Abstract
Tourette's syndrome (TS) is a neurodevelopmental disorder characterized by fluctuating motor and vocal tics, usually preceded by sensory premonitions, called premonitory urges. Besides tics, the vast majority—up to 90%—of TS patients suffer from psychiatric comorbidities, mainly attention deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). The etiology of TS remains elusive. Genetics is believed to play an important role, but it is clear that other factors contribute to TS, possibly altering brain functioning and architecture during a sensitive phase of neural development. Clinical brain imaging and genetic studies have contributed to elucidate TS pathophysiology and disease mechanisms; however, TS disease etiology still is poorly understood. Findings from genetic studies led to the development of genetic animal models, but they poorly reflect the pathophysiology of TS. Addressing the role of neurotransmission, brain regions, and brain circuits in TS disease pathomechanisms is another focus area for preclinical TS model development. We are now in an interesting moment in time when numerous innovative animal models are continuously brought to the attention of the public. Due to the diverse and largely unknown etiology of TS, there is no single preclinical model featuring all different aspects of TS symptomatology. TS has been dissected into its key symptomst hat have been investigated separately, in line with the Research Domain Criteria concept. The different rationales used to develop the respective animal models are critically reviewed, to discuss the potential of the contribution of animal models to elucidate TS disease mechanisms.
Highlights
Tourette’s syndrome (TS) Definition, Epidemiology, Symptoms, and Natural CourseTourette’s Syndrome (TS) was named after Georges Gilles de la Tourette (1857–1904) who first described it as a “tic syndrome” in 1885 and whose observations are still considered mostly valid today
Movements occur as the motor cortex is activated by the thalamus, which is controlled by the subthalamic nucleus (STN)-Globus pallidus externus (GPe)-globus pallidus internus (GPi) microcircuit
Tics are supposed to be caused by a deregulated activity of the basal ganglia, which consequentially leads to disinhibition of the thalamus and a hyperexcitability of the motor cortex (Albin and Mink, 2006; Wang et al, 2011)
Summary
Tourette’s Syndrome (TS) was named after Georges Gilles de la Tourette (1857–1904) who first described it as a “tic syndrome” in 1885 and whose observations are still considered mostly valid today. Animal Models of Tourette Syndrome multiple motor and one or more vocal tics with an onset before age 18 years and a persistence for at least 1 year (DSM-5). Movements occur as the motor cortex is activated by the thalamus, which is controlled by the STN-GPe-GPi microcircuit. When the pre-motor cortex activates the putamen, the inhibitory striatal projection neurons release the thalamus from inhibition held by the STN-GPe-GPi, and eventually the motor cortex can be activated, leading to movement (Obeso and Lanciego, 2011). Tics are supposed to be caused by a deregulated activity of the basal ganglia, which consequentially leads to disinhibition of the thalamus and a hyperexcitability of the motor cortex (Albin and Mink, 2006; Wang et al, 2011). The use of animal models could help the major means of investigations of TS thanks to their ability to verify pathophysiological hypotheses and test pharmacological compounds
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