Tourette Syndrome: Looking Past the Tics

At a Glance

  • Three out of every 1,000 children between the ages of 6 and 17 have Tourette syndrome.
  • Researchers at Weill Cornell published the first genetic study that suggested that Tourette’s was a much more complex disorder than originally thought.
  • Comprehensive Behavioral Intervention for Tics has been found to help patients become aware of situations in which tics are more likely to occur.

“We’ve come to understand that if you want to help someone with Tourette’s, you have to empower them to manage their tics psychologically and environmentally to reduce their overall tic severity.”

John T. Walkup, MD

In France in 1825, 60 years before Tourette syndrome was defined as a discrete phenomenon, Marquise de Dampierre, a noblewoman who twitched and jerked uncontrollably and screamed obscenities in gatherings of high society became the first patient with the syndrome to be described in the medical literature. By 1885, neurologist Georges Gilles de la Tourette singled out nine patients, using Madame de Dampierre as a base exemplar, to demonstrate the distinct features of the “maladie des tics.” For the next 80 years, Tourette’s and chronic tic disorders – burdened with social stigma and conflicting etiologic and treatment theories – were considered rare with only references in the medical literature. The efficacy of pharmacotherapy in the 1960s changed our conceptual understanding of Tourette’s and tics and led to a dramatic increase in recognition and treatment.

More recently, the demonstration that behavioral treatment could reduce tic severity again changes our fundamental understanding of tic disorders.

John T. Walkup, MD, Director, and Shannon M. Bennett, PhD, Co-Director, lead the Tourette Syndrome Center for Excellence at Weill Cornell Medicine — one of nine Centers of Excellence across the country established by the Tourette Association of America, all based at premier medical and academic centers. For more than 30 years, Dr. Walkup has been working with patients with Tourette syndrome, a disorder that affects three out of every 1,000 children between the ages of 6 and 17.

For many of those years, Dr. Walkup served as Chair of the Medical Advisory Board of the national Tourette Association. He has conducted both genetic and treatment research on the disorder, and he and his colleagues published the first genetic study that suggested that Tourette’s was a much more complex disorder than originally thought – much like other neuropsychiatric disorders.

Dr. John T. Walkup and Dr. Shannon M. Bennett

Dr. John T. Walkup and Dr. Shannon M. Bennett

Dr. Walkup is also a staunch advocate for those affected, routinely reaching out to schools and community groups through the Tourette Association to increase awareness of this still stigmatizing disorder and disseminate the newest thinking on treatment.

“Tics are very common in childhood,” says Dr. Bennett, who specializes in behavior therapy for Tourette Syndrome. “Upwards of 10 to 20 percent of very young children will have transient tics. A smaller number – three to four percent – will have chronic tics, and about between 0.5 to 1 percent of children will develop Tourette Syndrome. Coprolalia – a vocal tic that involves curse words or inappropriate language – is present in about 10 percent of individuals with Tourette Syndrome. What’s important to know is that the term Tourette’s conjures up a severe, loud, interfering disorder, likely due to how it is portrayed in the movies. While Tourette Syndrome can be extremely impairing in life, it very rarely looks like what you see in the movies or on talk shows.”

What We Know About Tourette syndrome

The 1960s found patients with Tourette’s responding positively to antipsychotic medications. “Movement disorders often involve perturbation of the dopamine system,” says Dr. Walkup. “If you think of antipsychotics from a mechanistic point of view, they are potent dopamine blockers. They essentially quiet the dopamine system, and with quieting of the dopamine system you’ll see a reduction of tics. Unlike some movement disorders, however, Tourette’s is particularly sensitive to the environment and other triggers. The biggest game changer in Tourette’s has been the shift from thinking of it as a neurologic disorder that needed to be treated with medication to a disorder with a neurologic basis but whose symptoms are highly sensitive to influences from the environment.

“The most important shift that has happened in Tourette’s in the past 30 years is probably the development of behavioral treatment,” continues Dr. Walkup. “This approach provides our young patients with an activity that can help them manage their tic severity and enable them to stay in situations rather than avoid them.” Environmental triggers, including stress and other psychological and social triggers, clearly provoke ticking and other symptoms, but calm focused activities can also be used to quiet tic expression. “We’ve come to understand that if you want to help someone with Tourette’s, you have to empower them to manage their tics psychologically and environmentally to reduce their overall tic severity,” says Dr. Walkup. “It’s not so much about lowering their stress level or avoiding stress, which historically is what patients were told; it’s about improving their capacity to tolerate stress.”

Habit reversal therapy is a behavioral intervention that has been in use for decades to help people change problematic habit behaviors.

“When behavioral psychologists started using habit reversal therapy to see if they could inhibit tic behaviors,” says Dr. Bennett, “they found that it was possible to limit the expression of tics by channeling the energy – the premonitory urge – that comes right before a tic.”

Comprehensive Behavioral Intervention for Tics, or CBIT, is derived from the tradition of habit reversal therapy. “Many patients will experience an uncomfortable urge that is alleviated by the expression of the tic,” says Dr. Bennett. “This, however, creates a negative reinforcement loop, or a feedback loop, in that once that urge returns, the person has the tic again. It’s a behavior that patients feel they can’t control. CBIT helps them to become aware of these urges and the situations and context in which the tics are more likely to occur. Giving them a competing response or an action that they can take to break the loop between the urge and the tic has been very effective. It’s an intense treatment in the beginning as individuals learn to respond to the tics differently, especially for those with frequent tics. But over time CBIT helps the tics to fade into the background.”

“Perhaps the most important consideration in Tourette’s is the other conditions with which it is associated,” emphasizes Dr. Walkup. “The average age of diagnosis is about seven. It tends to peak in severity in the early teen years and then dissipate. But the conditions that it can co-occur with – ADHD, obsessive-compulsive disorder, anxiety disorders in prepubertal children, and then mood disorders in older adolescents and young adults–start about the same time. While the tics get better on their own, the other conditions, if they are not recognized and treated, pick up momentum and increase disability over time. The biggest contribution that I feel I have made to the management of Tourette’s is to refocus thinking away from the tics alone to the co-occurring conditions that are oftentimes substantially more disabling than the tics themselves.”

“As are most of our psychiatric diagnoses, Tourette’s is likely a multigenic illness in which different genes turning on or off leave someone with a relative vulnerability,” adds Dr. Bennett. “We do believe that it runs in families, but we don’t know why it happens at a particular time of life. In terms of comorbidities though, we do know that it’s highly comorbid with ADHD – about 50 percent of individuals with Tourette Syndrome also have ADHD. About 30 percent of individuals with Tourette’s also have OCD. Tourette Syndrome and OCD share a similar neurobiological circuit in the brain. Depression often comes later, so we see many adults with Tourette Syndrome who may have developed depression over time due less to a biological relationship, but often because it’s an added burden or consequence of living with Tourette Syndrome.”

“You can go after the tics, but if you’re not thinking in a comprehensive way about the assessment and as a result leave other issues untreated, you’re likely to engage in a process that might help one aspect of the problem, but may not address the larger concerns,” says Dr. Walkup. “The tics may get better but the patients are left with residual symptoms in other domains that are really causing them distress and impairment.”

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