Tourette’s Syndrome (Ts)

TS is a neurobehavioral disorder named after the French neurologist Georges Gilles de la Tourette. It predominantly affects males, TS is characterized by multiple motor tics often accompanied by vocalizations (phonic tics).
A tic is a brief, rapid, recurrent, and seemingly purposeless stereotyped motor contraction. Motor tics can be simple, with movement only affecting an individual muscle group (e.g., blinking, twitching of the nose, jerking of the neck), or complex, with coordinated involvement of multiple muscle groups [e.g., jumping, sniffing, head banging, and echopraxia (mimicking movements)]. Vocal tics can also be simple (e.g., grunting) or complex [e.g., echolalia (repeating other people’s words), palilalia (repeating one’s own words), and coprolalia (expression of obscene words)]. Patients may also experience sensory tics, composed of unpleasant focal sensations in the face, head, or neck. Patients characteristically can voluntarily suppress tics for short periods of time, but then experience an irresistible urge to express them. Tics vary in intensity and may be absent for days or weeks only to recur.Tics tend to present between ages 2 and 15 years (mean 7 years) and often lessen or even disappear in adulthood. Associated behavioral disturbances include anxiety, depression, attention deficit hyperactivity disorder, and obsessive-compulsive disorder. Patients may experience personality disorders, self-destructive behaviors, difficulties in school, and impaired interpersonal relationships.

Etiology and Pathophysiology

TS is thought to be a genetic disorder, but no specific gene mutation has been identified. Current evidence supports a complex inheritance pattern, with one or more major genes, multiple loci, low penetrance, and environmental influences. The pathophysiology of TS is not known, but alterations in dopamine neurotransmission, opioids, and second-messenger systems have been proposed.

Treatment:

Patients with mild disease often only require education and counseling (for themselves and family members). Drug treatment is indicated when the tics are disabling and interfere with quality of life. Therapy is generally initiated with the ALPHA-agonist clonidine, starting at low doses and gradually increasing the dose and frequency until satisfactory control is achieved. Guanfacine (0.5–2 mg/d) is an ALPHA-agonist that is preferred by many clinicians because it only requires once-a-day dosing. If these agents are not effective, antipsychotics can be employed. Atypical neuroleptics (risperidone, olanzapine, ziprasidone) are preferred as they are thought to be associated with a reduced risk of extrapyramidal side effects. If they are not effective, low doses of classical neuroleptics such as haloperidol, fluphenazine, or pimozide can be tried. Behavioral features, and particularly anxiety and compulsions, can be a disabling feature of TS and should be treated