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Key Features

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Essentials of Diagnosis
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  • 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

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General Considerations
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  • 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

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Clinical Findings

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Symptoms and Signs
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  • 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

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Differential Diagnosis
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  • 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

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Diagnosis

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  • Most patients with tics require no special diagnostic workup

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

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Treatment

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Nonpharmacologic
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  • 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

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Pharmacologic
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  • Most cases of tics can be monitored without treatment

  • Haloperidol and pimozide

    • Only two FDA-approved medications for Tourette syndrome

    • However, given the risk of tardive ...

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