Tamara Pringsheim, MD, MScTamara Pringsheim, MD, MSc
For patients with Tourette syndrome and other chronic tic disorders, a treatment approach that includes holistic and behavioral strategies, as well as medications may help improve tic management over time, according to new treatment guidelines from the American Academy of Neurology.1

The guidelines, which are endorsed by the Child Neurology Society and the European Academy of Neurology, were presented at the 2019 American Academy of Neurology Annual Meeting, May 4-10 in Philadelphia.

Notably, the guidelines place strong emphasis on the need for clinicians to educate patients and their caregivers about the natural history of tic disorders.

“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,” said guideline lead author Tamara Pringsheim, MD, MSc, FAAN, of the University of Calgary in Alberta, Canada.2

Tics often begin in childhood and peak around ages 10 to 12 years, with most patients experiencing improvements in adolescence. According to the guidelines, 60% of patients will have minimal or mid tics within 6 years of initial evaluation, with 18% reporting no tics.

In patients who present with tics that do not interfere with their activities of daily living, or cause them emotional or social distress, the guidelines recommend a “watch and wait” approach. However, if patients are open to treatment, clinicians may initiate the use of Comprehensive Behavioral Intervention for Tics (CBIT) as first-line treatment, which utilizes relaxation training, habit-reversal training, and behavioral therapy. There is evidence that supports CBIT’s effectiveness in both children and adults with little to no side effects.

As comorbid behavioral disorders, including attention deficit/hyperactivity disorder (ADHD), obsessive compulsive disorder (OCD), are common in people with tic disorders, assessment and treatment of the behavioral disorders with specifically indicated medications and interventions is essential for the overall management and improvement of the tic disorder.

The guideline includes level B recommendations for the use of alpha-2 adrenergic agonists in patients with comorbid ADHD and tics, including clonidine and guanfacine.

A review of evidence also demonstrated some support for the use of antipsychotics in patients with tic disorders when the benefits of these drugs outweigh the risks. The medications, including haloperidol, risperidone, aripiprazole, and tiapride, as well as pimozide, ziprasidone, and metoclopramide, can be used to reduce tic severity in patients who don’t respond well to or are not indicated for CBIT. Clinicians should counsel patients on the risks for associated side effects of these drugs, including extrapyramidal, hormonal, and metabolic adverse effects, and monitor patients for their occurrence using evidence-based monitoring protocols.

The guidelines also include a level C recommendation for the use of botulinum toxin injections for the treatment of tic disorders, including localized and bothersome motor tics and disabling or aggressive vocal tics.

In patients who don’t respond well to other treatments, clinicians can consider prescribing topiramate at the lowest effective dose.

For those patients who are refractory to behavioral and pharmaceutical interventions, clinicians may consider deep brain stimulation, though there is limited evidence from randomized trials available.

“An important challenge and limitation in the evaluation of the evidence around DBS in TS is that, even in expert DBS centers, few operations per year are performed,” the guideline authors wrote. While there is no consensus on the most appropriate brain targets for treatment of tic disorders, the centromedian thalamus, the globus pallidus internus (ventral and dorsal), the globus pallidus externus, the subthalamic nucleus, and the ventral striatum/ventral capsular nucleus accumbens regions have been targeted with some success.

“Appropriate patient selection is one of the most important predictors of success of DBS treatment, making multidisciplinary evaluation essential,” the authors wrote. “Because of the complexity of the patient population, centers performing DBS have been encouraged to screen candidates preoperatively and to follow them postoperatively.”

Finally, the guideline authors recognize that some patients with tic disorders self-medicate with cannabis in an effort to control their tics and related comorbidities. Since there is limited evidence of effectiveness, clinicians are encouraged to direct patients to appropriate medical supervision when the patient opts to self-medicate with cannabis. Where legislation allows, clinicians may consider prescribing cannabis-based treatment to patients with refractory tic disorders (level C), however this treatment modality should not be prescribed or encouraged in children, women who are pregnant or breastfeeding, or patients with psychosis.

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REFERENCES
1. Pringsheim T, Holler-Managan Y, Okun MS, et al. Practice guideline: The treatment of tics in people with Tourette syndrome and chronic tic disorders. Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. Published online May 6, 2019. Doi: 10.1212/WNL.0000000000007466
https://www.aan.com/Guidelines/Home/GetGuidelineContent/964
2. New AAN guideline for treating tourette syndrome and other chronic tic disorders [news release]. Minneapolis, MN: American Academy of Neurology. May 6, 2019. Accessed May 6, 2019.