Overview
Definition:
Chronic tic disorder (CTD) is a neurological disorder characterized by the presence of motor and/or vocal tics that persist for more than one year, beginning before age 18
Tics are sudden, rapid, recurrent, non-rhythmic motor movements or vocalizations
Comprehensive Behavioral Intervention (CBI) is the first-line treatment for chronic tic disorders.
Epidemiology:
The prevalence of chronic tic disorders, including Tourette Syndrome, varies but is estimated to be around 0.5-1.9% in school-aged children
Boys are more commonly affected than girls
Tics often appear between ages 5-7 and can fluctuate in severity.
Clinical Significance:
CTD can significantly impact a child's social, academic, and emotional well-being
Untreated or poorly managed tics can lead to bullying, social isolation, anxiety, depression, and academic difficulties
Effective CBI empowers patients and families with strategies to manage tics and improve quality of life, making it a crucial component of pediatric care and essential knowledge for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Sudden, involuntary, repetitive motor movements (e.g., blinking, head jerking, shoulder shrugging, facial grimacing)
Sudden, involuntary, repetitive sounds or vocalizations (e.g., throat clearing, sniffing, grunting, barking)
Tics can be simple (single muscle group/sound) or complex (sequences of movements/sounds)
Premonitory urges (uncomfortable sensation preceding a tic) are common
Tics worsen with stress, fatigue, excitement, and can be suppressed temporarily
Often associated with ADHD and OCD.
Signs:
Observable repetitive motor movements and/or vocalizations during physical examination
Tics may be observed in various body parts (face, neck, torso, limbs) or manifest as vocalizations
Examiner should note the type, frequency, complexity, and impact of tics
Observe for involuntary nature and potential suppression.
Diagnostic Criteria:
Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5) criteria for Tourette Disorder or Persistent (Chronic) Motor or Vocal Tic Disorder: 1
Single or multiple motor tics AND at least one vocal tic have been present at some time during the illness, though not necessarily concurrently
2
Tics have occurred many times a day and nearly every day for more than 1 year, or at intervals, but with the tic-free period between occurrences not longer than 3 months
3
Onset before age 18 years
4
The disturbance is not attributable to the direct physiological effects of a substance (e.g., stimulant medication) or another medical condition (e.g., Huntington's disease, postviral encephalitis)
5
A diagnosis is based on clinical history and direct observation of tics, and no other cause is found.
Diagnostic Approach
History Taking:
Detailed history of tic onset, type, frequency, severity, and variability
Duration of tics (must be > 1 year)
Age of onset (<18 years)
Presence of premonitory urges
History of exacerbating/remitting factors (stress, fatigue, illness)
Associated comorbidities like ADHD, OCD, anxiety, depression
Family history of tics or related disorders
Impact of tics on daily functioning (school, social, emotional)
Rule out other medical causes (e.g., medications, infections, neurological conditions).
Physical Examination:
Thorough neurological examination to rule out underlying neurological conditions
Assess for other movement disorders
General physical examination to identify any associated medical issues
Observe for tics during the examination and note their characteristics
Examination should be conducted in a calm environment
observe for natural tic expression.
Investigations:
Generally, no specific laboratory investigations are required for diagnosing uncomplicated chronic tic disorder
Investigations are reserved for cases where an organic cause is suspected
These might include: Neuroimaging (MRI brain) to rule out structural abnormalities if neurological signs are present or if there is a suspicion of secondary tics
EEG if epilepsy is suspected
Blood tests to rule out metabolic or infectious causes if indicated by clinical presentation.
Differential Diagnosis:
Other movement disorders (e.g., chorea, dystonia, myoclonus)
Stereotypies (repetitive, rhythmic movements often seen in developmental disabilities)
Tardive dyskinesia (drug-induced movement disorder)
Obsessive-compulsive disorder (OCD) – distinguish repetitive behaviors from true tics
Attention-Deficit/Hyperactivity Disorder (ADHD) – often comorbid, but tics are distinct from ADHD symptoms
Psychogenic non-epileptic seizures (PNES)
Stereotypic movement disorder
Huntington's disease
Wilson's disease
Sydenham's chorea.
Management
Initial Management:
Education and reassurance for the patient and family about the nature of the disorder and its typical course
Psychoeducation is paramount
Emphasize that tics are involuntary
Discuss management strategies, focusing on CBI as the first-line approach
Address any comorbid conditions like ADHD or OCD promptly, as their treatment can sometimes indirectly reduce tic severity
Avoid stigmatizing language.
Comprehensive Behavioral Intervention:
Comprehensive Behavioral Intervention for Tics (CBIT) is the gold standard
It typically involves: 1
Psychoeducation: Educating the patient and family about tics, their triggers, and management strategies
2
Awareness Training: Helping the patient become more aware of their premonitory urges and tics
3
Competing Response Training (CRT): Teaching the patient to perform a specific, voluntary motor or vocal behavior that is incompatible with the tic when the premonitory urge is felt
This aims to replace the tic with a less disruptive behavior
4
Functional Intervention: Identifying environmental factors that may exacerbate tics and developing strategies to modify them
This is often delivered by trained therapists (psychologists, occupational therapists).
Pharmacological Management:
Medication is generally considered when tics are severe, cause significant distress, or interfere with functioning, and after CBI has been tried or is not feasible
Options include: Alpha-adrenergic agonists (e.g., Clonidine, Guanfacine) – generally considered first-line due to better safety profile, particularly in children
Dopamine receptor blockers (e.g., Haloperidol, Risperidone, Aripiprazole) – more effective but associated with greater side effects (sedation, weight gain, extrapyramidal symptoms)
Other agents like Topiramate may be used off-label
Medication choice depends on tic severity, associated comorbidities, side effect profile, and patient/family preference.
Supportive Care:
School accommodations: Working with schools to implement strategies such as allowing breaks, providing a supportive classroom environment, and educating teachers
Social skills training: To help children cope with social challenges and potential stigma
Parental support and training: Empowering parents with strategies to manage tics at home and support their child
Psychological support: Addressing anxiety, depression, or self-esteem issues that may arise
Regular follow-up to monitor tic severity, treatment efficacy, side effects, and adjust management plan as needed.
Complications
Early Complications:
Social isolation and peer rejection
Bullying
Academic difficulties due to distraction
Frustration and low self-esteem
Worsening of tics due to anxiety
Minor injuries from repetitive movements.
Late Complications:
Chronic anxiety and depression
Development of comorbid psychiatric disorders
Persistent impact on educational and vocational opportunities
Significant impact on quality of life and interpersonal relationships
Development of other movement disorders (rare).
Prevention Strategies:
Early and effective implementation of CBI
Prompt management of comorbidities (ADHD, OCD)
Providing a supportive and understanding environment at home and school
Educating peers and teachers about CTD
Promoting self-advocacy skills in children
Regular psychological support for emotional well-being
Avoiding stigmatization and focusing on strengths.
Prognosis
Factors Affecting Prognosis:
Severity and complexity of tics at onset
Presence and severity of comorbid conditions (ADHD, OCD)
Family history of tic disorders
Response to initial treatment, particularly CBI
Socioeconomic and environmental support systems
Age at onset
earlier onset may be associated with longer duration of tics.
Outcomes:
Many children experience a reduction in tic severity with age, with some experiencing remission in adolescence or adulthood
However, a significant proportion continue to have bothersome tics throughout life
With effective CBI and management of comorbidities, individuals can achieve good functional outcomes and lead fulfilling lives
Quality of life is a key indicator of successful management.
Follow Up:
Regular follow-up appointments are crucial to monitor tic trajectory, assess treatment effectiveness, manage side effects of medications, and address any emerging comorbidities or psychosocial issues
Frequency of follow-up depends on the severity of tics and the treatment plan, typically ranging from every 3-6 months, or more frequently if initiating or adjusting medications
Long-term follow-up may be necessary for individuals with persistent and severe tics.
Key Points
Exam Focus:
CBIT is the first-line treatment for CTD
DSM-5 criteria for diagnosis
Common comorbidities: ADHD, OCD
Medications: Alpha-adrenergic agonists (Clonidine, Guanfacine) and dopamine blockers (Haloperidol, Risperidone)
Differentiate tics from stereotypies and other movement disorders
Importance of psychoeducation.
Clinical Pearls:
Always inquire about premonitory urges – their presence is a hallmark of tics
Tics often wax and wane
avoid overreacting to temporary increases in severity
Empower families with tools and strategies through CBI rather than solely relying on medication
Consider the impact of school environment and social factors on tic management
Remember that a significant portion of patients with Tourette syndrome also have ADHD and/or OCD.
Common Mistakes:
Diagnosing CTD without ruling out secondary causes of tics
Relying solely on medication without considering behavioral interventions
Underestimating the impact of comorbidities on tic severity
Dismissing tics as "just a habit" or behavioral problem
Inadequate psychoeducation for the patient and family, leading to non-adherence to treatment.