How should a 23-year-old female with Tourette syndrome (TS) and recently increased motor and vocal tics be investigated and treated?

Management of Worsening Tics in Adult Tourette Syndrome

For this 23-year-old woman with worsening tics, begin with comprehensive screening for comorbid ADHD and OCD (present in 50-75% and 30-60% of cases respectively), assess functional impairment and quality of life, then initiate behavioral therapy as first-line treatment, reserving pharmacotherapy for cases causing significant psychosocial or functional impairment. 1

Investigation Approach

Clinical Assessment (Diagnosis is Primarily Clinical)

• Document the impact on function and quality of life, as this drives treatment decisions 1
• Confirm DSM-IV-TR criteria are met: multiple motor tics plus at least one vocal tic persisting >1 year with childhood onset 1
• Assess for characteristic features that distinguish tics from other movement disorders:
  • Ability to temporarily suppress tics followed by intensification of premonitory sensation 2
  • Waxing-waning pattern of symptoms 2
  • Variability and suppressibility 2

Essential Comorbidity Screening

• Screen for ADHD (present in 50-75% of Tourette patients) 1, 2
• Screen for OCD or obsessive-compulsive behaviors (present in 30-60%) 1, 2
• Evaluate for depression, anxiety, and emotional instability 3
• These comorbidities often cause greater impairment than tics themselves and may be driving the recent worsening 4, 3

Avoid Excessive Testing

• Excessive medical testing causes iatrogenic harm; diagnosis is clinical 1, 2
• No routine neuroimaging or laboratory studies are indicated unless atypical features suggest secondary causes 1

Treatment Algorithm

First-Line: Behavioral Interventions

• Habit reversal training and exposure-response prevention should be first-line approaches 1
• Comprehensive behavioral intervention for tics (CBIT) has demonstrated efficacy 4, 5
• Requires cooperative patient, presence of premonitory urge, and committed support system 4

Pharmacotherapy Indications

Initiate medication when tics cause: 4

• Psychosocial problems (loss of self-esteem, peer comments, excessive worry, diminished activity participation)
• Functional difficulties or physical discomfort
• Significant quality of life impairment 1

Medication Selection: Two-Tier Approach

First-Tier Agents (Milder Tics)

• Alpha-2 adrenergic agonists (e.g., clonidine) are recommended for milder tics, especially beneficial when ADHD is comorbid 1, 4

Second-Tier Agents (Moderate to Severe Tics)

Atypical Antipsychotics (Preferred):

• Risperidone: Start 0.25 mg daily at bedtime, maximum 2-3 mg daily in divided doses 1

  • Best evidence level among atypical antipsychotics 6
  • Monitor for extrapyramidal symptoms at doses ≥2 mg daily 1
  • Avoid coadministration with QT-prolonging medications 1

• Aripiprazole: Second choice with promising data and low adverse reaction risk 1, 6

• Olanzapine: Initial dose 2.5 mg daily at bedtime 1

  • Diminished extrapyramidal symptom risk compared to typical agents 1

Typical Antipsychotics (Second Choice):

• Pimozide: Best evidence among typical antipsychotics but requires cardiac monitoring due to significant QT prolongation risk 1, 6
• Tiapride: Largest clinical experience in Europe with low adverse reaction rate 6
• Should not be used as first-line due to higher risk of irreversible tardive dyskinesia 1

Management of Comorbidities

If ADHD is comorbid:

• Atomoxetine, stimulants, or clonidine should be considered 6
• If tics are severe, combine stimulants with risperidone 6
• Methylphenidate is preferred over amphetamine-based medications, which may worsen tic severity 1

If OCD/anxiety/depression is comorbid:

• Mild to moderate tics with obsessive-compulsive symptoms: sulpiride monotherapy 6
• More severe cases: combine risperidone with selective serotonin reuptake inhibitor 6

Advanced Treatment (Treatment-Refractory Cases)

• Deep brain stimulation may be considered for severe treatment-refractory cases in patients above 20 years of age 1, 7
• Reserved only after failure of standard pharmacological and behavioral therapies 7
• Targets centromedian-parafascicular thalamus and globus pallidus interna 1
• Approximately 97% of patients show improvement in published studies 7

Critical Pitfalls to Avoid

• Failing to screen for and address comorbid ADHD and OCD, which may be exacerbating tic symptoms 7
• Avoiding benztropine or trihexyphenidyl for managing extrapyramidal symptoms in this population 1
• Starting with typical antipsychotics as first-line due to tardive dyskinesia risk 1
• Misdiagnosing tics as habit behaviors or psychogenic symptoms, leading to inappropriate interventions 1, 2