What is Tourette's syndrome?

What is Tourette Syndrome?

Tourette syndrome is a childhood-onset neurodevelopmental disorder characterized by the presence of multiple motor tics and at least one vocal (phonic) tic persisting for at least 1 year, with onset before adulthood. 1

Core Clinical Features

Tic Characteristics:

• Simple motor tics include eye blinking, facial grimacing, head jerking, and shoulder shrugging 1, 2
• Simple phonic tics include throat clearing, sniffing, grunting, and other basic sounds 1, 2
• Tics are suppressible temporarily, followed by intensification of the premonitory sensation (an uncomfortable urge that precedes the tic) 2
• Tics demonstrate a waxing-waning pattern with variability in frequency and intensity over time 2

Epidemiology and Demographics

• Boys are affected more commonly than girls, with a prevalence of approximately 1 per 1,000 male children 1, 2
• Overall population prevalence is 0.3-1% 3
• Nearly half of patients experience spontaneous remission by age 18 1

Essential Comorbidities

The majority of patients with Tourette syndrome have comorbid conditions that often cause more functional impairment than the tics themselves:

• Attention deficit hyperactivity disorder (ADHD) is present in 50-75% of children with Tourette's 1, 2, 4
• Obsessive-compulsive disorder (OCD) or behaviors are present in 30-60% of children with Tourette's 1, 2, 4
• Approximately 90% of patients have at least one comorbid neuropsychiatric disorder 5

Diagnostic Approach

Diagnosis is primarily clinical and requires:

• Fulfillment of DSM criteria: multiple motor tics AND at least one vocal tic 1, 2
• Duration of at least 1 year 1
• Onset in childhood 1
• Comprehensive neurological, neuropsychiatric, and neuropsychological assessment by a multidisciplinary team including a neurologist, psychiatrist, and clinically qualified psychologist 1

Critical Differential Diagnoses

Avoid misdiagnosing the following conditions:

• Transient tic disorder is more common (4-24% of elementary school children) and resolves within one year 1, 2
• Chronic vocal tic disorder (isolated vocal tics without motor tics) 2
• Habit behaviors or psychogenic symptoms 1, 2

Important Clinical Pitfalls

• Excessive medical testing can cause iatrogenic harm; diagnosis is primarily clinical 1, 2
• Misdiagnosing tics as habit behaviors or psychogenic symptoms leads to inappropriate interventions 1, 2
• Failing to screen for and address comorbidities (ADHD, OCD) which may cause more impairment than the tics themselves 4

Treatment Framework

First-line approaches:

• Behavioral techniques such as habit reversal training and exposure and response prevention should be first-line approaches 1
• Watchful waiting is reasonable in milder cases given the high rate of spontaneous remission 1

Pharmacological options when treatment is needed:

• Alpha-2 adrenergic agonists (clonidine, guanfacine) are preferred first-line medications, particularly when comorbid ADHD or sleep disorders are present 1
• Anti-dopaminergic drugs (haloperidol, pimozide, risperidone, aripiprazole) are effective but have more concerning side-effect profiles 1, 6, 3

Advanced interventions:

• Deep brain stimulation is reserved exclusively for severe, treatment-refractory cases with significant functional impairment in patients above 20 years of age 1, 4
• Treatment-refractory status requires failure of behavioral techniques AND therapeutic doses of at least three proven medications 1