Overview
Definition:
Tics are sudden, rapid, recurrent, non-rhythmic motor movements or vocalizations
They are typically classified as transient tic disorder, persistent (chronic) motor or vocal tic disorder, or Tourette disorder
Tics can range from simple (e.g., eye blinking, sniffing) to complex (e.g., touching, jumping, uttering words/phrases).
Epidemiology:
Tics disorders affect approximately 5-10% of school-aged children
Tourette disorder, the most severe form, occurs in about 0.5-1% of children
Males are affected more frequently than females
Onset is typically between ages 5 and 7 years.
Clinical Significance:
Tics can significantly impact a child's social, academic, and emotional well-being
They are often associated with comorbid conditions like ADHD, OCD, anxiety, and learning disabilities, requiring a comprehensive management approach
Understanding pharmacological options is crucial for effective treatment and improving quality of life.
Clinical Presentation
Symptoms:
Sudden, involuntary, recurrent motor tics
Examples: eye blinking, head jerking, shoulder shrugging, grimacing
Sudden, involuntary, recurrent vocal tics
Examples: throat clearing, sniffing, grunting, uttering obscenities (coprolalia, rare)
Pre-monitory urges (a sensory discomfort that is relieved by the tic)
Fluctuations in tic severity, frequency, and type over time
Comorbid symptoms of ADHD (inattention, hyperactivity) or OCD (obsessions, compulsions).
Signs:
Observed involuntary motor movements or vocalizations during physical examination
Assessment of the type, frequency, and complexity of tics
Evaluation for associated neurological signs (rarely present)
Observation of pre-monitory urges
Assessment of functional impairment in social, academic, or occupational settings.
Diagnostic Criteria:
Diagnosis is based on DSM-5 criteria for Tourette Disorder: multiple motor tics and at least one vocal tic present simultaneously for over a year, with onset before age 18
For Persistent (Chronic) Motor or Vocal Tic Disorder: single or multiple motor OR vocal tics, but not both, for over a year, onset before 18
For Provisional Tic Disorder: single or multiple motor AND/OR vocal tics for less than a year, onset before 18
Rule out substance/medication-induced causes or other medical conditions.
Diagnostic Approach
History Taking:
Detailed history of tics: onset, duration, frequency, severity, type (motor/vocal, simple/complex), fluctuations, pre-monitory urges
History of developmental milestones
Screening for comorbid conditions: ADHD, OCD, anxiety, depression, learning disabilities
Family history of tics or related disorders
Impact of tics on daily functioning: school, social interactions, self-esteem
Medications currently used and their potential side effects
Red flags: sudden onset of severe tics, neurological deficits, significant behavioral changes suggesting other primary neurological or psychiatric conditions.
Physical Examination:
Complete neurological examination to rule out other causes of involuntary movements
Focus on cranial nerves, motor strength, coordination, gait, and reflexes
Assess for stereotyped movements
Careful observation for tics during the examination, noting their characteristics
General physical examination to identify any systemic illness
Brief screening for developmental delays.
Investigations:
Typically, no specific laboratory investigations are required for the diagnosis of tic disorders
Neuroimaging (MRI/CT brain) may be considered if there are neurological signs suggesting an underlying structural abnormality, movement disorder, or secondary cause of tics
EEG is rarely useful unless epilepsy is suspected
Genetic testing is not routinely indicated.
Differential Diagnosis:
Chorea (e.g., Huntington's disease, Sydenham's chorea)
Myoclonus
Stereotypies
Habit spasms
Tourette mimic disorders
Tardive dyskinesia
Restless legs syndrome (especially if prominent leg movements)
Seizure disorders
Obsessive-compulsive disorder (distinguishing compulsions from complex tics)
Attention-Deficit/Hyperactivity Disorder (distinguishing hyperactivity from motor tics).
Management
Initial Management:
Psychoeducation for the child and family about tic disorders is paramount
Discuss natural course, potential for remission, and management options
Behavioral interventions are often first-line for mild to moderate tics: Habit Reversal Training (HRT), Comprehensive Behavioral Intervention for Tics (CBIT)
Educate on tic suppression strategies and stress management.
Medical Management:
Pharmacotherapy is indicated for tics that cause significant distress or functional impairment
Alpha-2 Adrenergic Agonists (e.g., Clonidine, Guanfacine) are often first-line for reducing tic severity and are well-tolerated
Doses for children: Clonidine 0.05-0.3 mg PO TID
Guanfacine 0.5-3 mg PO BID
Antipsychotics (e.g., Haloperidol, Risperidone, Aripiprazole, Pimozide) are more potent but have higher risk of side effects (sedation, weight gain, EPS, QTc prolongation)
Doses for children are typically lower than adult doses and initiated with caution: Risperidone 0.25-1 mg PO BID
Aripiprazole 0.5-2 mg PO QD
Consider dopamine depleters like Tetrabenazine for severe, refractory tics (less common in pediatrics).
Surgical Management:
Deep Brain Stimulation (DBS) is a last resort for severe, medically refractory Tourette disorder in adolescents and adults, not typically considered in younger children
Other ablative neurosurgical procedures are rarely performed and investigational.
Supportive Care:
Addressing comorbid conditions (ADHD, OCD, anxiety) with appropriate pharmacological or behavioral interventions
School accommodations to minimize academic and social challenges related to tics
Support groups for children and families
Regular follow-up to monitor tic severity, functional impact, medication efficacy, and side effects.
Alpha 2 Agonists Vs Antipsychotics
Alpha 2 Agonists Advantages:
Generally well-tolerated, fewer serious side effects compared to antipsychotics
Effective for tics, often also help with comorbid ADHD symptoms
Can be initiated at lower doses and titrated gradually
Available as oral and transdermal formulations (clonidine patch).
Alpha 2 Agonists Disadvantages:
May have sedating effects, particularly at higher doses
Can cause dry mouth and hypotension
May be less effective for severe, complex tics compared to antipsychotics
Efficacy can wane over time for some individuals.
Antipsychotics Advantages:
Potent tic suppressants, particularly effective for severe and disabling tics
Can be highly effective when alpha-2 agonists fail or are insufficient
Some atypical antipsychotics (e.g., aripiprazole) have a more favorable side effect profile than older typical agents.
Antipsychotics Disadvantages:
Significant potential for side effects: sedation, weight gain, metabolic syndrome, extrapyramidal symptoms (EPS), hyperprolactinemia, QTc prolongation (especially with haloperidol/pimozide)
Requires careful monitoring for efficacy and adverse events
Not typically first-line due to risk profile.
Selection Criteria:
Choice depends on tic severity, functional impairment, presence of comorbidities, patient's age, and prior treatment response
Mild-moderate tics with comorbid ADHD symptoms may benefit from alpha-2 agonists
Severe, disabling tics refractory to behavioral therapy and alpha-2 agonists may warrant a trial of antipsychotics under strict supervision.
Complications
Early Complications:
Medication side effects: sedation, dizziness, dry mouth, hypotension (alpha-2 agonists)
weight gain, metabolic disturbances, EPS, sedation (antipsychotics).
Late Complications:
Worsening of comorbidities (ADHD, OCD) if not adequately managed
Social isolation, bullying, and poor academic performance due to severe tics
Long-term effects of chronic medication use (e.g., metabolic syndrome with antipsychotics)
Potential for medication non-adherence due to side effects or perceived lack of efficacy.
Prevention Strategies:
Judicious selection of medication based on individual patient profile
Start low, go slow dosing strategy
Thorough patient and family education regarding potential side effects and monitoring
Regular follow-up appointments to assess efficacy and tolerability
Prompt management of comorbidities
Promoting positive self-esteem and coping mechanisms.
Prognosis
Factors Affecting Prognosis:
Severity of tics at onset
Presence and severity of comorbidities (especially ADHD and OCD)
Family history of tics
Response to behavioral and pharmacological interventions
Social support system
Degree of functional impairment
The natural course of Tourette disorder involves improvement in many individuals during adolescence, though some may continue to experience significant tics into adulthood.
Outcomes:
With appropriate management, most children can achieve significant reduction in tic severity and functional impairment
For many, tics may significantly improve or even remit
However, some individuals may experience persistent, disabling tics
Comorbid conditions can significantly impact overall outcomes.
Follow Up:
Regular follow-up visits are essential, especially during initiation or titration of medications
Frequency of follow-up will depend on the severity of tics, presence of comorbidities, and medication regimen
Typically, every 3-6 months for stable patients, or more frequently if there are concerns about efficacy, side effects, or new symptoms
Long-term monitoring for medication effects and the natural history of the disorder is crucial.
Key Points
Exam Focus:
Differentiating tic disorders from other movement disorders
Understanding DSM-5 criteria for Tourette and other tic disorders
First-line management options (behavioral therapy)
Indications for pharmacotherapy
Key drugs, typical doses, and major side effect profiles for alpha-2 agonists (clonidine, guanfacine) and antipsychotics (risperidone, aripiprazole) in pediatrics
Management of comorbidities like ADHD and OCD.
Clinical Pearls:
Always start with behavioral interventions if tics are not severely impairing
Alpha-2 agonists are often the first-line pharmacological choice due to their favorable safety profile and efficacy for mild-moderate tics and comorbid ADHD
Antipsychotics are reserved for severe, disabling tics refractory to other treatments, with careful monitoring for serious side effects
Remember pre-monitory urges as a key characteristic of tics
Tics fluctuate
assess severity over a period rather than a single encounter.
Common Mistakes:
Misdiagnosing tics as willful misbehavior or attention-seeking
Prescribing antipsychotics as a first-line agent without considering behavioral therapies or alpha-2 agonists
Inadequate monitoring for medication side effects, especially metabolic changes and EPS with antipsychotics
Neglecting the management of comorbid conditions, which significantly impacts overall outcome
Focusing solely on tic suppression without addressing functional impairment and quality of life.