The goal of treatment is not necessarily to completely eliminate all the tics, but to achieve a tolerable suppression of the involuntary movements and noises. Because of the variability of tics in terms of severity, frequency, and distribution, the assessment of efficacy of a therapeutic intervention on tics is often quite problematic. Before initiating any therapy, the patients and their parents should be instructed how to evaluate and "quantitate" the tics so they can provide objective assessment of the response to the therapeutic intervention. Among the guiding principles in the treatment of tics is to have realistic expectations. Furthermore, it is essential that each medication is given adequate time to work and that the patients are observed for evidence of potential adverse effects without necessarily panicking when a new symptom occurs, as this may or may not be a side effect of the prescribed medication, but may be completely unrelated.

     Over the years, placebo-controlled trials as well as observations by experienced clinicians have concluded that drugs that block dopamine transmission are by far the most effective in suppressing tics. Almost all currently used anti-tic drugs, called neuroleptics, act by blocking the brain's dopamine receptors, but one drug, tetrabenazine, acts by depleting the storage of dopamine. Although haloperidol (Haldol) and pimozide (Orap) are the only neuroleptics approved by the Food and Drug Administration (FDA) for the treatment of TS, I rarely ever use these two drugs, because of relative lack of efficacy and high incidence of side effects. These include sedation, weight gain, school phobia, liver toxicity, and many other potential adverse effects, including tardive dyskinesia. This latter condition consists of continuous involuntary movements, different from tics, that usually involve the lower face, jaw and tongue, which may be permanent, particularly if not detected early. This potential complication is one reason why all patients treated with neuroleptics should be carefully monitored by their physicians who must be skilled in recognizing early signs of tardive dyskinesia. Of the dozen or so neuroleptics currently on the market, I prefer fluphenazine (Prolixin) since it appears to have a lower incidence of sedation and other side effects than the other neuroleptics. Furthermore, after treating hundreds and hundreds of TS patients with this drug I have never observed tardive dyskinesia, even though this is a potential complication of this treatment. If fluphenazine fails to adequately control tics, I usually substitute risperidone (Risperdal), but in many cases, particularly if their tics are troublesome, I will initiate therapy with tetrabenazine. While a powerful anti-tic drug, tetrabenazine has not yet been approved for clinical use in the United States. We are working diligently with the FDA to have the drug finally approved in 2008, at least for the treatment of chorea associated with Huntington's disease. Once approved for this indication, physicians will be able to prescribe it for treatment of other movement disorders, including tics. Since I received a special permission from the FDA in 1979 to use tetrabenazine, I have used it in well over a thousand patients and have never seen tardive dyskinesia as a complication. Over the past three decades I have published dozens of articles demonstrating the efficacy and safety of tetrabenazine in the treatment of a variety of movement disorders including tics associated with TS. Although usually well tolerated, tetrabenazine may cause drowsiness, slowness of movement, and mood changes, but all these side effects are dose related and usually resolve when the dosage is reduced or the drug is discontinued.

     In patients who have localized (focal) but disabling tics, local injection of botulinum toxin may provide a 3-6 month relief. We were the first to publish results of botulinum toxin injection into vocal cords in patients with TS who experience phonic tics, such as loud screaming and shouting of obscenities (coprolalia). Botulinum toxin is also very effective in treating blinking tics that may otherwise render patients essentially blind, severe "whiplash" tics manifested by sudden extension or flexion of the neck that could otherwise damage the cervical spinal cord, and other troublesome tics. Taking advantage of the well known premonitory sensation, a feeling in the region of the tic prior to the movement, the botulinum toxin injection can be targeted to the most appropriate muscle(s). Although botulinum toxin is the most powerful biologic toxin known to man, when used appropriately it rarely causes serious side effects, but it may be associated with transient weakness of the injected muscles.

Finally, more recently I and my associates in the TS Clinic at Baylor College of Medicine have also used surgery to treat patients who have such disabling tics and obsessive compulsive disorder that their health and life were in jeopardy, hence the term "malignant TS." Placing electrodes into the part of the brain that is involved in generation of tics and stimulating it with a high frequency stimulus has been found effective not only in controlling the tics but also the associated behavioral problems. With better understanding of the genetics and pathophysiology of tics, it is likely that more effective therapies will be developed in the future.

    Joseph Jankovic, M.D. is the Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine in Houston and Chairman of the TSA of Texas Medical Advisory Board.  Click here to visit Dr. Jankovic's website for further information.