Gilles de la Tourette syndrome:: Clinical spectrum and management

Objective. To present the current concepts of the clinical phenomenology and natural history of Gilles de la Tourette syndrome (GTS), differential diagnosis with other involuntary movements, its pathogenesis and current management: Development. GTS is a disorder characterized by a spectrum of both motor and sonic tics, and a spectrum of behavioral disorders. There is not a biological marker that confirms or refutes the diagnosis of GTS, so this diagnosis remains purely clinical. It has been found to be present in 1 to 3% of school population. An specific cause for GTS is unknown, though most cases appear to occur on an hereditary polygenetic basis. Observations that drugs increasing dopamine neurotransmission, including levodopa and the dopamine receptor agonist pergolide lessen tics, have called into question the dopamine receptor supersensibility hypothesis. The hypothesis proposing basal ganglia and frontostriatal pathways involvement in the pathophysiology of the disorder is the most likely. Frequently, disruption due to tics is overshadowed by comorbid conditions, like obsessive compulsive behaviors, attention deficit hyperactivity disorder, other behavioral disorders and learning disabilities, so the management should be targeted to them. Conclusion. The knowledge about the basic mechanisms and the integral definition of the clinical spectrum of tics and neurobehavioral manifestations, and its natural history in a patient with GTS, allow us to establish a more rational approach for management and prognosis.