Current Pharmacology of Tourette Syndrome

by Roger Kurlan, MD

Chronic, multiple motor and vocal tics are the most prominent clinical features of Tourette Syndrome (TS) and represent the signs upon which the diagnosis of the disorder is currently based. Tic severity encompasses a wide spectrum. Although a number of patients with TS experience severe and disabling tics, recent family studies indicate that for most individuals with the disorder, tic severity is relatively mild and medical attention is not required.

Tics may also be accompanied by a variety of associated behavioral disorders. For example, recent genetic work suggests that obsessions (recurrent, persistent ideas, thoughts, images, impulses) and compulsions (repetitive behaviors performed as rituals or in a stereotyped fashion) may be clinical manifestations of TS. Others have suggested that attention deficit disorder (short attention span, daydreaming, poor concentration) with hyperactivity (ADHD) also may be associated with TS. For some individuals, such behavioral disturbances may represent the predominant clinical manifestations of the disorder. In addition, patients with TS may display a variety of other psychopathological conditions (e.g., depression, anxiety, conduct disorder) and personality traits (e.g., irritability, argumentativeness, stubbornness, impulsivity) that may be part of the disorder or, alternatively, may represent psychological responses to living with a chronic illness.

Taken together, current evidence indicates that the clinical manifestations of Tourette Syndrome can be quite variable. It is, therefore, important to evaluate each individual closely to determine which aspects are most disabling. For example, school performance may be impaired by frequent tics, obsessional thinking, attention deficit, personality disorder, or various combinations of those difficulties. For most patients, one or two of the clinical aspects will predominate and can serve as specific target symptoms for therapy.

Ideally, patients with mild cases of TS who have made a good adaptation in their lives can avoid the use of any medications. Our impression is that the majority of patients with TS can manage well without drug therapy. Educating patients, family members, and school personnel concerning the nature of TS; restructuring the school environment (e.g., small group teaching, one-on-one tutoring; allowing TS students to work at their own pace); and providing supportive counseling, are measures that may be sufficient to avoid medications.

Medication therapy should be considered if it is determined that the symptoms of TS are functionally disabling and not remediable to non-drug interventions. A variety of therapeutic agents are now available to treat the symptoms of TS. Each medication should be chosen on the basis of specific target symptoms and potential side effects. For example, in one patient, tic-suppression may be the important goal, while treatment of obsessive-compulsive features may take precedence in another. Dosages should be titrated slowly in order to achieve the lowest satisfactory dosage. The maximum dosage utilized depends on achieving a "tolerable" suppression of symptoms. "Tolerable" is determined by the nature of the symptoms (for example, coprolalia is usually less tolerable than eye tics) and the ability of an individual to exercise voluntary modulation of his/her vocalizations and motor tics. Some children may have relatively few tics in school but a great many at home, thus allowing less overall use of medication. In our view, home is a haven where a child can have some relief from holding symptoms in check. It is vital that the patient and the family understand the ever changing nature of TS, so that medications can be adjusted in a rational fashion, increasing when the symptoms upsurge and decreasing during periods of relative remission. It is essential for effective dosage adjustment that "target symptoms" are monitored at all times. For example, if an individual is treated with so much medication that all movements are suppressed, it can never be known when tics, which tend to wax and wane in severity during the course of the illness, decrease spontaneously. In the long run, our goal is to use as little medication as possible (i.e., "less is best").

Pharmacotherapy of Tourette Syndrome

Dr. Kurlan is an Associate Professor, Department of Neurology, University of Rochester School of Medicine, Rochester, NY

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Reviewed: 03/2006