The following article is part of conference coverage from the 2019 American Academy of Neurology Annual Meeting (AAN 2019) in Philadelphia, PA. Neurology Advisor’s staff will be reporting breaking news associated with research conducted by leading experts in neurology. Check back for the latest news from AAN 2019.
PHILADELPHIA — An expert subcommittee of the American Academy of Neurology (AAN) has released an updated evidence-based clinical practice guideline on treating Tourette syndrome and other chronic tic disorders. The guideline, endorsed by the European Academy of Neurology and the Child Neurology Society, was published in Neurology.
Recommendation 1: Counseling on Natural History of Tourette Syndrome
Clinicians are advised to give patients and families information about the disorder to help guide treatment decisions. After evaluating the level of functional impairment, clinicians should inform patients and caregivers that watchful waiting is the appropriate response for individuals not experiencing functional impairment from their tics. For patients motivated to start treatment in this population, cognitive behavioral intervention for tics (CBIT) can be prescribed as an initial treatment relative to watchful waiting. Clinicians who prescribe medications for tics that are causing functional impairments must periodically re-evaluate the need for ongoing treatment.
Recommendation 2: Psychoeducation, Teacher, and Classroom
Tourette syndrome affects approximately 1% of children and psychoeducation about the disorder with a patient’s peers and teachers can result in more positive classroom outcomes. Clinicians should refer patients to psychoeducation resources that can be offered at schools, such as the Tourette Association of America.
Recommendation 3: Assessment and Treatment of ADHD in Children with Tics
ADHD is a common comorbidity in patients with Tourette syndrome (30% to 50%). Clinicians should perform an ADHD assessment and evaluate the symptom burden in patients with both disorders. Appropriate ADHD treatment should be provided for patients with both tics and functionally impairing ADHD. Clinical trials have shown atomoxetine does not worsen tics compared with placebo and reduces ADHD symptoms, and clonidine, clonidine plus methylphenidate, methylphenidate, and guanfacine are more likely to reduce tic severity and ADHD symptoms compared with placebo.
Recommendation 4: Assessment and Treatment of OCD in Children with Tics
Obsessive-compulsive disorder (OCD) is a common comorbidity in patients with Tourette syndrome (10% to 50%). Clinicians should perform an assessment for OCD and provide appropriate treatment when warranted. Trials of OCD interventions for children suggest that patients with tics may not respond to selective serotonin reuptake inhibitors as well as those without tics, but do respond equally well to cognitive behavioral therapy (CBT) for the symptoms of OCD.
Recommendation 5: Other Psychiatric Comorbidities
Patients with Tourette syndrome are at high risk for psychiatric comorbidities such as mood disorders, oppositional defiant disorder, and anxiety disorders, and patients with comorbidities run an increased risk for suicide. Appropriate screenings must be performed, and treatments provided. Clinicians should also inquire about suicidal ideations and actions and refer patients to appropriate resources if needed.
Recommendation 6: Tic Severity Assessment and Treatment Expectations
Using one of the available rating scales, such as the Yale Global Tic Severity Scale, clinicians should measure tic severity in order to be able to assess treatment effects. Patients should be advised that while behavioral therapy, medications, and neurostimulation can significantly reduce tics, they rarely lead to complete cessation.
Recommendation 7: Behavioral Treatments
Patients receiving CBIT are more likely to experience reduced tick severity than those receiving psychoeducation and supportive therapy. After an 8-session protocol, most patients age 9 and older who have a positive response will maintain these gains for at least 6 months. CBIT can be effective in younger patients, but there is little evidence relating to efficacy. If face-to-face CBIT is unavailable, clinicians may offer sessions via teleconference or online delivery. Other acceptable behavioral interventions include exposure and response prevention when CBIT is unavailable.
Recommendation 8: α-Agonists for the Treatment of Tics
Clinicians should counsel patients with comorbid ADHD that α2 adrenergic agonists can be beneficial for both conditions but there are common side effects, such as sedation. Clinicians should only prescribe α2 adrenergic agonists when the benefits outweigh the risks and patients taking these medications should have their heart rate and blood pressure monitored. Patients taking guanfacine extended release must have the QTc interval monitored if they have a history of cardiac conditions, are taking other QT-prolonging agents, or have a family history of long QT syndrome. Clinicians discontinuing treatment with α2 adrenergic agonists must gradually taper the medication to avoid rebound hypertension.
Recommendation 9: Antipsychotic Treatment for Tics
When the benefits outweigh the risks, antipsychotics may be prescribed for tics at the lowest effective dose. Patients should be counseled on the potential adverse hormonal, extrapyramidal, and metabolic effects to inform decision-making. Clinicians should monitor patients taking antipsychotics for these adverse effects, and if discontinuing, gradually taper dosage over weeks to months to prevent withdrawal dyskinesias.
Recommendation 10: Botulinum Toxin Injections for Tics
When the benefits outweigh the risks, injections with onabotulinumtoxinA botulinum toxin may be prescribed to adults and adolescents with bothersome, localized, simple motor tics and aggressive or severely disabling vocal tics. Patients should be counseled that all effects are temporary, and the injections may cause hypophonia and weakness.
Recommendation 11: Topiramate for the Treatment of Tics
When the benefits outweigh the risks, topiramate can be prescribed, once patients have been counseled on the common adverse effects, which include cognitive and language problems, weight loss, somnolence, and increased risk for kidney stones.
Recommendation 12: Cannabis-based Medications
Some patients with Tourette syndrome self-medicate with cannabis. In places where legislation allows, clinicians should direct self-medicating patients and patients with treatment-resistant and relevant tics to medically supervised cannabis treatments at the lowest effective dose. When prescribing cannabis-based medications, physicians should periodically re-evaluate the need for ongoing treatment. Patients should be advised that the medication impairs driving ability and clinicians should periodically reevaluate the need for continuing treatment. Cannabis-based medications should not be prescribed to children or adolescents, both due to a lack of evidence regarding efficacy and to associations with potentially harmful affective and cognitive outcomes in adulthood. Likewise, women who are pregnant or breastfeeding, and patients with psychosis should not take cannabis-based medications.
Recommendation 13: Deep Brain Stimulation (DBS)
Patients with severe, treatment-resistant Tourette syndrome may benefit from DBS, although treatment availability can be limited and there is limited clinical trial evidence available for analysis and interpretation. To determine if the benefits of DBS will outweigh the risks, clinicians must perform a multidisciplinary evaluation (psychiatrist or neurologist, neurosurgeon, and neuropsychologist). The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition Tourette syndrome diagnosis must be confirmed and secondary and functional tic-like movements excluded. Clinicians must confirm that behavioral therapy and multiple pharmacological treatments have been attempted or are contraindicated. Preoperative and postoperative screening for psychiatric disorders must be performed by mental health professionals. A mental health professional must screen patients preoperatively and continue to follow-up postoperatively for psychiatric disorders. Clinicians may consider DBS for patients with severe and self-injurious tics like severe cervical tics that can result in spinal injury.
The lead guideline author states, “Tourette syndrome and other chronic tic disorders can be of great concern to the person diagnosed and their family, so it is important that doctors let those affected know that tics may improve with time. Treatments can help decrease tic frequency and severity, but they rarely eliminate all tics. It is important that people are informed of all the available treatment options, which include education, behavioral therapies, medication, or watchful waiting.”
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Pringsheim T, Okun MS, Muller-Vahl K, et al. Practice guideline recommendations summary: treatment of tics in people with Tourette syndrome and chronic tic disorders [published online May 6, 2019]. Neurology doi:10.1212/WNL.0000000000007466