Tourette's Syndrome: clinical review and analysis of 70 Chilean patients

Background: Tourette's syndrome is a childhood-onset hereditary neurobehavi oural disorder believed to occur without geographical restrictions. Althoug h there have been reports of this disorder worldwide just a few are from La tin America. Aim: To report a preliminary experience with a series of 70 pa tients and to review recent advances in this disorder. Patients and Method: We reviewed patients seen in pediatric and adult neurological clinics in S antiago, Chile, all of whom fulfilled clinical diagnostic criteria for Tour ette Syndrome. Results: Seventy patients were studied, 54 males (77.1%) and 16 females (22.8%), their mean age at first evaluation was 13.6 years (ran ge 2.46). The mean age of onset of symptoms was 6.4 (range 2-20), the mean time of follow-up was 3 years. Fifty-eight patients showed simple motor tic s (blinking, facial grimacing, shoulder shrugging), whereas dystonic tics l ike head jerking were seen in 38 patients, torticollis in 6 and oculogyric movements in 2. Complex motor tics like jumping, antics, trunk bending and head shaking were present in 16 subjects. Vocal tics were predominantly of the simple type: sniffing, throat clearing, blowing, and whistling. Complex vocal tics were seen in 12 patients, five cases showed palilalia, 3 echola lia and only six displayed coprolalia (8.5%). Tics were of mild to moderate severity in most patients. Obsessive-compulsive disorder was observed in 2 2.8% and attention deficit and hyperactivity disorder were present in 35.7% . Forty-five patients (64.2%) had a first degree relative with tics, nine p atients (12.8%) had a family history of obsessive-compulsive disorder. The current evidence involving desinhibition of cortico-striatum-thalamic-corti cal neuronal circuits in the pathogenesis of this disorder is analyzed. Con clusion: Our report supports the recognized clinical homogeneity and geneti cal basis of Tourette's syndrome regardless of geographical region and ethn ic origin.