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Tourette's Syndrome

High EvidenceUpdated: 2025-12-24

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Red Flags

  • Sudden onset severe tics after Strep throat (PANDAS?)
  • Tics appearing in adulthood (Huntington's / Drug induced?)
  • Dystonia or Ataxia (Not Tourette's)
Overview

Tourette's Syndrome

1. Clinical Overview

Summary

Tourette's Syndrome (TS) is a childhood-onset neurodevelopmental disorder characterized by Multiple Motor Tics and at least one Vocal Tic, persisting for more than 1 year. Tics are sudden, rapid, recurrent, non-rhythmic movements or vocalizations. The condition waxes and wanes. It is often comorbid with ADHD and OCD (the "Tourette's Triad"). [1,2]

Key Facts

  • Evolution: Tics typically start with simple motor tics (blinking) around age 5-7. Vocal tics appear later (age 11). Peak severity is early adolescence (10-12). Improvement often occurs in late adolescence.
  • Premonitory Urge: 90% of patients describe an uncomfortable sensation (itch, tension, pressure) that builds up before the tic. Performing the tic relieves this urge. This is a crucial differentiator from other movement disorders (like chorea or myoclonus) which are involuntary and unexpected.
  • Coprolalia: Involuntary swearing. Famous in media, but actually Rare (less than 10% of cases). Most vocal tics are simple grunts, coughs, or sniffing.

Clinical Pearls

Suppressibility: Unlike seizures or dystonia, tics are "unvoluntary" rather than involuntary. Patients can often suppress them temporarily (e.g., in the doctor's office or at school), often leading to a "rebound explosion" of tics when they get home.

The "Wax and Wane": It is normal for tics to change character. A child may have sniffing for 3 months, then that disappears and is replaced by shoulder shrugging. This fluctuation confirms the diagnosis.

PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections): A controversial entity where tics/OCD explode suddenly after a Strep throat infection. Test for Strep/ASOT if onset is explosive.


2. Epidemiology

Demographics

  • Prevalence: ~1% of school-age children.
  • Gender: Males > Females (4:1).
  • Genetics: Highly heritable, but polygenic (no single gene).

Comorbidities

  • ADHD: 60% of cases.
  • OCD: 50% of cases.
  • Anxiety/Mood Disorders: Common.
  • Note: Often the comorbidities cause more impairment than the tics themselves.

3. Pathophysiology

Mechanism

  • Cortico-Striato-Thalamo-Cortical (CSTC) Circuits: Dysfunction in the "brakes" of the brain.
  • Dopamine Hypothesis: Excess dopamine activity or receptor sensitivity in the basal ganglia (striatum). This explains why dopamine antagonists (antipsychotics) work.

4. Clinical Presentation

Motor Tics

Vocal Tics

Temporal Course


Simple
Eye blinking (most common first sign), grimacing, shoulder shrugging, head jerking.
Complex
Jumping, touching objects, twirling, echopraxia (copying movements).
5. Clinical Examination
  • Observation: Watch the child while talking to the parents. (Tics often vanish when staring directly at the child).
  • Neurological Exam: Should be normal.
    • Rule out: Kayser-Fleischer rings (Wilson's), Chorea (Sydenham's/Huntington's), Ataxia.

6. Investigations

Diagnosis

  • Clinical: Based on history. No blood test or scan confirms TS.

Exclude Mimics

  • MRI Brain: Only if atypical signs (e.g. onset age >18, neurological deficits).
  • Copper studies: Wilson's disease.
  • ASOT/Throat swab: If PANDAS suspected.

7. Management

Management Algorithm

           DIAGNOSIS OF TOURETTE'S
      (Motor + Vocal Tics >1y, Onset less than 18)
                    ↓
          ASSESS IMPAIRMENT
    (Are tics causing pain? Social stigma?
     Or is it just parental anxiety?)
                    ↓
        ┌───────────┴───────────┐
     MILD/NONE             MODERATE/SEVERE
  (Not bothered)         (Distress/Pain)
        ↓                       ↓
  PSYCHOEDUCATION         BEHAVIOURAL RX
  (Reassure family)       (First Line)
  (Inform School)         - CBIT
                          - Habit Reversal
                                ↓
                          PHARMACOTHERAPY
                          (Second Line)
                          - Alpha Agonists
                          - Antipsychotics

1. Psychoeducation

  • Crucial. Explain it is biological, not "bad behaviour".
  • Liaise with school (allow exam breaks, ignore tics).

2. Behavioural Therapy (First Line)

  • CBIT (Comprehensive Behavioural Intervention for Tics).
  • Habit Reversal Training: Identify the premonitory urge -> Perform a "Competing Response" (e.g. if urge is to jerk neck, tense the neck muscles symmetrically) until urge passes.

3. Pharmacotherapy

  • Alpha-2 Agonists: Clonidine / Guanfacine. (Useful if ADHD comorbid). Side effects: Sedation, hypotension.
  • Antipsychotics (Dopamine antagonists):
    • Aripiprazole: First choice (partial agonist, lower metabolic risk).
    • Risperidone / Haloperidol: Effective but high side effect profile (Weight gain, EPS).
    • VMAT2 Inhibitors: Tetrabenazine (depletes dopamine).
  • Botox: For single focal motor tics (e.g. severe neck jerking).

8. Complications
  • Physical: Cervical radiculopathy (from neck tics), self-injury.
  • Social: Bullying, isolation.
  • Academic: Disruption in class.

9. Prognosis and Outcomes
  • Rule of Thirds:
    • 1/3 disappear in adulthood.
    • 1/3 improve significantly.
    • 1/3 persist.
  • Tics usually peak at 10-12 years and decline by 18.

10. Evidence and Guidelines

Key Guidelines

GuidelineOrganisationKey Recommendations
Tic DisordersESSTS (European)Behavioural therapy (CBIT) is first line over drugs.
Tourette'sAAN (American Academy of Neuro)Discuss "watch and wait". Screen for ADHD/OCD.

Landmark Evidence

1. Piacentini et al (JAMA)

  • Large RCT showed that CBIT is significantly more effective than supportive therapy, with effects lasting >6 months. This firmly established behavioural therapy as the first-line standard instead of jumping to antipsychotics.

11. Patient and Layperson Explanation

What is Tourette's?

It is a condition where the brain sends extra "signals" to the muscles, causing sudden twitches (tics) or sounds. It's like having a hiccup - you might be able to hold it in for a moment, but eventually, it has to come out.

Why does he sweat?

People with Tourette's almost never swear (only 1 in 10). Most just blink, shrug, or make grunting noises.

Will he grow out of it?

Most likely. It tends to be worst around age 10-12 and gets much better as the brain matures in the late teens.

Is he doing it for attention?

No. In fact, most kids try very hard to hide it, which is exhausting. When they are relaxed at home, the tics often come out more because they stop holding them in.


12. References

Primary Sources

  1. Roessner V, et al. European clinical guidelines for Tourette syndrome and other tic disorders. Eur Child Adolesc Psychiatry. 2011.
  2. Pringsheim T, et al. Practice guideline recommendations summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders. Neurology. 2019. (AAN Guideline).
  3. Piacentini J, et al. Behavior therapy for children with Tourette disorder: a randomized controlled trial. JAMA. 2010.

13. Examination Focus

Common Exam Questions

  1. Diagnosis: "Definition?"
    • Answer: Motor + Vocal tics, >1 year duration, onset less than 18y.
  2. Comorbidity: "Most common associated conditions?"
    • Answer: ADHD and OCD.
  3. Treatment: "First line therapy?"
    • Answer: CBIT (Behavioural).
  4. Symptom: "Coprolalia?"
    • Answer: Involuntary swearing.

Viva Points

  • Differential Diagnosis: Sydenham's Chorea (Rheumatic fever - dancing hand movements, vanishing grip). Wilson's Disease. Drug-induced (Stimulants for ADHD can sometimes unmask tics, though recent evidence suggests therapeutic doses are usually safe).

Medical Disclaimer: MedVellum content is for educational purposes and clinical reference. Clinical decisions should account for individual patient circumstances. Always consult appropriate specialists.

Last updated: 2025-12-24

At a Glance

EvidenceHigh
Last Updated2025-12-24

Red Flags

  • Sudden onset severe tics after Strep throat (PANDAS?)
  • Tics appearing in adulthood (Huntington's / Drug induced?)
  • Dystonia or Ataxia (Not Tourette's)

Clinical Pearls

  • Perform a "Competing Response" (e.g. if urge is to jerk neck, tense the neck muscles symmetrically) until urge passes.

Guidelines

  • NICE Guidelines
  • BTS Guidelines
  • RCUK Guidelines