New AAN Guideline on Tourette Syndrome, Tic Disorders

Susan Jeffrey

May 06, 2019

PHILADELPHIA — The American Academy of Neurology (AAN) has released a new guideline on the management of Tourette Syndrome and other chronic tic disorders.

The guideline and the systematic review of the literature that supported their recommendations were published online today in Neurology to coincide with the American Academy of Neurology 2019 Annual Meeting. The document is endorsed by the Child Neurology Society and the European Academy of Neurology. This is the first guideline for American neurologists, the authors say; previous guidelines have been issued in Canada and Europe.

The good news is that after a systematic review of the evidence, authors were able find evidence to support use of several treatment approaches.

"For people living with Tourette syndrome and other chronic tic disorders, an accurate diagnosis, ongoing medical assessment of the severity of tics, as well as treatments that include holistic care, behavioral strategies, and medications based on the latest scientific evidence, could mean a better chance of managing tics over time," coauthor Tamara Pringsheim, MD, associate professor with the Department of Clinical Neurosciences, Psychiatry, Pediatrics and Community Health Sciences at the University of Calgary in Canada, told a press conference here.

The guidelines recommend that when a person "has tics that are not causing physical impairment, pain, emotional distress or social embarrassment, that watching and waiting is an acceptable management strategy when also combined with providing education that helps the person understand and better cope with the disorder," Pringsheim noted.

Treatment decisions should be shared between patient, their caregivers, and the physician, she noted. "The guideline also recommends that doctors tell children with tics and their caregivers that there is a good chance that their symptoms will improve in late adolescence."

Systematic Review and Guideline

For the guideline, the authors considered systematic reviews and randomized controlled trials on the treatment of tics that included at least 20 participants (10 participants if a crossover trial), except for neurostimulation trials, for which no minimum sample size was required. "To obtain additional information on drug safety, we included cohort studies or case series that specifically evaluated adverse drug effects in individuals with tics," they write.

Recommendations include:

  • For people with tics who have access to Comprehensive Behavioral Intervention for Tics (CBIT), clinicians should prescribe CBIT as an initial treatment option relative to other psychosocial/behavioral interventions (Level B), and relative to medication (Level B). "Clinicians may prescribe CBIT delivered over teleconference or secure voice-over-Internet protocol delivery systems if face-to-face options are unavailable in a patient care center," they note. "If CBIT is unavailable, other behavioral interventions for tics may be acceptable, such as exposure and response prevention (Level C)."

"The thing that we're trying to convey with this guideline is the importance of the Comprehensive Behavioral Intervention as a treatment strategy so that more neurologists are aware of it," Pringsheim said. "As physicians, we're used to prescribing medications or medical interventions rather than behavioral therapies, so we want to bring this awareness to neurologists, that they should be recommending this treatment."

CBIT, which runs about 6 to 12 weeks, "combines habit-reversal training, which teaches patients how to control their urges to tic, with other behavioral strategies to reduce stress and other factors that also make tics worse," said John Piacentini, PhD, coauthor of the guideline and professor of psychiatry and biobehavioral sciences in the David Geffen School of Medicine at UCLA and the UCLA Semel Institute, Los Angeles.

CBIT is a relatively new approach, he explained, with efficacy trials just published in the last 10 years, so accessibility is an issue. "We need more providers trained," he told Medscape Medical News. "There are a number of providers and the numbers are growing pretty dramatically working with the Tourette Association; we have a behavior therapy institute where we are trying to train people. The CDC also sponsors some work in this area."

"But there are a lot of areas of the country that don't have easy access to these therapists, and for the therapists who do the work, often times they can be pretty full, because Tourette's is a relatively common disorder," Piacentini said. "So we're working on this issue and we're hoping over the coming decade that we'll be able to get the number of therapists up."

  • There was "moderate confidence" the authors write, that haloperidol, risperidone, aripiprazole, tiapride, clonidine, onabotulinumtoxinA injections, 5-Ling granule, Ningdong granule, and deep brain stimulation of the globus pallidus were "probably more likely than placebo" to reduce tics.

It's common for people with tic disorders to have other neurodevelopmental and psychiatric conditions, Piacentini noted. "The guideline recommends that people with tics are evaluated for attention deficit hyperactivity disorder, obsessive-compulsive disorder, and mood and anxiety disorders, since treatment for these disorders may also be needed," he said. "In some cases, one medication can help lessen the symptoms of both the tic disorder and the coexisting disorder."  

Deep brain stimulation can be considered, but only after multidisciplinary evaluation that includes a psychiatrist or neurologist; a neurosurgeon; and neuropsychologists to establish when benefit outweighs risk for medication-resistant motor and phonic tics, they note; physicians, however, "must confirm" that multiple classes of medications and behavioral therapies have failed or have been contraindicated. 

  • There was "low confidence" that pimozide, ziprasidone, metoclopramide, guanfacine, topiramate, and tetrahydrocannabinol were possibly more likely than placebo to reduce tics.

The authors have several recommendations in this category on the use of cannabis by patients with tic disorders. "Due to the risks associated with cannabis use and widespread self-medication with cannabis for tics, where regional legislation and resources allow, physicians must offer to direct patients to appropriate medical supervision when cannabis is used as self-mediation for tics (Level A)," they write. Also where regional legislation allows, physicians may consider treatment with cannabis-based medication in otherwise treatment-resistant adults with clinically relevant tics (Level C), or adults with Tourette syndrome who already use cannabis efficiently as a self-medication, "in order better control and improve quality of treatment (Level C)."

  • Evidence of harm associated with various treatments was also demonstrated, the authors write, including weight gain, drug-induced movement disorders, elevated prolactin levels, sedation, and effects on heart rate, blood pressure, and ECGs.

"This systematic review summarizes the evidence for efficacy and harms of interventions for the treatment of tics in individuals with TS and chronic tic disorders," the authors conclude. While there is evidence to support the efficacy of several treatments, knowledge gaps remain. Many of the interventions have only been studied in one randomized controlled trial of short duration, with modest sample sizes, they point out.

"The inherent features of tic disorders, with waxing and waning of symptoms over time, placebo effects, as well as suppression of tics during clinical encounters, may confound symptom assessment in clinical trials," they note. "There remains a great need for randomized controlled trials of interventions for tics to further evaluate both long term efficacy and safety."

This practice guideline was developed with financial support from the American Academy of Neurology (AAN). Authors who serve as AAN subcommittee members, methodologists, or who are AAN staff, were reimbursed by the AAN for expenses related to travel to subcommittee meetings where drafts of manuscripts were reviewed. Pringsheim has disclosed no relevant financial relationships. Piacentini has received royalties from Guilford Press and Oxford Press; has received honoraria from Tourette Association of America (TAA), the International OCD Foundation, the TLC Foundation for BFRBs, OCD New Jersey, the University of Michigan, Cairo University, and the University of Oslo; has received financial or material support from Pfizer Pharmaceuticals, Psyadon Pharmaceuticals, the National Institute of Mental Health of the NIH, and from the academic entity of 501C3s, the TAA, and the Pettit Family Foundation. Disclosures for coauthors can be found in the document.

Neurol. Published online May 6, 2019. Guideline, Systematic Review

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