Overview

Definition:
-Tourette Syndrome (TS) is a neurodevelopmental disorder characterized by multiple motor tics and at least one vocal tic that have been present for more than a year
-Obsessive-Compulsive Disorder (OCD) is an anxiety disorder characterized by unwanted, intrusive thoughts (obsessions) that lead to repetitive behaviors or mental acts (compulsions)
-Comorbidity between TS and OCD is common, with studies indicating a significant overlap in genetic and neurobiological pathways.
Epidemiology:
-The prevalence of TS is estimated to be around 1 in 160 children aged 5-17 years in the US
-OCD affects approximately 1-3% of children and adolescents
-The comorbidity rate between TS and OCD is high, with estimates ranging from 30% to 50% of individuals with TS also meeting criteria for OCD, and a substantial proportion of individuals with OCD having a history of tics.
Clinical Significance:
-The co-occurrence of TS and OCD presents unique challenges in diagnosis and management
-Untreated or inadequately treated comorbidity can lead to significant functional impairment, decreased quality of life, and increased risk of psychiatric complications
-Effective therapy sequencing is crucial for optimizing outcomes and improving patient well-being.

Clinical Presentation

Symptoms:
-Motor tics: sudden, rapid, recurrent, nonrhythmic movements
-Simple motor tics include blinking, shrugging, grimacing
-Complex motor tics involve purposeful-seeming movements like touching, jumping, or uttering specific words or phrases
-Vocal tics: sudden, brief, repetitive sounds
-Simple vocal tics include sniffing, throat clearing, grunting
-Complex vocal tics include words or phrases, echolalia, or coprolalia (involuntary shouting of obscenities)
-Obsessions: recurrent and persistent thoughts, urges, or images that are experienced as intrusive and unwanted
-Common themes include contamination, symmetry, aggressive or horrific impulses, and forbidden thoughts
-Compulsions: repetitive behaviors or mental acts that the individual feels driven to perform in response to an obsession or according to rigid rules
-Examples include excessive washing, ordering, checking, counting, or mental rituals.
Signs:
-Observed tics during physical or neurological examination
-presence of repetitive, seemingly ritualistic behaviors
-signs of anxiety or distress related to obsessions
-impaired social functioning or academic performance due to tics and compulsions.
Diagnostic Criteria:
-Diagnosis of TS is based on DSM-5 criteria: multiple motor tics and at least one vocal tic present simultaneously for more than 1 year, onset before age 18, not attributable to the physiological effects of a substance or another medical condition
-Diagnosis of OCD is based on DSM-5 criteria: presence of obsessions, compulsions, or both
-obsessions/compulsions are time-consuming or cause clinically significant distress or impairment
-not attributable to the physiological effects of a substance or another medical condition
-not better explained by another mental disorder.

Diagnostic Approach

History Taking:
-Detailed history of tic onset, type, frequency, severity, and impact on daily functioning
-Inquire about onset and nature of obsessions and compulsions, triggers, and associated distress
-Screen for comorbid conditions like ADHD, anxiety disorders, depression
-Family history of TS, OCD, or other neurodevelopmental/psychiatric disorders is important
-Rule out medical causes of tics (e.g., post-infectious autoimmune disorders like PANDAS/PANS, metabolic disorders, drug-induced tics).
Physical Examination:
-Complete neurological examination to rule out focal deficits or other neurological signs
-General physical examination to assess for any underlying medical conditions
-Observation for the presence and characteristics of tics and compulsions during the interview.
Investigations:
-Generally, investigations are not required to diagnose TS or OCD
-However, if a secondary cause for tics or OCD symptoms is suspected, further investigations may be warranted: Neuroimaging (MRI brain) to rule out structural lesions if focal neurological deficits are present
-Blood tests (e.g., thyroid function tests, autoimmune markers if PANDAS/PANS is suspected)
-Genetic testing is generally not indicated for routine diagnosis
-Neuropsychological testing may be useful for assessing comorbid ADHD or learning disabilities.
Differential Diagnosis:
-Other tic disorders (persistent motor tic disorder, persistent vocal tic disorder)
-stereotyped movement disorders
-chorea
-myoclonus
-paroxysmal dyskinesias
-other anxiety disorders
-schizophrenia (for delusions/hallucinations)
-For OCD, consider generalized anxiety disorder, social anxiety disorder, hoarding disorder, trichotillomania, excoriation disorder.

Management

Initial Management:
-Psychoeducation for the patient and family about TS and OCD, their chronic nature, and available treatments
-Emphasize that tics can wax and wane and that OCD can be managed
-A comprehensive assessment of functional impairment is essential to guide treatment intensity.
Medical Management:
-For Tics: Alpha-adrenergic agonists (clonidine, guanfacine) are first-line options for mild to moderate tics
-Dopamine receptor antagonists (haloperidol, risperidone, aripiprazole) are effective for severe tics but carry a risk of side effects
-For OCD: Selective Serotonin Reuptake Inhibitors (SSRIs) are first-line pharmacotherapy
-Common SSRIs used in children include fluoxetine, sertraline, fluvoxamine, and citalopram
-Doses are typically higher for OCD than for depression
-Clomipramine, a tricyclic antidepressant with SSRI activity, is also highly effective but may have more side effects.
Behavioral Therapy:
-Comprehensive Behavioral Intervention for Tics (CBIT) is the first-line treatment for disruptive tics
-It includes habit reversal training and awareness training
-Exposure and Response Prevention (ERP) is the gold standard behavioral therapy for OCD
-It involves gradually exposing the individual to their feared situations or thoughts (exposure) while preventing them from engaging in their compulsive behaviors (response prevention).
Therapy Sequencing And Integration:
-The choice of initial therapy and sequencing depends on the severity and primary symptom burden
-If tics are more prominent and significantly impairing, starting with CBIT or alpha-agonists may be considered
-If OCD symptoms are more severe and causing significant distress or functional impairment, starting with ERP or an SSRI may be prioritized
-Often, a combined approach is most effective
-For example: 1
-If both TS and OCD are severe: Begin with ERP for OCD and consider a low-dose SSRI for OCD symptoms
-Concurrently, initiate CBIT for tics
-If tics remain bothersome, consider an alpha-agonist
-If OCD is refractory to SSRI or tics are extremely severe, augmentation with other agents or referral to specialized centers may be needed
-2
-If tics are the primary concern: Initiate CBIT
-If OCD symptoms are mild or subclinical, monitor closely
-If OCD emerges or worsens, introduce ERP and/or an SSRI
-3
-If OCD is the primary concern: Initiate ERP and/or an SSRI
-If tics emerge or worsen, introduce CBIT and/or an alpha-agonist
-Careful monitoring for medication side effects, particularly worsening tics with SSRIs or mood changes, is critical
-The goal is to address both symptom clusters simultaneously or sequentially based on individual presentation and response.
Supportive Care:
-Family counseling and support groups
-Educational support (e.g., 504 plans in schools) to accommodate tics and compulsive behaviors
-Regular follow-up with a multidisciplinary team including child psychiatrists, neurologists, therapists, and social workers.

Complications

Early Complications: Social isolation, bullying, academic difficulties, self-injurious behaviors (e.g., head banging, nail biting), increased anxiety and depression, sleep disturbances.
Late Complications: Chronic functional impairment, significant impact on vocational and social development, increased risk of substance abuse and personality disorders, persistent psychiatric comorbidities.
Prevention Strategies:
-Early identification and intervention
-Consistent and evidence-based treatment for both TS and OCD
-Psychoeducation and skill-building for patients and families
-School-based interventions and support
-Addressing comorbid conditions promptly.

Prognosis

Factors Affecting Prognosis:
-Severity and age of onset of TS and OCD
-Presence and severity of comorbid conditions (ADHD, depression, anxiety)
-Response to treatment
-Family support and engagement
-Access to specialized care.
Outcomes:
-With appropriate and integrated therapy, many individuals can achieve significant reduction in tic severity and frequency, and substantial improvement in OCD symptoms, leading to improved functioning and quality of life
-Symptoms may wax and wane over time
-While complete remission may not always be achieved, effective management can lead to long-term stability.
Follow Up:
-Regular monitoring by a qualified clinician is essential, especially during titration of medications and behavioral therapies
-Follow-up frequency will vary based on treatment response and symptom stability, typically ranging from monthly to quarterly visits
-Long-term follow-up is often necessary to manage fluctuating symptoms and address emerging issues.

Key Points

Exam Focus:
-Remember that comorbid TS and OCD is common
-Therapy sequencing is crucial: ERP/SSRI for OCD, CBIT for tics
-Alpha-agonists are first-line for tics, SSRIs for OCD
-Always consider PANDAS/PANS in the differential
-Monitor for worsening tics with SSRIs.
Clinical Pearls:
-Start behavioral interventions early
-they are often as effective as or more effective than medications for milder symptoms
-Integrate behavioral and pharmacological approaches for best outcomes
-Always involve parents/caregivers in treatment planning and execution
-Recognize that tic suppression can be exhausting and may increase anxiety.
Common Mistakes:
-Treating only one condition while ignoring the other
-Underestimating the severity of OCD in patients with TS
-Prescribing SSRIs without adequate monitoring for tic exacerbation
-Inadequate psychoeducation of families about the chronic and fluctuating nature of these disorders
-Relying solely on pharmacotherapy without incorporating behavioral interventions.