Because of the visible nature of Tourette syndrome (TS) – the repetitive, involuntary movements and utterances, sometimes of offensive words – most people think they understand it, but it is actually much more complex and often intertwined with other behavioral disorders.
Elena Bortan, MD, a specialist with the Hartford HealthCare Ayer Neuroscience Institute Movement Disorders Center, said there are multiple layers to TS, making each diagnosis unique to the patient.
“Diagnosis of TS is based on the clinical features of the disease, with onset before age 18 or 21,” she explained. “Interestingly, the motor tics are sudden, rapid, recurrent and non-rhythmic twitches of body parts or facial muscles preceded by an urge. They can be temporarily suppressed, but typically increase with stress and decrease with distraction and concentration.”
Named after Dr. Georges Gilles de la Tourette, who first described it in 1885, TS is defined by
motor tics, which can include repeatedly blinking eyes, shrugging shoulders or scrunching the nose, and phonic tics or vocalizations.
Evaluations for TS should include careful reviews of the patient’s medical, social and family histories for tics or tic-related disorders, as well as the degree of functional impairment they cause, Dr. Bortan said.
“Neurological exams of patients with TS are often normal except for the presence of tics,” she said, noting that CT scans and MRIs are “unremarkable” in these patients. “The diagnosis is often supported by the presence of additional behavioral disorders.”
A number of different conditions can coexist with TS, including:
- Attention deficit hyperactivity disorder (ADHD) in 30 to 60 percent of patients.
- Obsessive-compulsive disorder (OCD) in 10 to 50 percent of patients.
- Anxiety in up to 30% of patients.
- Other disruptive behaviors.
The main goals of treatment for TS are to reduce tic frequency and manage coexisting behavioral disorders to improve function and quality of life.
“There is no cure for TS, and treatment rarely leads to complete suppression of tics. But, treatment is worthwhile when TS symptoms are interfering with the patient’s social interactions, school or job performance, or causing subjective discomfort, pain or injury,” Dr. Bortan said.
If tics are mild, the treatment approach is called “watchful waiting,” and includes education, counseling and supportive care. Patients should start treatment for any underlying conditions like ADHD or OCD.
When tics cause psychosocial, physical, functional or other problems, a more complex strategy is recommended, Dr. Bortan said. Comprehensive Behavioral Intervention for Tics can be used for habit reversal training. If this is not an option, medications can be tried based on the severity of tics and coexisting conditions such as hypertension. Botulinum toxin injections into the affected muscle can also help with focal motor tics.
“In terms of therapy duration, the consensus is to gradually taper off medications during a non-stressful time once good long-term tic control is achieved,” Dr. Bortan said. “This works well during the teenage years, when tics tend to improve spontaneously. Approximately one third of tics resolve completely, one third improve, and one third continue without reduction.”