Gilles-de-la-Tourette Syndrome in Childhood: Molecular, Neurobiological and Genetic Aspects

Abstract

Animals often exhibit repetitive and predictable behaviors. These repetitive actions can be learned and become habits, which can be advantageous from an evolutionary standpoint as they reduce cognitive strain and attentional resources. Repetitive behaviors can also be intentional and conscious, occurring without habit formation, especially in normal child development or certain neuropsychiatric conditions. When these behaviors disrupt social interactions and daily functioning, they may be considered pathological. Conditions such as Gilles de la Tourette syndrome (GTS) can manifest as compulsive, stereotyped, and ritualistic behaviors. The dopaminergic and serotonergic pathways are believed to play an important role in the development of GTS. The striatum nucleus in the basal ganglia is believed to play a key role in regulating these repetitive behaviors through its connections with various areas of the cortex. However, the specific mechanisms within the striatum, including its organization, cellular functions, and connections, are still actively researched. At the cellular level, post-mortem studies have found a reduced number of parvalbumin-expressing and cholinergic interneurons. There is extensive evidence that the dopaminergic signal transmission in the striatum is disrupted. This work focus on an overview of Gilles de la Tourette syndrome with special focus on molecular aspects, treatment options and recent research with a focus on new targets to treat this rare hereditary disorder in childhood.