Tourette’s syndrome is a disorder of both motor and vocal tics. If you see or hear both of these, you can make the diagnosis with confidence. The media tends to characterize Tourette’s by the presence of corprolalia, the disorder of sudden explosions of expletives, but this actually only occurs in about 10% of cases (Singer HS, Lancet Neurol 2005;4(3):149–159).
Most vocal tics are brief coughs, sniffs, grunts or guttural noises, but vocal tics can be whistles, hums, words, phrases, obscenities, absurdities, echolalia, and even “kissy” noises. Motor tics can be hard to differentiate from complex motor habits and sometimes from partial seizures and dyskinesias. Motor tics are most often sudden, short, explosive movements involving one muscle group—jerks rather than anything much more complicated: eye blinks, an arm flying up, a bow at the waist are all examples.
However, any motor movement could be a tic, and tics are stereotyped in the short term but can change over time. A patient may have frequent bursts of eye blinks for several months, then the eye blinks will stop and a shoulder shrug will begin, only to be replaced by a head twist.
Tics behave oddly. They tend to come in bursts, both in the micro time scale (eg, three tics, then a pause) and in larger time scales (several days of worse tics, then a pause), and in larger time scales than those (a month of multiple episodes of a few days of tics occurring several times in a row, several times a day). A tic diary can help with medication trials, so you know whether the effect is due to the natural cycle of the tics or the medication.
Tics are often made more severe by pointing them out, or by anxiety or excitement. They can be triggered by unexpected sensory stimuli—a particular sound or sight can induce a bout of tics. Tics can be controlled in the short term, leading parents and teachers to sometimes conclude they are willful, but the suppression builds up tension that then requires release. It’s a bit like suppressing a sneeze. Some children will “hold” their tics until then can go to a private place, then tic many times before coming out again. This ability to wait to express a tic is the basis for one of the most successful treatment approaches, habit reversal training.
Tics commonly emerge between the ages of three and seven, are generally worst around age 10 to 12, and then decrease throughout adolescence, although they can persist into adulthood. Motor tics usually come first, followed by vocal tics a few years later. Simple tics usually precede more complex ones. Obsessive compulsive disorder and attention deficit disorder are common comorbidities—the majority of patients with Tourette’s syndrome have at least one if not both additional disorders.
Comorbid ADHD presents a lifelong course as it does in other ADHD sufferers, while comorbid OCD behaves differently. The OCD tends to emerge as the tics are resolving in adolescence, and may spontaneously resolve in early adulthood. Compulsions can be difficult to differentiate from tics, but the premonitory feeling or urge is different, so your best bet is to ask the patient whether it feels like a tic or a compulsion.
There are some differences in the expression of OCD among those with comorbid Tourette’s and those with garden variety OCD. For example, TS patients tend to have obsessions centered on symmetry and getting things “just right”; in addition, they tend to have more violent and sexual obsessions, are more often male, have an earlier age of onset, and may be less responsive to treatment with SSRIs. They also report more touching, counting, blinking and staring obsessions; whereas patients with OCD without Tourette’s report more contamination obsessions and washing behaviors (Miguel EC et al, Mol Psychiatry 2005;10(3):258–275;Miguel EC et al, Adv Neurol 2001;85:43−55).
Disruptive behavior and anger outbursts are also common with tic disorders. These may be a manifestation of the emotional impulsivity of ADHD rather than a separate syndrome. Learning disabilities, mood disorders, and other impulse control disorders can also occur.
The approach to treatment of children with Tourette’s is to first assess the patient for symptoms of ADHD, OCD, and other psychiatric disorders, then determine which bothers the child the most. Parents often focus on the tics, but that may not be the source of the greatest functional impairment. If OCD or ADHD appears to be causing the most trouble, treat either as you would the isolated disorder. Decreasing anxiety often improves both focus and tics, and increasing focus often improves both anxiety and tics.
As Dr. Coffey points out in this issue’s Expert Q&A, tics do not necessarily worsen, and may in fact improve, when the patient is given stimulants. If the tics are the most impairing disorder for your patient, habit reversal training is first line treatment. (See the sidebar for a review of this.)
Sometimes, however, medication is needed. First line medication treatment is alpha-adrenergic blockers (such as guanfacine 1 mg to 2 mg daily) or clonidine (0.05 mg to 0.2 mg three or four times a day)—more due to safety than efficacy. They don’t work for everyone but they are much safer for those they do help. The FDA approved treatment is low-dose, high potency neuroleptics, haloperidol (Haldol) or pimozide (Orap), 0.5 mg daily of either, but due to the long term risks, newer high potency antipsychotics such as risperidone (Risperdal) or aripiprazole (Abilify) are preferred by most physicians. Low dosing is still generally appropriate, 0.25mg to 0.5 mg of risperidone or 1 mg to 2 mg of aripiprazole is a good place to start.
An important intervention is educating the patient, family, teachers, and peers. To this end, the Tourette’s Syndrome Association maintains an excellent website and educational material for both patients and clinicians, including videos, a newsletter written by kids with Tourette’s, research links, conferences, lobbying, and activities. They even have their own drug study! You can visit the site at www.tsa-usa.org. You may also want to check out the Emmy Award winning HBO documentary, “I Have Tourette’s, But Tourette’s Doesn’t Have Me.”
Information from the following sources was used in this article: Leckman JF, Brain Dev 2003;25(Suppl 1):S24–S28; Grados MA and Mathews CA, J Psychosom Res 2009;67(6):491–496.