| Instrument | Purpose | Description | Design | Administration frequency |
|---|---|---|---|---|
| Yale Global Tic Severity Scale (YGTSS) | Assess tic severity | Review of motor and vocal tics. Rate number, frequency, intensity, complexity, and interference on a 5-point scale | Clinician-rated | Annual and as needed for increased tics |
| Premonitory Urge for Tics Scale (PUTS) | Detect the presence of unpleasant sensations that precedes tics | 10 questions | Self-report | Annual and as needed for increased tics |
| Gilles de la Tourette Syndrome Quality of Life Scale (GTS-QOL) | Measure quality of life | 27 questions, 4 subscales: psychological, physical, obsessional, and cognitive | Self-report | Annual and as needed for increased tics |
Table 4
Medications with evidence of tic-suppressing effects*
| Category A evidence | ||
|---|---|---|
| Medication | Starting dose | Target dose |
| Haloperidol | 0.25 to 0.5 mg/d | 1 to 4 mg/d |
| Pimozide | 0.5 to 1 mg/d | 2 to 8 mg/d |
| Risperidone | 0.25 to 0.5 mg/d | 1 to 3 mg/d |
| Category B evidence | ||
| Medication | Starting dose | Target dose |
| Fluphenazine | 0.5 to 1 mg/d | 1.5 to 10 mg/d |
| Ziprasidone | 5 to 10 mg/d | 10 to 80 mg/d |
| Clonidine | 0.025 to 0.05 mg/d | 0.1 to 0.3 mg/d |
| Guanfacine | 0.5 to 1 mg/d | 1 to 3 mg/d |
| Botulinum toxin | 30 to 300 units | |
| Category C evidence | ||
| Medication | Starting dose | Target dose |
| Olanzapine | 2.5 to 5 mg/d | 2.5 to 12.5 mg/d |
| Tetrabenazine | 25 mg/d | 37.5 to 150 mg/d |
| Baclofen | 10 mg/d | 40 to 60 mg/d |
| Nicotine patch | 7 mg/d | 7 to 21 mg/d |
| Mecamylamine | 2.5 mg/d | 2.5 to 7.5 mg/d |
| Flutamide | 250 mg/d | 750 mg/d |
| *Category A: supported by ≥2 placebo-controlled trials; category B: supported by 1 placebo-controlled trial; category C: supported by open-label study | ||
| Source: Reference 6 | ||
The first-line pharmacologic agent for tic suppression generally is an alpha-adrenergic medication, unless the tics are severe.6
Clonidine and guanfacine usually are started at low doses and increased gradually. Although not as effective as neuroleptics, alpha-adrenergics have a lower potential for side effects and are easier to use because no laboratory tests need to be monitored. Adverse effects associated with alpha-adrenergic medications include sedation, dry mouth, dizziness, headache, and rebound hypertension if discontinued abruptly.
If tics are causing pain, some clinicians prefer conservative measures such as heat or ice, massage, analgesics, relaxation therapy, and reassurance.
Second-line agents include typical and atypical antipsychotics. Haloperidol and pimozide have shown efficacy in reducing tics in placebo- controlled studies,7,8 as have risperidone (in 4 randomized controlled trials [RCTs]) and ziprasidone (in 1 RCT).9,10 The emergence of serious side effects is a risk for both typical and atypical antipsychotics (Table 5).
Table 5
Potential adverse effects of antipsychotic treatment in children*
| Adverse effect | Examples |
|---|---|
| Sedation | — |
| Acute dystonic reactions | Oculogyric crisis, torticollis |
| Appetite changes | Weight gain |
| Endocrine abnormalities | Amenorrhea, diabetes, galactorrhea, gynecomastia, hyperprolactinemia |
| Cognitive effects | Impaired concentration |
| Akathisia | Difficulty sitting still |
| ECG changes | Prolonged QT interval |
| Parkinsonism | Tremor, bradykinesia, rigidity, postural instability |
| Tardive syndrome | Orofacial dyskinesia, chorea, dystonia, myoclonus, tics |
| Neuroleptic malignant syndrome | Potentially fatal; consists of muscular rigidity, fever, autonomic dysfunction, labile blood pressure, sweating, urinary incontinence, fluctuating level of consciousness, leukocytosis, elevated serum creatine kinase |
| *Potential adverse effects are listed from most to least likely to occur | |
As part of your informed consent discussion, weigh the risk of side effects against the benefits of treatment. Point out to patients and their families that they can expect to see a decrease in tic frequency, but symptoms will not necessarily disappear with any medication. We tell our patients that with antipsychotics the best we can hope for is to reduce tic frequency by approximately one-half.6
When treating tics, start with 1 medication. However, if the tics are severe enough to require more than 1 medication, check for drug interactions.
Third-line agents. Agents that have not been tested in placebo-controlled trials can be considered third line; these are listed as category C (supported by open-label studies) in Table 4. Botulinum toxin injection has been found to be effective for motor and vocal tics.11,12 Botulinum toxin and implantation of deep brain stimulators13 are invasive options and generally are reserved for severe, treatment-resistant tics.
CASE CONTINUED: Managing antipsychotics
After trying guanfacine for 12 weeks, Sammy notices no tic reduction. His parents consent to a low dose of risperidone. you review with them the American Psychiatric Association (APA)/American Diabetes Association (ADA) guidelines14 for managing metabolic problems in patients treated with atypical antipsychotics.
As instructed in the APA/ADA guidelines, obtain baseline measurements and monitor for metabolic effects of antipsychotic therapy over time (Table 6). Sammy starts risperidone at 0.5 mg once daily. After 2 weeks, he notices a decrease in his tics. At the 3-month visit after starting risperidone, he is happy with his risperidone dose and does not want to increase it. He has gained 3 pounds, and you instruct him to eat a well-balanced diet and exercise routinely. At the 6-month visit, his tics are minimal and his weight has stabilized.
Table 6
Children receiving antipsychotics: monitoring recommendations