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Tic Disorders and Tourette Syndrome in Children and Adolescents

by Margaret C. McBride, MD

Tics are defined as repeated, sudden, rapid, nonrhythmic muscle movements including sounds or vocalizations.

Tics vary widely in severity; they occur in about 20% of children, many of whom are not evaluated or diagnosed. Tourette syndrome, the most severe type, occurs in 3 to 8/1000 children. Male to female ratio is 3:1.

Tics begin before age 18 years (typically between ages 4 and 6 yr); they increase in severity to a peak at about age 10 to 12 yr and decrease during adolescence. Eventually, most tics disappear spontaneously. However, in about 1% of children, tics persist into adulthood.

Etiology is not known, but tic disorders tend to be familial. In some families, they appear in a dominant pattern with incomplete penetrance.

Comorbidities are common. Children with tics may have attention-deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), and/or separation anxiety disorder; these disorders often interfere more with children's development and well-being than the tics. ADHD is the most common comorbidity, and sometimes tics first appear when children with ADHD are treated with a stimulant; these children probably have an underlying tendency to tics. Adolescents and adults may have depression, bipolar disorder, or substance abuse. Comorbidities are common. Children with tics may have attention-deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), and/or separation anxiety disorder; these disorders often interfere more with children's development and well-being than the tics. ADHD is the most common comorbidity, and sometimes tics first appear when children with ADHD are treated with a stimulant; these children probably have an underlying tendency to tics. Adolescents and adults may have depression, bipolar disorder, or substance abuse.

Classification

Tic disorders are divided into 3 categories by the Diagnostic and Statistical Manual of Mental Disorders, 5th edition (DSM-5):

  • Tourette syndrome (Gilles de la Tourette syndrome): Both motor and vocal tics have been present for > 1 year.

  • Persistent (chronic) tic disorder: Single or multiple motor or vocal tics (but not both motor and vocal) have been present for > 1 yr.

  • Provisional tic disorder: Single or multiple motor and/or vocal tics have been present < 1 yr.

In all categories, age at onset must be < 18 yr, and the disturbance cannot be due to physiologic effects of a substance (eg, cocaine) or another disorder (eg, Huntington disease, postviral encephalitis).

Symptoms and Signs

Patients tend to manifest the same set of tics at any given time, although tics tend to vary in type, intensity, and frequency over a period of time. They may occur multiple times in an hour, then remit or barely be present for ≥ 3 mo. Typically, tics do not occur during sleep.

Tics can be motor or vocal and simple or complex (see Types of Tics). Simple tics are a very brief movement or vocalization, typically without social meaning. Complex tics last longer and may involve a combination of simple tics. Complex tics may appear to have social meaning (ie, be recognizable gestures or words) and thus seem intentional. However, although some patients can voluntarily suppress their tics for a short time (seconds to minutes) and some notice a premonitory urge to perform the tic, tics are not voluntary and do not represent misbehavior.

Stress and fatigue can make tics worse, but tics are often most prominent when the body is relaxed, as while watching TV. Tics may lessen when patients are engaged in tasks (eg, school or work activities). Tics rarely interfere with motor coordination. Mild tics often cause few problems, but severe tics, particularly coprolalia (which is rare), are physically and/or socially disabling.

Sometimes tics are explosive in onset, appearing and becoming constant within a day. Sometimes explosive onset of tics and related obsessive compulsiveness may be related to a streptococcal infection—a phenomenon sometimes called pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). Many investigators do not believe that PANDAS is distinct from the spectrum of tic disorders.

Types of Tics

Classification

Motor

Vocal

Simple

Blinking

Grimacing

Head jerking

Shoulder shrugging

Grunting or barking

Sniffing or snorting

Throat clearing

Complex

Combinations of simple tics (eg, head turning plus shoulder shrugging)

Copropraxia: Using sexual or obscene gestures

Echopraxia: Imitating someone's movements

Coprolalia: Uttering socially inappropriate words (eg, obscenities, ethnic slurs)

Echolalia: Repeating one's own or another's sounds or words

Diagnosis

  • Clinical evaluation

Diagnosis is clinical. To differentiate Tourette syndrome from transient tics, physicians may have to monitor patients over time.

Treatment

  • Sometimes clonidine or antipsychotics

  • Treatment of comorbidities

Treatment to suppress tics is recommended only if they are significantly interfering with children’s activities or self-image; treatment does not alter the natural history of the disorder. Often, treatment may be avoided if clinicians help children and their families understand the natural history of tics and if school personnel can help classmates understand the disorder.

Clonidine 0.05 to 0.1 mg po once/day to 4 times/day is effective in some patients. Adverse effects of fatigue may limit daytime dosage; hypotension is uncommon.Antipsychotics may be required—for example, risperidone 0.25 to 1.5 mg po bid, haloperidol 0.5 to 2 mg po bid or tid, pimozide 1 to 2 mg po bid, olanzapine 2.5 to 5 mg po once/day). Fluphenazine is also effective in suppressing tics.

With any drug, the lowest dose required to make tics tolerable is used; doses are tapered as tics wane. Adverse effects of dysphoria, parkinsonism, akathisia, and tardive dyskinesia are rare but may limit use of antipsychotics; using lower daytime doses and higher bedtime doses may decrease adverse effects.

Treating comorbidities is important. ADHD can sometimes be successfully treated with low doses of stimulants without exacerbating tics, but an alternative treatment (eg, atomoxetine) may be preferable. If obsessive or compulsive traits are bothersome, an SSRI may be useful. Children who have tics and who are struggling in school should be evaluated for learning disorders and provided with support as needed.

Key Points

  • Tics are repeated, sudden, rapid, nonrhythmic muscle movements or vocalizations that develop in children < 18 yr old.

  • Tics are common, but the most severe manifestation of tics, coprolalia, is rare.

  • Simple tics are a very brief movement or vocalization (eg, head jerk, grunt), typically without social meaning.

  • Complex tics may appear to have social meaning (ie, be recognizable gestures or words) and thus seem intentional, but they are not.

  • Use of clonidine or an antipsychotic may lessen severe or troublesome tics, which also tend to lessen with time although a few persist into adulthood.

  • Comorbidities (eg, ADHD, OCD) are common and must also be diagnosed and treated.

Resources In This Article

Drugs Mentioned In This Article

  • Drug Name
    Select Trade
  • RISPERDAL
  • HALDOL
  • ORAP
  • STRATTERA
  • ZYPREXA
  • CATAPRES
  • No US brand names

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