Tourette Syndrome

Tourette syndrome is a neurodevelopmental disorder with multiple motor tics and at least one vocal tic for over one year, onset before age 18.

• Prevalence 0.3 to 1%; boys 3 to 4 times more affected; onset at 4 to 8 years, peak severity at 10 to 12, with improvement in adulthood in many.

• Comorbidities: ADHD (50%), obsessive compulsive disorder (30 to 50%), anxiety, depression, autism, self injury, rage attacks.

• Complex genetic inheritance; dopaminergic and cortico striato thalamocortical circuit involvement.

• Motor tics (eye blinking, head jerks, shrugs), vocal tics (throat clearing, sniffing, grunts, coprolalia in under 10%).

• Premonitory urge preceding tics and brief suppressibility; waxing and waning course; worsening with stress and excitement.

• Distress, bullying, academic impairment.

• Clinical (DSM-5): multiple motor and at least one vocal tic over 1 year, onset before 18, not due to substances or other medical conditions.

• Yale Global Tic Severity Scale.

• Screen for ADHD, OCD, anxiety, depression, learning difficulties.

• Investigations only for atypical features (adult onset, abrupt onset, regression): MRI, EEG, copper, drug review.

• Psychoeducation for patient, family and school.

• Behavioural therapy: full behavioural intervention for tics (CBIT) or habit reversal training first line for those with impairment.

• Medication if tics cause distress or functional impairment: alpha 2 agonists (clonidine 0.05 to 0.3 mg/day, guanfacine 1 to 4 mg/day) first line, especially with ADHD.

• Antipsychotics: aripiprazole 2 to 10 mg daily (first line), risperidone 0.5 to 3 mg, haloperidol 0.5 to 2 mg, pimozide 1 to 4 mg (ECG for QTc).

• Tetrabenazine 12.5 to 50 mg, topiramate 50 to 200 mg, botulinum toxin for focal tics.

• Treat comorbid ADHD (stimulants safe with tics, atomoxetine) and OCD (SSRI with CBT).

• Deep brain stimulation of the thalamus or globus pallidus for severe refractory adult disease.