Tics & Tourette Syndrome

Key Features

Essentials of Diagnosis

• Tics are repetitive, rapid, stereotyped, unwanted muscle contractions that involve discrete muscle groups

• Tourette syndrome is characterized by multiple motor tics and at least one phonic tic with onset before age 21 years and occurring for at least 1 year

General Considerations

• The usual age of onset for all tic disorders is 4–8 years (median age 6)

• Familial incidence is 35–50%

• Tics may be triggered by stimulants such as methylphenidate and dextroamphetamine

• No single chromosome/gene defect is causative; many "hot spots" have been identified

• Important comorbidities

  • Attention-deficit/hyperactivity disorder (ADHD)

  • Obsessive-compulsive disorder (OCD)

  • Learning disabilities

  • Migraine (25%)

  • Sleep difficulties

  • Anxiety and mood swings

• Tics may persist into sleep

• Transient tics of childhood (12–24% incidence in school-aged children) last from 1 month to 1 year and seldom need treatment

Clinical Findings

Symptoms and Signs

• Characteristics of tics

  • A premonitory urge ("I had to do it") is unique to tics

  • Quick repetitive but irregular movements, often stereotyped, and briefly suppressible

  • Coordination and muscle tone are not affected

  • Can occur anywhere on the body, but most commonly are found on the head, neck, and upper body

  • Usually come and go over time

• Facial tics include grimaces, twitches, and blinking

• When the trunk and extremities are involved, twisting or flinging movements may be present

• Vocal tics

  • Less common and are highly suggestive of Tourette syndrome

  • Can manifest as grunting, throat clearing, and in complex cases, as utterances of words

• Tourette syndrome

  • Characterized by multiple fluctuating motor and vocal tics with no obvious cause lasting more than 1 year

  • Tics evolve slowly, new ones being added to or replacing old ones

  • Coprolalia and echolalia are relatively infrequent

  • Complex motor tics are coordinated sequenced movements mimicking normal motor acts or gestures; for example, ear scratching, head shaking, twisting, and "giving the finger"

  • Self-injurious behavior is not uncommon

Differential Diagnosis

• Brain injury

• Autism

• Rett syndrome

• Numerous genetic neurodevelopmental disorders

• Postinfectious causes, such as pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections, has been long debated but should be considered if history is suggestive

• Medications and toxins that may induce or worsen tics

  • Amphetamines

  • Cocaine

  • Heroin

  • Methylphenidate

  • Pemoline

  • Antipsychotic antidepressants, older generation antiepileptics, and levodopa

Diagnosis

• Most patients with tics require no special diagnostic workup

• However, if the history suggests secondary causes, targeting neuroimaging or genetic diagnosis may be helpful

Treatment

Nonpharmacologic

• Supportive counseling

• Education of patients, family members, and school personnel

• In some cases, restructuring the school environment to prevent tension and teasing may be necessary

Pharmacologic

• Most cases of tics can be monitored without treatment

• Haloperidol and pimozide

  • Only two FDA-approved ...