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Evidence-Based Reviews


Children with tic disorders: How to match treatment with symptoms

Algorithm helps determine when behavioral therapy, medication is appropriate

Vol. 9, No. 3 / March 2010

Discuss this article

Sammy, age 7, is referred to you by his pediatrician because of a 4-week history of frequent eye blinking. His parents say he blinks a lot when bored but very little when playing baseball. They recall that he also has intermittently sniffed and nodded his head over the last 12 months. Neither Sammy nor his friends seem to be bothered by the blinking. Except for the tics, Sammy’s physical and mental status exams are normal.

Since preschool, Sammy’s teachers have complained that his backpack and desk are always a mess. Sammy is well-meaning but forgetful in his chores at home. A paternal uncle has head-turning movements, counts his steps, and becomes distressed if books on his shelf are not in alphabetical order.

Tics, such as strong eye blinks or repetitive shoulder shrugs, can distress a child or his/her parents, but the conditions associated with tic disorders often are more problematic than the tic disorder itself. High rates of comorbid conditions are recognized in persons with Tourette syndrome, including:

  • obsessive-compulsive disorder (OCD) in >80%1
  • attention-deficit/hyperactivity disorder (ADHD) in ≤70%2
  • anxiety disorders in 30%3
  • rage, aggression, learning disabilities, and autism less commonly.

The strategy we recommend for managing tic disorders includes assessing tic severity, educating the family about the illness, determining whether a comorbid condition is present, and managing these conditions appropriately. Above all, we emphasize a risk-benefit analysis guided by the Hippocratic principle of “do no harm.”

Characteristics of tic disorders

You diagnose Sammy with Tourette syndrome because he meets DSM-IV-TR criteria of at least 2 motor tics and 1 vocal tic that have persisted for 1 year without more than a 3-month hiatus, with tic onset before age 18. Because tics may resemble other movement disorders, you rule out stereotypies, dystonia, chorea, ballism, and myoclonus (Table 1). You explain to his parents that Sammy’s condition is a heritable, neurobehavioral disorder that typically begins in childhood and is associated in families with OCD, ADHD, and autism spectrum disorders.

His parents ask about the difference between tics and other movements. You explain that eye-blinking tics—like other motor tics—appear as sudden, repetitive, stereotyped, nonrhythmic movements that involve discrete muscle groups. (View a video of a patient with tics.) Simple motor tics are focal movements involving 1 group of muscles, whereas complex tics are sequential patterns of movement that involve >1 muscle group or resemble purposeful movements (Table 2).

Table 1

Features of 5 movement disorders that may resemble tics

Tics

Stereotypies

Dystonia

Chorea

Ballism

Myoclonus

Sudden, repetitive, stereotyped, nonrhythmic movements or sounds

Patterned, nonpurposeful movement

Cocontraction of agonist and antagonist muscles, causing an abnormal twisting posture

Continuous, flowing, nonrhythmic, nonpurposeful movement

Forceful, flinging, large amplitude choreic movement

Sudden, quick, shock-like movement

Usually start after age 3

Usually start before age 3 and resolve by adolescence

More common in adults

Decrease when focused; increase when stressed, anxious, fatigued, or bored

Occur when the child is excited

Worsens during motor tasks

Worsens during motor tasks

Worsens during motor tasks

Comorbid conditions include OCD and ADHD

Common in children with mental retardation or autism

Can occur after streptococcal infection

Can occur after streptococcal infection

Preceded by a premonitory urge or sensation

Possibly preceded by an urge

Not preceded by an urge

Not preceded by an urge

Not preceded by an urge

Not preceded by an urge

Temporarily suppressible

Suppressible

Not suppressible

Partially suppressible; can incorporate into semi-purposeful movements

Partially suppressible

Not suppressible

ADHD: attention-deficit/hyperactivity disorder; OCD: obsessive-compulsive disorder

Table 2

Characteristics of simple and complex motor and vocal tics*

Simple tics

Complex tics

Eye blinking or eye rolling
Nose, mouth, tongue, or facial grimaces (nose twitch, nasal flaring,
chewing lip, teeth grinding, sticking out tongue, mouth stretching, lip licking)
Head jerks or movements (neck stretching,
touching chin to shoulder)
Shoulder jerks/movements (shoulder shrugging, jerking a shoulder)
Arm or hand movements (flexing or extending
arms or fingers)
Coughing
Throat clearing, grunting
Sniffing, snorting, shouting
Humming

Jumping
Spinning
Touching objects or people
Throwing objects
Repeating others’ action (echopraxia)
Obscene gestures (copropraxia)
Repeating one’s own words (palilalia)
Repeating what someone else said (echolalia)
Obscene, inappropriate words (coprolalia)

*Simple tics are focal movements involving 1 group of muscles; complex tics are sequential patterns of movement that involve >1 muscle group or resemble purposeful movements

Older children frequently describe a premonitory urge prior to the tic. Patients typically can suppress tics for a transient period of time, although during tic suppression they usually feel restless and anticipate performing their tic. The ultimate performance of the tic brings relief. Tic suppression also occurs during focused activity. Emotional stress, fatigue, illness, or boredom can exacerbate tics.

To begin monitoring Sammy’s clinical course, you administer 3 assessment tools described inTable 3. You explain to Sammy’s parents that these tests will be repeated yearly or when tics worsen. However, you tell his parents that these scores alone will not determine present or future clinical decisions, including treatments. You also recommend that they connect with support groups on the Tourette Syndrome Association (TSA) Web site.

CASE CONTINUED: Changes over time

Sammy’s parents appreciate your explanation and say they will share information from the TSA Web site with Sammy’s principal, teachers, and classmates. The family agrees to return in 6 months or sooner if the tics worsen.

By age 8, Sammy develops multiple tics: facial grimacing, looking upwards, punching movements, whistling, and throat clearing. He is slightly bothered by these tics, and his friends have asked him about them. He tells them he has Tourette syndrome, and that usually ends the questioning. He returns for a follow-up visit because his parents notice a dramatic increase in his tics after Sammy’s father loses his job.

Treatment options

When deciding to treat a child’s tics, the first step is to determine whether to pursue a nonpharmacologic or pharmacologic approach (Algorithm). To tailor an approach most suited for an individual child, discuss with the family their feelings about therapy and medications. This information—along with tic severity—will help determine a treatment plan.

Behavior therapy and medication are management strategies; neither can cure a tic disorder. The most conservative approach to tic treatment is to:

  • provide the child and family with basic guidelines for managing tics
  • help alleviate environmental stress and other potential triggers.

Algorithm: Recommended treatment of tics in children and adolescents


CASE CONTINUED: A first intervention

You discuss treatment options with Sammy’s family, and they view medication as a last resort. Sammy does not seem to be bothered by his tics, and his parents do not wish to start him on daily medications. Given this situation, habit reversal therapy (HRT) is appropriate for Sammy because he is old enough to participate in HRT to reduce his tics.

HRT is an effective nonpharmacologic approach to help children with tics.4 Its 3 components are:

  • awareness training
  • competing response training
  • social support.5

This simplified version of the original HRT can be completed in eight 1-hour sessions. Good candidates are patients who are cognitively mature enough to understand the therapy’s goals and compliant with frequent clinic visits. They also must practice the strategies at home.

It should not be difficult for psychiatrists to learn HRT—or refer to therapists who are willing to learn it—with the available instructional manual.

CASE CONTINUED: Practicing alternatives

You ask Sammy to imitate his tics. After helping him become more aware of his tics, you encourage him to develop a more socially appropriate movement to engage in whenever he feels the urge to punch. Sammy chooses to clench his fist in his pocket. He also learns to breathe in whenever he has an urge to whistle. you advise Sammy’s parents to reward his efforts to suppress the tics. He practices the strategies daily.

At age 12, Sammy returns to your office. He has begun to have frequent neck-jerking tics, which cause neck pain and daily headaches. He also is slapping his thigh and having frequent vocal tics characterized by loud shrieking. The vocal tics are disruptive in class, even though Sammy sits toward the back of the room. Sammy’s classmates tease him, and he is very frustrated.

Medication approach

The decision to start a medication for tics is complex. Scores from the YGTSS, PUTS, and GTS-QOL scales (Table 3) provide only a partial clinical picture. This decision should be reached after a detailed discussion with the family about benefits and risks of medications and ensuring that everyone’s expectations are reasonable.

A variety of medications are available to treat patients with tics (Table 4). No medication can completely eliminate tics, however, and many have substantial side effects. Before initiating medical treatment, consider 3 questions:

  • Is moderate or severe pain involved?
  • Is there significant functional interference?
  • Is there significant social disruption despite efforts to optimize the social environment for the child?

Sammy’s frequent neck-jerking tics now cause chronic daily headaches, and his shrieking vocal tics are interfering with classroom activities, so we recommended a 3-month trial of guanfacine following the dosing schedule in Table 4.

Table 3

3 scales for assessing tic severity and impact on functioning

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

Continued...
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