Font Size: 

Tourette syndrome (TS) is a neuropsychiatric disorder. Based on the clinical and neurophysiological evaluation, we suggested that the pathophysiology of TS involveddecreased tyrosine hydroxylase (TH) activity of the terminal of nigrostriatal dopamine (NS-DA) neuron and associated developmental DA receptor upregulation. TH activity of the terminal of NS-DA neuron is known to show the age related exponential decrease, high ininfancy rapidly decreasing in the first decade, moderately in the second decade, andstabilizing after. Acceleration of this age dependent change of TH activity is reflected in thecharacteristic age dependent changes of the clinical features of TS.We experienced newly diagnosed 611 cases of TS (male 451, female 160) in our clinic inpast 2 years 11 months. Clinical features show the interesting homogeneities andheterogeneities in tics and comorbidities. In addition to the typical motor and vocal tics,complex movements similar to dystonia or epileptic attacks were observed. In addition tofrequently observed comorbidities such as attention deficit hyperkinetic disorder (ADHD)and obsessive compulsive disorder (OCD), various sleep disorders, sleep rhythmdisorders, restless leg syndrome and autism spectrum disorders were frequently observed.Sleep disorders occurred clock-dependently and suggested REM (rapid eye movement)sleep stage related disorders. These REM sleep related disorders reflect the DA receptorsupersensitivity.Low dose levodopa, which improves the DA receptor supersensitivity, or low dose ofaripiprazole showed improvement not only tics but also above sleep disorders and restlessleg syndrome.These clinical experiences supports the hypothesis that TS, as a developmental dopaminedisorder.

tourett syndrome; ADHD; Obsessive Compulsive Disorder