Gilles De La Tourette’s Syndrome

Tourette’s Syndrome is initially characterized by motor tics in 80% of cases, and these tics most commonly involve the face. In the remaining 20%, the initial symptoms are phonic tics; all patients ultimately develop a combination of different motor and phonic tics. These are noted first in childhood, generally between the ages of 2 and 15. Motor tics occur especially about the face, head, and shoulders (eg, sniffing, blinking, frowning, shoulder shrugging, head thrusting, etc).

Phonic tics commonly consist of grunts, barks, hisses, throat-clearing, coughs, etc, but sometimes also of verbal utterances including coprolalia (obscene speech). There may also be echolalia (repetition of the speech of others), echopraxia (imitation of others’ movements), and palilalia (repetition of words or phrases). Some tics may be self-mutilating in nature, such as nail-biting, hair-pulling, or biting of the lips or tongue. The disorder is chronic, but the course may be punctuated by relapses and remissions.

Obsessive-compulsive behaviors are commonly associated and may be more disabling than the tics themselves.
Examination usually reveals no abnormalities other than the tics. In addition to obsessive-compulsive behavior disorders, psychiatric disturbances may occur because of the associated cosmetic and social embarrassment. Electroencephalography may show minor nonspecific abnormalities of no diagnostic relevance.

The diagnosis of the disorder is often delayed for years, the tics being interpreted as psychiatric illness or some other form of abnormal movement. Patients are thus often subjected to unnecessary treatment before the disorder is recognized. The tic-like character of the abnormal movements and the absence of other neurologic signs should differentiate this disorder from other movement disorders presenting in childhood. Wilson’s disease, however, can simulate the condition and should be excluded.

Treatment

Treatment is symptomatic and may need to be continued indefinitely. Haloperidol is generally regarded as the drug of choice. It is started in a low daily dose (0.25 mg) that is gradually increased (by 0.25 mg every 4 or 5 days) until there is maximum benefit with a minimum of side effects or until side effects limit further increments. A total daily dose of between 2 and 8 mg is usually optimal, but higher doses are sometimes necessary. Treatment with clonazepam (in a dose that depends on response and tolerance) or clonidine (2–5 mg/kg/d) may also be helpful, and it seems sensible to begin with one of these drugs in order to avoid some of the long-term extrapyramidal side effects of haloperidol. Phenothiazines, such as fluphenazine (2–15 mg daily), have been used, but patients unresponsive to haloperidol are usually unresponsive to these as well.

Pimozide, an oral dopamine-blocking drug related to haloperidol, may be helpful in patients who cannot tolerate or have not responded to haloperidol. Treatment is started with 1 mg daily and the daily dose increased by 1–2 mg every 10 days; the average dose is between 7 and 16 mg daily.

There are a few anecdotal reports that calcium channel blockers may be helpful, but this requires further study. Online Canadian Pharmacy

10240:21:1 Weeks RA et al: Tourette’s syndrome: A disorder of cingulate and orbitofrontal function? Q J Med
1996;89:401. (Clinical characteristics and treatment strategies.)

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