What is Tourette syndrome?

From the Guidelines

Tourette syndrome should be treated with a combination of behavioral therapy and medication, with alpha-2 adrenergic agonists or antipsychotics such as pimozide being considered as first-line treatment options, as they have been shown to be effective in controlling tics 1.

Key Considerations

• The diagnosis of Tourette syndrome is based on the presence of repetitive, involuntary movements and vocalizations called tics, and must be differentiated from other conditions such as habit cough or psychogenic cough 1.
• Behavioral therapy, including Comprehensive Behavioral Intervention for Tics (CBIT) and habit reversal training, can be effective in teaching patients to recognize premonitory urges and develop competing responses.
• Medication options include alpha-2 adrenergic agonists like clonidine or guanfacine, which are often tried first, especially in children with mild to moderate tics.
• For more severe cases, antipsychotics such as risperidone, aripiprazole, or pimozide may be prescribed, with pimozide being shown to be superior to haloperidol in efficacy and side effects 1.

Treatment Approach

• Treatment should be individualized, as Tourette's often co-occurs with conditions like ADHD, OCD, or anxiety that may require additional management.
• Patients should be monitored for medication side effects, which can include sedation, weight gain, or movement disorders with antipsychotics.
• Support groups and education about the condition are also important components of comprehensive management.

Important Considerations

• Tics often improve during adolescence, but some patients require ongoing treatment into adulthood.
• The ability of patients with Tourette syndrome to suppress their tics helps to differentiate tics from other hyperkinetic movement disorders such as chorea and dystonia 1.
• Double-blind, placebo-controlled trials have shown that dopamine receptor-blocking drugs, such as pimozide, are effective in controlling the tics of Tourette syndrome 1.

From the Research

Definition and Characteristics of Tourette Syndrome

• Tourette syndrome is a childhood onset neurodevelopmental disorder characterized by multiple motor and vocal tics 2
• The majority of patients also have one or more psychiatric comorbid disorders, such as attention deficit hyperactivity disorder and/or obsessive-compulsive disorder 2

Treatment Options for Tourette Syndrome

• Treatment is multimodal, including both pharmacotherapy and cognitive behavioral treatment, and requires disentanglement of tics and the comorbid symptoms 2
• The α-adrenoceptor agonists guanfacine and clonidine have an established evidence base for both efficacy and tolerability, and are usually recommended as initial pharmacotherapy 2, 3
• Atypical neuroleptics, such as aripiprazole or risperidone, are typically used if the α-adrenoceptor agonists are ineffective or intolerable 2
• Behavioral therapies, such as Comprehensive Behavioral Intervention for Tics (CBIT) and habit reversal training (HRT), are recommended as a first-line intervention for Tourette syndrome and persistent motor or phonic tic disorder 4, 5, 3

Efficacy of Treatment Options

• CBIT has demonstrated acute and durable efficacy when delivered alone or in combination with medication, in person, or via telehealth, and in the presence or absence of common comorbid conditions 4
• There is high-quality evidence for face-to-face one-on-one treatment with CBIT, and data suggesting that one-on-one treatment by videoconference provides similar benefit 5
• Antipsychotic drugs have been shown to improve tic scores, but certainty in the effect estimate is low and they carry the risk of harm 3
• Botulinum toxin injections can be used in patients with bothersome focal tics, and deep brain stimulation may be considered for patients with tics that are refractory to conventional treatments 6