eMedicine Specialties > Neurology > Pediatric Neurology
Tourette Syndrome and Other Tic Disorders: Treatment & Medication
Updated: Sep 2, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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Treatment
Medical Care
General principlesSome general principles must be kept in mind. First, all present treatments of TS are purely symptomatic. No curative or preventive treatments are known. Second, tics often are not the worst problem. Third, this is a chronic disorder, and usually the goal is long-term benefit rather than quick improvement at any cost. Fourth, symptoms frequently improve or worsen over any period of time, even in untreated TS. Corollaries of these principles include the following: Treatment is not always needed; treatment should be directed first at the most troublesome symptom; apparent success or failure of any treatment may be coincidental; and beginning with reasonable trials of single agents is usually better than rushing to high doses or polypharmacy.
TS has been described as either a neurological or a psychiatric disorder. These labels have nothing to do with the cause or treatment of TS but simply relate to the fact that neurologists and psychiatrists have been the main medical experts who have researched and treated TS. These specialists have been well represented on the medical and scientific advisory boards to the TSA. A parent of a child with TS gave the author the following advice on choosing a physician: "We don't care if it's a psychiatrist or a neurologist, but we do care that it is someone who has experience treating Tourette's syndrome and who will treat all the symptoms."
Chronic motor (or vocal) tic disorder is managed similarly to TS and not discussed separately.
Discussed below are proven treatments for tics from replicated controlled studies, other treatments for tics, treatment for obsessive-compulsive symptoms in patients with tics, treatment for ADHD in patients with tics, and treatment for other symptoms in patients with tics.
- Proven treatments for tics from replicated controlled studies
- Dopamine D2 receptor antagonists: In 1959, soon after its introduction, chlorpromazine was reported to dramatically improve tic severity.127 Since then, several allocation RCTs with various neuroleptics (eg, haloperidol, fluphenazine, pimozide) have confirmed these initial results.128 On average, tic severity declines by approximately 50-80% with neuroleptic treatment.
- Neuroleptic drugs are the current standard in terms of efficacy in the treatment of tics. They can be effective at doses far below the usual treatment dose for psychosis, and most adverse effects are manageable with pharmacologic manipulations. Unfortunately, many patients do not tolerate acute adverse effects (most commonly sedation, weight gain, depression, lethargy, and akathisia), and prolonged treatment poses a small risk of tardive dyskinesia. Therefore, other treatments have been investigated.
- Risperidone, olanzapine, and ziprasidone produced at least as much clinical effect as a classic neuroleptic comparator, with fewer adverse effects.129,130,131 A small study of clozapine suggested little effect.132 Small studies of the dopamine D2R partial agonist aripiprazole effective for tic suppression,133 but RCT data are not yet available.
- Metoclopramide is a D2 receptor antagonist that is usually used for nausea. A case series134 and an RCT135 suggest it treats tics with good short-term tolerability. However, long-term use of metoclopramide has been associated with tardive dyskinesia.
- Dopamine agonists: Paradoxically, several mixed dopamine agonists have also been proven effective in reducing tic frequency.14,15,136,17 To date, they have been tested exclusively in relatively low doses, partly because of a theory that, at these doses, they must antagonize dopamine function by selective action at presynaptic receptors. However, accumulating evidence suggests that this rationale is faulty, and trials with higher doses may be expected. Similarly, the present author and colleagues currently are conducting a placebo-controlled double-blind study of levodopa as a treatment for tics.137
- Habit reversal therapy: Five RCTs have demonstrated the efficacy of a specific form of behavior therapy for tics.138,139,140,141 The originally tested treatment consisted of a package of interventions called habit reversal therapy142 : monitoring, relaxation, and other nonspecific elements of behavior therapy. The most important element is application of a competing response whenever the patient notices either a tic or the urge to tic.
- Initially, heavy effort on the part of the patient may be needed. However, in all 4 reported studies, at long-term follow-up at least one half of treated patients had greater than 75% reduction in overall tic severity, whether based on self-report of home tic counts or on blind review of a videotape filmed in the clinic.
- The effort expended by patients decreased dramatically as tic frequency declined, usually within the first few weeks of treatment. No substitution of other tics was noted, which commonly occurs when patients substitute a volitional action on a haphazard basis (see Media file 5).
Tourette syndrome and other tic disorders. In a randomized controlled trial of habit reversal therapy (HRT), results differed significantly from those of a control therapy (massed practice; P<.001, analysis of variance). The HRT group had a 97% reduction in tics at 18-month follow-up, with 80% of patients tic-free.
- Anecdotally, others have not found such impressive results, which may relate to patient selection or therapeutic technique. Further replication studies are being supported by the TSA.
- Interestingly, several elements of this treatment are reminiscent of treatments used by Brissaud in 1902 (though with a radically different theoretical background).4 Some data now explain why his treatments may not have been as effective. If the competing response is not paired with tic urges or tics, no benefit is observed.143 Similarly, other behavior therapies used in the last several decades (eg, massed practice) are relatively ineffective.
- Since the realization of the failures of psychoanalysis in treating tic disorders in the 1970s, patients and physicians have looked askance at psychological treatment, including behavior therapy. The available data no longer justify this view. In fact, the plausibility of behavior therapy makes some sense on an intuitive level. Since tics respond briefly even to random environmental influences, it is not surprising that a well-designed behavioral intervention may produce more satisfactory results. Note that this is very different from simply telling the patient not to tic, or from "trying harder," neither of which tends to be effective over the long run.
- A parallel is present with obsessions and compulsions, which share many phenomenologic characteristics with tics. OCD symptoms do not respond well to psychodynamic treatment but are effectively treated with behavior therapy. Such treatment has biologic effects, such as normalization of abnormally high baseline metabolism in orbitofrontal cortex. Case series have shown a reduction in tics by using the same behavior therapy method proven to benefit patients with OCD.144
- Dopamine D2 receptor antagonists: In 1959, soon after its introduction, chlorpromazine was reported to dramatically improve tic severity.127 Since then, several allocation RCTs with various neuroleptics (eg, haloperidol, fluphenazine, pimozide) have confirmed these initial results.128 On average, tic severity declines by approximately 50-80% with neuroleptic treatment.
- Other treatments for tics
- Guanfacine: This agent was tested in an RCT in children with both ADHD and chronic tic disorders.145 The drug was clearly superior to placebo in the reduction of both ADHD and tic symptoms (31% on average), with few adverse effects. It also has been shown to be efficacious in adults with nontic ADHD.
- Clonidine: This drug has frequently been used to treat tics. A large RCT confirmed its efficacy for both ADHD symptoms and tics in patients with TS. Clonidine or guanfacine may be appropriate as a first agent in many patients.
- Norepinephrine reuptake inhibitors: Both desipramine and atomoxetine have shown definite though modest benefit for tics in tic patients being treated for ADHD.146,147
- Botulinum toxin injections and oral baclofen: After enthusiastic retrospective reports, blinded trials of these 2 agents have revealed statistically significant but clinically modest benefit compared with placebo.148 Botulinum toxin injections may improve urges or sensory tics, as well as observable tics, and it may be the treatment of choice for patients with a single, especially problematic, dystonic tic.
- Tetrabenazine: This is a presynaptic dopamine-depleting agent with the advantage that it has not been reported to cause tardive movement disorders. A retrospective report noted marked clinical improvement in 57% of 47 patients with TS.149 Its acute adverse effects are similar to those of neuroleptics. Tetrabenazine is approved by the US Food and Drug Administration (FDA) only for the treatment of chorea in Huntington's disease.
- Baclofen: This drug has little effect on average, but it also has relatively few adverse effects and may be appropriate in select patients.150
- Benzodiazepines: Retrospective reports suggest that benzodiazepines, such as clonazepam, reduce tic severity in some patients. The effect is less than that of neuroleptics and probably nonspecific. Adverse effects are fairly common. However, clonazepam is tolerated better than haloperidol on average, and when no clinical pressure exists for urgent treatment, it is a reasonable option.
- Levetiracetam: Although an initial study suggested that this medication is well tolerated and might prove effective,151 two subsequent double-blind studies found no benefit.
- SSRIs: These agents (eg, clomipramine, fluoxetine) improve tics in some patients, worsen them in others, and have no effect on tics in yet others.152,153,154,155 SSRIs may be reasonable first agents in patients with significant depression or OCD symptoms.
- Ondansetron: A double-blind RCT in patients aged 12-46 years with TS of ondansetron 8-24 mg/d showed efficacy for a self-report but not an observer-rated measure of clinical improvement.
- Naltrexone/naloxone: These agents have been reported helpful in a few patients, but other studies have shown transient worsening of tics with opioid antagonists.156,50 An RCT of naloxone showed some benefit at low doses, but worsening of tics at higher doses. Case reports also have described benefit with opioid agonists.157,158
- Cannabinoids: Two RCTs support the efficacy of cannabinoids in reducing tic severity in some patients.10
- Nicotine: Both nicotine and a nicotine antagonist, mecamylamine, have been touted as treatments for tics. The antagonist has few adverse effects at the doses recommended, but 1 RCT found no statistically significant effect versus placebo.159 A small blinded study did show some benefit.76 However, given that nicotine is not a safe drug, its therapeutic use should await more compelling proof of efficacy.
- Repetitive transcranial magnetic stimulation (rTMS) has not been effective in TS.160
- Surgical treatments are described in Surgical Care.
- Treatment for obsessive-compulsive symptoms in patients with tics
- Initial treatment of OCD in patients with tics usually consists of a selective serotonin reuptake inhibitor, generally at 3-4 times the antidepressant dose. More recently, risperidone monotherapy has been advocated as a first treatment, especially in patients with significant impairment from tics and from OC symptoms.
- Behavior therapy for OCD (eg, exposure and response prevention) is clearly proven to be effective.161 A trial of behavior therapy is indicated for every patient with clinically significant OCD symptoms unless the symptoms are substantially remitted by another intervention.
- In patients with tics (and perhaps in their relatives), obsessions respond better to fluoxetine plus haloperidol than to fluoxetine plus placebo.162 Therefore, even if tics are well-controlled, addition of a D2 antagonist is indicated if bothersome OCD symptoms do not respond adequately to conventional initial treatment.
- In a highly select group of patients who fit research criteria for sudden onset of tics or OCD associated with a proven recent streptococcal infection, OCD responded dramatically to intravenous immunoglobulin G (IVIG) or plasmapheresis.123
- See the eMedicine article on Obsessive-Compulsive Disorder or the 2003 review by Miguel and colleagues for further details of OCD treatment.
- Treatment for ADHD in patients with tics
- ADHD can be significant in patients referred for treatment of TS.
- Stimulants such as methylphenidate or dextroamphetamine represent the oldest class of psychotropic drugs still in common use, and have known safety profiles. They are the most effective treatments of ADHD. Their labeling includes warnings that they may cause tics,163 but several recent prospective studies show that their effect on tics is at worst temporary, even with continued use.164,165
- Stimulant use in people with ADHD does not cause future drug abuse and may even prevent it.166
- A comorbid tic disorder should not be considered a serious contraindication to the use of stimulants for treatment of ADHD.167 Several studies have shown that stimulants do not cause lasting worsening of tics.
- Methylphenidate may be better tolerated than dextroamphetamine in people with TS.163
- Clonidine has also been proven useful for ADHD in people with TS. The benefits of clonidine and methylphenidate are additive. Guanfacine most likely has similar effects.
- RCTs have also shown that desipramine and atomoxetine help with ADHD symptoms in people with TS146,147 ; tics also improve slightly.
- A double-blind RCT showed possible benefit for selegiline on ADHD symptoms and tics.168
- Bupropion may benefit ADHD but may temporarily worsen tics.169
- See the eMedicine article on Attention Deficit/Hyperactivity Disorder for further details on the conventional pharmacologic and behavioral treatment of ADHD.
- Treatment for other symptoms in patients with tics
- In carefully selected, tic-free adolescents with affect-laden episodes of aggression, replicated results from controlled trials show substantial efficacy of divalproex.170 Whether these results can be confirmed for rage attacks in TS remains to be proven. A retrospective observational study found that explosive outbursts refractory to previous treatment improved with aripiprazole in 24 of 25 patients; however, 22% of subjects discontinued treatment due to inability to tolerate aripiprazole.
- Selective serotonin reuptake inhibitors (SSRIs) may also be useful.152
- Research on the management of (other) conduct disorder symptoms in TS is sorely needed.
Surgical Care
Stereotactic neurosurgery, either to place deep brain stimulators or to ablate tissue, is indicated only rarely for the treatment of obsessions, compulsions, and possibly tics. Recent case reports suggest deep brain stimulation (DBS) in various sites may be helpful. However, this approach is limited to patients with exceptionally debilitating symptoms and those in whom prior, thorough trials of less dramatic interventions were ineffective. Such surgery should be carried out only in referral centers experienced with these procedures and after multispecialty evaluation of the patient.Consultations
- Patients should be evaluated at least once by someone with experience treating patients with TS, and they should be informed about how to contact a local support group or the national Tourette Syndrome Association office.
- Additional referrals may be needed for the following measures, depending on the needs of the patient and the skills of the primary physician evaluating the patient's TS:
- Botulinum injections
- Habit reversal therapy for tics; behavior therapy for OCD, ADHD, or conduct disorder; or psychiatric care of OCD, ADHD, or comorbid anxiety or depressive illness
- Neuropsychological testing and educational interventions to address learning disabilities or to help formulate an individualized education program
- Education of teachers, classmates, or work colleagues may be helpful.
- Legal assistance (eg, to protect a child's educational rights under the federal Individuals with Disabilities Education Act or for protection under the Americans with Disabilities Act)
- Family counseling
Diet
- Ordinary diet is not known to have an effect on tics.
- Some concentrated dietary supplements used as drugs (also called nutraceuticals) may affect tic severity.
- For example, one of the author's patients had a marked increase in tic severity while taking an herbal product marketed for weight loss and containing ephedrine, ginkgo, caffeine, guaraná, and other ingredients.
- Some nutraceuticals may possibly improve tic symptoms, but no adequate evidence exists at present.
- Furthermore, because these products do not undergo the meticulous scrutiny required of other drugs by the FDA, their safety in general is not well established. This is important since a large majority of patients with TS have used these drugs (Mantel, 2004).
- However, both the National Institutes of Health and the TSA have expressed interest in supporting properly designed research on such treatments, and adequately tested products may be hoped for in the future.
Activity
Activity may be undertaken as the patient wishes.
Medication
Choice of initial treatment depends largely on the following factors: (1) which symptoms (eg, tics, obsessions, impulsivity) are most problematic at presentation, (2) the severity of presenting symptoms, (3) the patient's sense of urgency for treatment, and (4) the patient's aversion to risk of likely or unlikely adverse effects.
For many patients the most reasonable option is to forgo treatment altogether. Education of patient and family (and teacher or employer) may suffice. If a single dystonic tic predominates, especially in the face, neck, or larynx, botulinum toxin injection is a reasonable first treatment.
If ADHD symptoms predominate the presentation, they can be addressed first. Guanfacine or clonidine has the best evidence for also improving tics; stimulants have the best efficacy for ADHD symptoms. Other options are noted in the treatment section above.
If OCD symptoms predominate the presentation, they can be addressed first, most likely with a serotonin reuptake inhibitor and/or risperidone.
If severe tics are the presenting symptom, a newer antipsychotic agent may be the best initial treatment. The dose used is substantially lower than the dose used to treat psychosis.
If tics are mild to moderate in severity or if they occur in risk-averse patients, any of the non-antipsychotic treatments described in Medical Care can be tried sequentially. Clonidine may be the most widely used, while habit reversal therapy likely has the lowest risk of serious adverse effect.
The combination of dopamine antagonists with stimulants is used sometimes, yet it makes little enough sense pharmacologically that other options should be explored thoroughly.
Antipsychotic agents
These agents affect dopamine receptors but also affect serotonin receptors involved with frontal lobe functions.
Risperidone (Risperdal)
Mixed dopamine-serotonin antagonist. Compared with other antipsychotics, may produce less sedation. Theoretically has a lower risk of tardive dyskinesia than haloperidol; clearly produces fewer acute adverse effects.
Adult
0.25-3 mg PO bid or equivalent dose qhs (mean final daily dose in 1 multicenter study was 3.8 mg)
Pediatric
Not established; 0.25-2 mg PO bid or equivalent dose qhs
Carbamazepine may decrease effects; may inhibit effects of levodopa; clozapine may increase levels
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Adverse effects of all antipsychotics may include sedation, akathisia, dystonia, parkinsonism, hyperprolactinemia, dysregulation of body temperature, and neuroleptic malignant syndrome; small risk of tardive dyskinesia at doses used to treat TS
Olanzapine (Zyprexa)
Atypical antipsychotic that produces fewer acute parkinsonian, akathitic, or dystonic adverse effects than haloperidol. In schizophrenia, has approximately 33%-50% the risk of tardive dyskinesia compared with haloperidol.
Adult
2.5-20 mg PO qhs
Pediatric
Not established; 2.5-5 mg PO qhs
Fluvoxamine may increase effects; antihypertensives may increase risk of hypotension and orthostatic hypotension; levodopa, pergolide, bromocriptine, charcoal, carbamazepine, omeprazole, rifampin, and cigarette smoking may decrease effects
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Adverse effects of all antipsychotics may include sedation, akathisia, dystonia, parkinsonism, hyperprolactinemia, dysregulation of body temperature, and neuroleptic malignant syndrome; small risk of tardive dyskinesia at doses used to treat TS; causes significant weight gain in many, though relative risk versus other antipsychotics controversial
Ziprasidone (Geodon)
Atypical antipsychotic. In a head-to-head study, caused less weight gain than olanzapine in schizophrenia.
Adult
Not established; 10-40 mg PO qhs
Pediatric
5-40 mg PO qhs (mean final daily dose in 1 study was 28.2 mg)
CYP-450-3A4 inhibitors (eg, erythromycin, ketoconazole) may increase serum levels; CYP-450-3A4 inducers (eg, carbamazepine, rifampin) may decrease serum levels; drugs that increase QT/QTc interval (eg, amiodarone, fluoroquinolones) increase risk of life-threatening arrhythmias
Documented hypersensitivity; patients with clinical or ECG evidence of long QT syndrome or those taking drugs that cause torsades de pointes or prolong QT interval (see CARE Foundation)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Adverse effects of all antipsychotics may include sedation, akathisia, dystonia, parkinsonism, hyperprolactinemia, dysregulation of body temperature, and neuroleptic malignant syndrome; small risk of tardive dyskinesia at doses used to treat TS; causes prolongation of QT interval more so than risperidone, olanzapine, or haloperidol, though less than thioridazine (Mellaril); for other drugs, ECG effect is associated with serious cardiac arrhythmias
Haloperidol (Haldol)
Anti-tic efficacy of haloperidol has been known for 40 y.
Adult
0.25-5 mg PO qhs
Pediatric
Administer as in adults
May increase serum concentrations of TCAs and hypotensive action of antihypertensive agents; phenobarbital or carbamazepine may decrease effects; anticholinergics may increase intraocular pressure; lithium may cause encephalopathy-like syndrome
Documented hypersensitivity; narrow-angle glaucoma; bone marrow suppression; severe cardiac or liver disease; severe hypotension
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Adverse effects of all antipsychotics may include sedation, akathisia, dystonia, parkinsonism, hyperprolactinemia, dysregulation of body temperature, and neuroleptic malignant syndrome; small risk of tardive dyskinesia at doses used to treat TS; possible dysphoria, sometimes of significant intensity; several cases of school phobia apparently related to haloperidol have been reported
Fluphenazine (Prolixin)
High-potency typical antipsychotic with pharmacology similar to that of haloperidol. Proven to diminish tic severity.
Adult
0.25-5 mg PO qhs
Pediatric
Not established
May potentiate effects of narcotics, including respiratory depression; lithium increases CNS effects; barbiturates may decrease effects
Documented hypersensitivity; narrow-angle glaucoma
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Besides extrapyramidal symptoms as with haloperidol, mild leukocytosis, leukopenia, and eosinophilia occasionally occur; dermatologic reactions common; watch for urinary retention, blurred vision, dry mouth, and constipation due to anticholinergic effects
Pimozide (Orap)
Atypical neuroleptic approved by FDA for treatment of tics. Rarely indicated in current practice: offers no substantial advantage over other high-potency neuroleptics (risperidone and olanzapine better tolerated), has significant drug interactions, and slight but serious risk of cardiac arrhythmia.
Adult
1-6 mg PO qhs; manufacturer recommends maximum total daily dosage of 10 mg
Pediatric
0.0 5 mg/kg or 1 mg PO qhs initially; not to exceed 0.2 mg/kg or 10 mg PO qhs
Increases toxicity of MAOIs, alfentanil, CNS depressants, guanabenz
Documented hypersensitivity; history of cardiac arrhythmias or long-QT syndrome; concurrent macrolide antibiotics
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
ECG recommended at initiation of therapy and at regular intervals thereafter; careful observation for extrapyramidal symptoms especially needed in geriatric patients
Presynaptic dopamine-depleting agents
These agents suppress tics. Presynaptic depleters have acute adverse effects similar to neuroleptics but theoretically may avoid risk of tardive dyskinesia.
Tetrabenazine
Investigational drug not approved by US FDA. May be obtained from manufacturer via named patient protocol (see Tetrabenazine).
Adult
25-100 mg PO qhs
Pediatric
Not recommended
Not established
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Investigational drug
Dopamine agonist
These agents suppress tics. Dopamine agonists have few adverse effects and modest but proven efficacy.
Pergolide (Permax)
Pergolide was withdrawn from the US market March 29, 2007, because of heart valve damage resulting in cardiac valve regurgitation. It is important not to abruptly stop pergolide. Health care professionals should assess patients' need for dopamine agonist (DA) therapy and consider alternative treatment. If continued treatment with a DA is needed, another DA should be substituted for pergolide. For more information, see FDA MedWatch Product Safety Alert and Medscape Alerts: Pergolide Withdrawn From US Market.
Mixed ergot derivative dopamine agonist. Proven effective for tic suppression.
Adult
Not established; 0.05-1 mg PO qhs to tid
Pediatric
0.05 mg PO qhs to 0.1 mg PO tid
Dopamine antagonists such as neuroleptics (eg, phenothiazines, butyrophenones, thioxanthenes) or metoclopramide may diminish effectiveness; because pergolide is >90% bound to plasma proteins, caution when administered with other drugs known to affect protein binding
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
May cause valvular heart disease (yearly echocardiograms recommended for patients on chronic therapy); inhibits secretion of prolactin; causes transient rise in serum concentrations of growth hormone and decrease in serum concentrations of luteinizing hormone; adverse effects include nausea, hypotension, hallucinations, and somnolence; use caution in patients who have been treated for cardiac dysrhythmias; may cause or exacerbate preexisting states of confusion and hallucinations or dyskinesia
Alpha2-adrenergic agonists
These agents are used for tic suppression or for treatment of ADHD.
Clonidine (Catapres)
Less effective than neuroleptics in suppressing tics and stimulants at treating ADHD symptoms. However, has modest adverse effects and benefits some patients.
Adult
0.05-0.3 mg PO bid/divided tid or by transdermal patch
Pediatric
0.05-0.1 mg bid/qid PO
TCAs inhibit hypotensive effects; beta-blockers may potentiate bradycardia; TCAs may enhance hypertensive response associated with abrupt withdrawal; hypotensive effects enhanced by narcotic analgesics
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Sedation is most common limiting adverse effect; hypotension can be problematic; do not discontinue suddenly because of risk of rebound hypertension
Guanfacine (Tenex)
Proven in RCT to benefit both ADHD and, to lesser extent, tic severity in children with chronic tics and ADHD.
Adult
0.5-2 mg PO tid
Pediatric
0.5-1 mg PO tid
Increases effect of other hypotensive agents; TCAs may decrease hypotensive effects
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in hepatic impairment, severe coronary insufficiency, recent myocardial infarction; taper dosage gradually
Skeletal Muscle Relaxant
These agents may suppress the severity of tics.
Baclofen (Lioresal)
Slightly superior to placebo in RCT in children with TS, though primary effect may not be on tics but on other symptoms; adverse effects modest.
Adult
10-20 mg PO tid/qid
Pediatric
Administer as in adults
Opiate analgesics, benzodiazepines, alcohol, TCAs, guanabenz, MAOIs, clindamycin, and hypertensive agents may increase effects
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in patients with history of autonomic dysreflexia and when spasticity utilized to increase function; autonomic dysreflexia can result from withdrawal of this medication
Benzodiazepines
By binding to specific receptor sites, these agents appear to potentiate the effects of GABA and facilitate inhibitory GABA neurotransmission and other inhibitory transmitters.
Clonazepam (Klonopin)
Reduces tics in some patients, although blinded controlled studies are lacking. Half-life >30 h, but clinical effect wanes more rapidly.
Adult
0.5-6 mg (possibly up to 12 mg) PO qhs or divided bid
Pediatric
0.5-6 mg PO qhs or divided bid
Phenytoin and barbiturates may reduce effects; CNS depressants increase toxicity (clinically significant pharmacokinetic interactions not common)
Documented hypersensitivity; severe liver disease; acute narrow-angle glaucoma
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution patients about operating hazardous machinery, including automobiles, until they are reasonably certain that therapy does not affect them adversely; class IV controlled substance; most problematic adverse effects are sedation, cognitive difficulties, ataxia, and disinhibition
Neuromuscular blockers
These agents suppression of tics and possibly premonitory sensations.
Botulinum toxin A (BOTOX®)
Inhibits release of acetylcholine at neuromuscular junction; injected directly into muscle. Most useful for dystonic tics (eg, sustained eye closure) or only 1 or 2 especially problematic tics (eg, repeatedly flinging head to 1 side causing neck pain and broken glasses). Tics and tic urges may improve; effect can be seen in absence of gross weakness. Successful outcomes require substantial specialized experience in the treating physician.
Adult
Varies with affected muscle to be injected, from approximately 1.25 U in orbicularis oculi to approximately 200 U total in neck or extremity muscles
Pediatric
Administer as in adults
Aminoglycosides or drugs that interfere with neuromuscular transmission may potentiate effects
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Do not exceed recommended dosages and frequencies of administration; presence of antibodies to botulinum toxin type A may reduce effects
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Further Reading
Keywords
Tourette's syndrome, TS, Gilles de la Tourette's syndrome, GTS, Tourette's disorder, DSM-IV-TR 307.23, Tourette disorder, Gilles de la Tourette syndrome, motor tics (simple and complex), vocal tics (simple and complex), echolalia, palilalia, coprolalia, coprophrasia, copropraxia, echopraxia, stereotypic movement disorder, chronic motor or vocal tic disorder, DSM-IV-TR 307.22, stereotyped motor movement, stereotyped vocalization, transient tic disorder, DSM-IV-TR 307.21, tic disorder not otherwise specified, DSM-IV-TR 307.20, Tourette Syndrome Study Group criteria, TSSG criteria, phonic tic disorder
