Videotapes recorded 1978 through 1991 of 56 children (ages 8 to 14) with Gilles de la Tourette syndrome (GTS) were reviewed and 31 of the patients (28 men and 3 women, age>20 years) were recruited for a second videotape and in-person assessment at Rush-Presbyterian-St Luke’s Movement Disorder Center, Chicago, IL. Tics were rated for body areas involved, motor and phonic tic frequency, and motor and phonic tic severity. Ninety percent of patients still had tics as adults, and 50% had objective evidence of tics. Adult patients who considered themselves free of tics were frequently found to exhibit tics on objective assessment. Despite this perceived disparity, the objective tic disability was significantly diminished in adults compared to that in childhood. All tic domains (body area involved, frequency, severity) had improved, and especially motor tic severity (p=0.008). Improvements were not related to medication use; only 13% of adults were receiving medication for tics compared to 81% during childhood. In spite of substantial childhood educational or social problems, most adults were either employed or attending school full-time. One quarter of the sample was disabled as adults, with alcohol abuse, unemployment, criminal activity or other social dysfunction. Early life dysfunction closely correlated with adult dysfunction. Tics in GTS will improve over time but often persist in to adulthood. Early and assertive intervention in childhood is recommended and may serve to prevent dysfunction in adulthood. [1]

COMMENT. Tourette syndrome (TS) is a relatively common neurobehavioral disorder of childhood with a prevalence of 0.7% to 4.2%. TS may persist in to adulthood, but its frequency and severity are usually diminished. TS is characterized by motor and vocal tics, and is frequently associated with attention deficit/hyperactivity disorder and obsessive-compulsive disorder. Autosomal dominant, recessive, and bilineal transmission has been postulated. The co-occurrence of patients with TS and Rett syndrome in the same families has been described, suggesting a common underlying genetic defect. Screening for mutations in the MECP2 (Rett syndrome) gene in 31 male TS patients with no family history of TS found none. MECP2 gene is neither a frequent cause nor a modifier of the clinical phenotype in TS. [2]