[Tourette's syndrome: psychopathology of development in a model of neuropsychiatric dysfunction in children].

The complex neuropsychiatric disorder described by Gilles de la Tourette is a childhood onset, generally lifelong condition diagnosed on the basis of multiple, everchanging motor and vocal tics. Genetic evidence indicates that the vulnerability to Tourette's Syndrome (TS) is transmitted as an autosomal dominant genetic trait. This vulnerability has a broad range of expression, from full-blown TS to chronic multiple tics; an alternate manifestation may be obsessive compulsive disorder. There is also a broad range of severity, from quite mild to incapacitating. No specific neuroanatomical or neurochemical basis has yet been discovered, but the basal ganglia and dopaminergic systems have been implicated. Children and adults with TS experience the intrusion into consciousness of unwanted and disturbing sensations, thoughts and desires; they repeatedly experience the need, arising from an "alien" force within themselves, to produce sounds and actions which they try to resist but to which they eventually capitulate. Their symptoms elicit strong responses from family and others, as well as themselves. Psychoanalytically guided, clinical study can help illuminate the inner world of individuals with TS and define the pathways between biological vulnerability and clinical expression. Treatment often requires a combination of approaches, including guidance, psychotherapy and, in a minority of cases, the careful use of medication. Over the past decade, TS has served as a model developmental disorder for studying the interactions between biological vulnerability and environmental responses in the emergence of a clinical disorder and in the shaping of a child's character. During the next years, further advances in the study of TS should help clarify basic issues in the transmission of constitutional vulnerability and suggest approaches to prevention and early intervention.