What is the recommended treatment approach for Tourette's syndrome (TS) in individuals with fetal alcohol syndrome (FAS)?

Treatment Approach for Tourette's Syndrome in Fetal Alcohol Syndrome

For individuals with Tourette's syndrome and fetal alcohol syndrome, a structured approach beginning with behavioral therapies is recommended, with medication management as second-line therapy, and deep brain stimulation reserved for severe refractory cases.

Initial Assessment and Diagnosis

• Confirm both diagnoses:

  • Tourette's syndrome: Multiple motor and vocal tics present for >1 year
  • Fetal alcohol syndrome: Document all three dysmorphic facial features (smooth philtrum, thin vermillion border, small palpebral fissures), growth deficits, and CNS abnormalities 1

• Evaluate for common comorbidities:

  • Attention deficit hyperactivity disorder
  • Obsessive-compulsive behaviors
  • Anxiety
  • Depression
  • Learning disabilities (particularly common in FAS)

First-Line Treatment: Behavioral Therapies

1. Habit Reversal Training (HRT)

   • Most evidence-supported behavioral intervention for tics 2
   • Teaches patients to recognize premonitory urges and develop competing responses
   • Should be implemented before pharmacological interventions

2. Comprehensive Behavioral Intervention for Tics (CBIT)

   • Combines HRT with relaxation training and functional interventions 3
   • Effective in reducing tic severity compared to supportive psychotherapy

3. Cognitive-Behavioral Therapy (CBT)

   • Particularly helpful for addressing comorbid anxiety and behavioral issues 4
   • Can be delivered via telehealth when in-person services are limited 3

Second-Line Treatment: Pharmacotherapy

When behavioral therapies are insufficient or unavailable, consider medication:

1. First-line medications:

   • Alpha-2 adrenergic agonists (clonidine, guanfacine)
   • Topiramate
   • Vesicular monoamine transport type 2 inhibitors 5

2. Second-line medications:

   • Antipsychotics (fluphenazine, aripiprazole, risperidone)
   • Use with caution due to risk of metabolic syndrome, tardive dyskinesia 5
   • May require lower starting doses in FAS patients due to potential CNS sensitivity

Third-Line Treatment: Deep Brain Stimulation

For severe, treatment-resistant cases:

• Consider DBS when:

  • Patient has failed at least three adequate medication trials 6
  • Symptoms cause severe functional impairment affecting quality of life
  • Patient is generally above 20 years of age 1, 6
  • Psychiatric comorbidities are stabilized for at least 6 months 6

• Target selection:

  • Common targets include centromedian-parafascicular (CM-Pf) nuclei of thalamus and globus pallidus interna (GPi) 1, 7
  • Evidence shows approximately 97% of patients experience tic improvement 1

Special Considerations for FAS Patients

• Patients with FAS may have additional cognitive and behavioral challenges requiring:

  • Simpler, more concrete instructions during behavioral therapy
  • More frequent reinforcement
  • Structured environment with consistent routines
  • Lower medication starting doses with careful titration
  • Close monitoring for side effects

• FAS-specific services should address:

  • Communication and social skills
  • Emotional regulation
  • Language usage and comprehension abilities 1

Monitoring and Follow-up

• Regular assessment of tic severity using standardized scales
• Monitoring for medication side effects
• Evaluation of impact on quality of life and daily functioning
• Adjustment of treatment plan based on response

Pitfalls to Avoid

• Focusing solely on tic management without addressing FAS-related cognitive and behavioral issues
• Rushing to pharmacotherapy before adequate trials of behavioral interventions
• Overlooking the need for educational and social support services
• Failing to consider the patient's cognitive level when implementing behavioral strategies
• Using standard medication dosing without considering potential CNS sensitivity in FAS