The Gilles de la Tourette syndrome (GTS) is a neuropsychiatric disorder with childhood onset and characterized by motor, vocal, sensory and cognitive tics that may vary from relatively mild to very severe. The tics are often accompanied by echo- and coprophenomena, all in a characteristic waxing and waning course. In clinicial populations obsessivecompulsive symptoms, attention deficit disorder with hyperkinesia, conduct disorder and self-injurious behaviors are frequently observed in GTS patients. The severity of the GTS symptoms differs from person to person and may vary within an individual during his life. The results of treatment, that consists of (supportive) counseling, pharmacotherapy and/ or (behavioral) therapy, are often not satisfactory, especially in the more severe cases. The exact etiology of GTS is unknown. Disturbances of the central dopaminergic, noradrenergic, serotonergic, GABA-ergic and endorphin systems in the brain are suggested to be involved in the pathogenesis of GTS. In view of the unknown etiology of this condition and the unsatisfactory treatment outcome in the majority of cases, the American Tourette Syndrome Association has launched research initiatives into the etiology of the disorder. In this program a number of American and European research groups, including a Dutch group, collaborate in trying to unravel the genetic background of GTS. Systematic family studies are carried out to establish the mode of inheritance and ultimately to map and clone the susceptibility gene(s). The study comprised the following steps: 1. The ascertainment of families of GTS probands and the diagnostic classification of the family members (described in section 2). 2. The statistical analysis of the family data to test the most likely mode of inheritance by segregation analysis and the power for linkage analysis by simulation studies (described in section 3). 3. The analysis of DNA marker data by linkage analysis (described in section 4).

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This study was carried out at the Departments of Psychiatry (Prof .Dr. W.J. Schudel) and Clinical Genetics (Prof. Dr. M.F. Nierrneijer, Prof .Dr. H. Galjaard, Dr. B.A. Oostra) of the Erasmus University Rotterdam and the University Hospital RotterdarnDijkzigt. It was supported by the American Tourette Syndrome Association, the Dutch 'Praeventiefonds', the 'Stichting Klinische Genetica Rotterdam' (Prof.Dr. H. Galjaard), Lundbeck BV. and Duphar-Nederland BV.
M.F. Niermeijer (Martinus) , W.J. Schudel (Willem)
Erasmus University Rotterdam
Erasmus MC: University Medical Center Rotterdam

van de Wetering, B. (1993, June 15). The Gilles de la Tourette syndrome : a psychiatrisch - genetic study. Retrieved from