temporospatial pattern from that of the BP, suggesting different neuronal mechanisms for the two.
Journal ArticleDOI

Altered parvalbumin-positive neuron distribution in basal ganglia of individuals with Tourette syndrome.

TL;DR: The imbalance in striatal and GPi inhibitory neuron distribution suggests that the functional dynamics of cortico-striato-thalamic circuitry are fundamentally altered in severe, persistent TS.
Journal ArticleDOI

Sensorimotor gating in boys with Tourette's syndrome and ADHD: Preliminary results

TL;DR: These findings, together with other converging lines of evidence, suggest that deficient pallidal inhibition may be etiologically related to tic and movement disorders.
Journal ArticleDOI

Neural correlates of tic generation in Tourette syndrome: an event-related functional MRI study.

TL;DR: The results of this study indicate that paralimbic and sensory association areas are critically implicated in tic generation, similar to movements triggered internally by unpleasant sensations, as has been shown for pain or itching.
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