Article In Brief
The lead author of the first AAN practice guideline on managing Tourette syndrome highlights what the evidence suggests about therapies that are both effective and ineffective.
The Centers for Disease Control and Prevention estimates that one in every 162 children (0.6 percent) has Tourette syndrome (TS), with about half of them undiagnosed. But little has been published to assess the evidence for treatment—until now. On May 6, the American Academy of Neurology (AAN) issued its first-ever practice guideline for the treatment of tics in people with TS and chronic tic disorders in the online edition of Neurology.
The guideline is based on a comprehensive review of the evidence conducted by a multidisciplinary panel consisting of nine physicians, two psychologists, and two patient representatives, who followed an Institute of Medicine-compliant process to ensure transparency and patient engagement.
For insights into the key points of the new guideline, Neurology Today spoke with lead author Tamara Pringsheim, MD, FAAN, associate professor in the department of clinical neurosciences, psychiatry, pediatrics and community health sciences at the Cumming School of Medicine of the University of Calgary in Alberta, Canada. Dr. Pringsheim is the director of the Calgary Tourette and Pediatric Movement Disorder Clinic at the Alberta Children's Hospital.
Why was this guideline needed now?
This is the first AAN guideline on the treatment of tics for TS. It was identified as a gap in our offerings. The AAN guideline committee wishes to serve AAN members by providing guidelines on topics that are useful and applicable to their clinical practice. We have a formal process at the AAN through which topics are nominated and the committee felt this was an important topic to be addressed.
What are the most important take-home messages from the guideline for clinicians?
One key point is that many people with TS and tic disorders have psychiatric comorbidities. It's a disorder that's rarely seen in isolation. The vast majority of patients will have a co-occurring disorder, and it's often those disorders that have greater impact on quality of life than the tics. Be aware of these and make sure that every patient with TS is screened for these disorders because diagnosing them can really improve a person's quality of life.
What are some of the most common co-occurring disorders?
In children, at least 50 percent of those with tic disorders will also have attention-deficit/hyperactivity disorder (ADHD). The child may be referred for tics, but we recommend to physicians that they be aware of this association and that children are appropriately screened. And 20-50 percent of people with TS will also have obsessive-compulsive disorder, so appropriate screening is also needed for that. Other psychiatric disorders associated with TS include generalized anxiety disorder, depression, and oppositional defiant disorder. There is an increased risk of suicide as well. The guideline provides recommendations for screening, assessment and management of co-occurring psychiatric disorders in people with tics.
Do all tics need to be treated?
No, not all tics need to be treated. Physicians need to counsel patients on the natural history of the disorder. In about 75 percent of children, by the time they reach late adolescence, their tics will either entirely resolve or be much improved so that they only have minimal or mild tics in adulthood. If the tics are not causing any pain or emotional distress and not impacting social engagement, then we can tell patients and their parents that watchful waiting is appropriate. We can monitor how the child is doing and consider not treating unless there are impairments that occur in quality of life, knowing that there is a good chance that things will get better with time.
If treatment is necessary, what are the options?
One thing that may really interest neurologists is the evidence around the use of the comprehensive behavioral intervention for tics (CBIT), which is a therapy performed with a trained psychologist. It has been shown to be just as effective as medication. There are no head-to-head trials directly comparing the two, but if you look at the effect sizes in the respective trials, CBIT is comparable with medications. I tell my patients it's the closest thing to a cure for tics because we're teaching people with tics how to train their brain to respond differently to the urges that drive their tics. This can be very effective if done properly.
How does CBIT work?
Most CBIT interventions that have been studied in trials involve at least eight hour-long sessions of therapy over a period of 10 weeks. In these sessions, patients learn to develop tic awareness and self-monitoring for tics and the urges around them. CBIT also incorporates competing response training, which involves engaging in a voluntary behavior that is physically incompatible with the tic when the urge to tic occurs. Let's say I have a tic where I lift my shoulder up in the air quickly and bring it down. Before I do that I get an itchy feeling in my shoulder. That itchy feeling drives me to repetitively lift my shoulder in the air. I would learn to pay attention to that warning signal, the itchy feeling in my shoulder. When I got that feeling, I would learn to tense my shoulder and push it down slightly—because you can't hold your shoulder down and lift it up at the same time—and wait for the urge to pass.
How might that work for a vocal tic?
For vocal tics, often the competing response is a slow, controlled exhalation. The urge associated with a vocal tic can be a sensation or pressure in the chest or throat or a feeling or thought that you need to do it. So instead of making the vocal tic, you do a slow, controlled breath out with your mouth open, or a series of breaths, until the urge passes. It varies depending on the tic. There is a lot of problem-solving that goes on between the therapist and the patient. They dissect the tic: what is the earliest warning that it's going to occur? What is the feeling that directly precedes the tic? This helps them find a competing response to engage in.
Are there other components to CBIT?
Yes, these include relaxation training as well as trying to identify situational factors that influence tic severity and developing behavioral strategies to modify those factors. The psychologist works with the person to assess their day and identify points when they are having a lot of tics. What are they thinking about? Who's around them? Is it an avoidant behavior? Is it stress related? There is often a reciprocal relationship between anxiety and tics. Patients will say, “The more anxious I get, the more I tic, and the more I tic, the more anxious I get.” So if you can identify those situations where anxiety is high and figure out a way to bring down the anxiety, it can improve the tics.
What does the evidence tell us about medication treatments?
The selection of which medication to use should be a collaborative decision between physicians and [their] patients, but we have general principles to guide us. With a disorder like TS, we obviously don't have as much data as we do for more common disorders. While the number of studies and participants are small, we do have some evidence to support certain medication treatments, and the guideline provides recommendations on how to prescribe all of these drugs safely.
The most studied classes of drugs for the treatment of TS and tics are antipsychotics and alpha-2 adrenergic agonists. We typically start with the alpha agonists, clonidine and guanfacine, because they have fewer side effects. While we do need to keep an eye on blood pressure and heart rate, and there can be problems with sedation, they are otherwise well tolerated medications. These medications have also been shown to improve ADHD symptoms of inattention and hyperactivity, so if you have a patient with both conditions, this drug would be a good option.
If these medications fail or tics are more severe, then often an antipsychotic will be tried. They are probably the most studied treatment for tics, but often we don't use them as a first line treatment because they are associated with a number of adverse effects including drug-induced movement disorders, metabolic side effects, and hormonal side effects. Because of this, they require close monitoring.
When I prescribe an antipsychotic for tics, I see the patient monthly for the first three months and then every three months for the first year to keep close watch for adverse effects. Lab work is also required at baseline and then every three or six months, depending on the medication, and we must keep close watch on height, weight, and waist circumference to monitor for weight gain. I also do an examination for drug-induced movement disorders. A lot of these adverse events are reversible, especially if we catch them early.
First-, second-, and third-generation antipsychotics have all been shown to be efficacious, so when we choose agents, we generally choose them based on adverse effects. Generally, second- and third-generation agents are preferred over first-generation antipsychotics, which are more likely to cause drug-induced movement disorders.
What other medications are available?
One option is botulinum toxin in adults. The guideline points out that we don't have a lot of direct evidence for this agent, but it is very well tolerated in adults with simple motor tics of the face and neck. Botulinum toxin injections are popular with patients because you can target the exact tic that's bothering them and you don't have to worry about a lot of systemic side effects. Although the injections do need to be repeated every three to four months, I find that patients adhere well to this treatment because they're not having to cope with a lot of adverse systemic effects.
Another drug that is generally well tolerated is topiramate, an antiseizure medication. But there are not a lot of data for it with tics, and only one positive trial that we report in the guideline.
Any final takeaways?
The guideline stresses that if you do decide to prescribe a medication to a child with a tic disorder, it's very important to re-evaluate the patient annually. Because of the natural history of the disorder, it could be that the child won't need the medication as they grow older. Review their symptoms on at least an annual basis and periodically try to taper the medication to see if it is no longer needed. You want to ensure that the effects that you are seeing are actually still a result of the medication and not due to the passage of time and continued neurodevelopment.
Dr. Pringsheim has no disclosures to report.