Just what is Tourette’s syndrome?
Tourette’s syndrome is a neurological disorder reported in 1885 by Gilles de la Tourette, a French neurologist, consisting of involuntary tics or movements. These tics are frequent, sudden, brief, intermittent and stereotyped. The frequency and severity of the tics may wax and wane over time.
What are the symptoms of Tourette’s syndrome?
There are two types of tics. The motor tics often consist of facial twitches, grimaces, shoulder shrugs, jerking, eye blinking, or obscene gestures. While the vocal tics may manifest as grunting, barking, sniffing, throat clearing, or swearing.
Children with Tourette’s syndrome cannot voluntarily control their tic behaviors, except briefly (like an itch that must be scratched sooner or later). Many children with Tourette’s syndrome will have motor and phonic tics even while sleeping.
Children with Tourette’s syndrome are often misunderstood and teased by other children. In reality, most children with Tourette’s syndrome just want to be treated normally – like everybody else.
Can children with Tourette’s syndrome control their tics?
The answer to this question is a bit complicated. The tics of Tourette’s syndrome are considered unvoluntary rather that strictly involuntary. Folks with Tourette’s syndrome can suppress their tics for a short time. However, they experience a build up in tension that ultimately results in tics expressed against their will. This phenomenon is much like an itch that can be delayed for a short while but which ultimately must be scratched. Emotional stress, fatigue, and anxiety are known to worsen the severity and frequency of tics.
Are there any associated symptoms in addition to the tic behaviors?
Although motor and vocal tics are the hallmark of Tourette’s syndrome, patients may also experience restless, difficult sleep, bedwetting, and nightmares.
In addition, there is a high frequency of other associated conditions (co-morbidities) in patients with Tourette’s syndrome. These co-morbid conditions include: attentional disorders (ADHD and ADD), oppositional defiant disorder (ODD), conduct disorder, obsessive-compulsive disorder (OCD), migraine headaches, and learning problems. Children with autistic spectrum disorder experience Tourette’s syndrome in approximately 20% of cases.
Tourette’s syndrome is not known to adversely affect intelligence nor life expectancy.
How often does Tourette’s syndrome occur?
Tourette’s syndrome is significantly more common than originally believed. Tourette’s syndrome occurs in 7-8 children per 1000 of the population. However, it is much more common in boys than girls. Tourette’s syndrome most often presents before the age of 11 years, but may begin any time between the ages of 2 and 18 years.
How do you test for Tourette’s syndrome?
The diagnosis of Tourette’s syndrome is made solely on clinical grounds. That is, your provider will diagnose Tourette’s syndrome based on your child’s symptoms. The diagnosis is on firmer ground if vocal tics are present along with motor tics some time during the illness, although it is not necessary to have both motor and vocal tics at the same time. The tic behaviors should begin prior to the 21st year of life and last longer than a year. The presence of co-morbid conditions (see above) known to be associated with Tourette’s syndrome also may help to confirm a diagnosis.
Laboratory tests, EEG’s and X-ray’s and other imaging tests such as CAT Scans are not helpful in the diagnosis of Tourette’s syndrome. Recently, MRI Scans and PET Scans have identified some brain abnormalities in Tourette’s syndrome patients in research settings.
Sometimes, however, blood tests, EEG’s or imaging studies are performed to rule out other conditions that may imitate Tourette’s syndrome.
How did my child catch Tourette’s syndrome?
Tourette’s syndrome is felt to be an inherited disorder. The genetics were felt to be largely autosomal dominant with incomplete penetrance. However, more recent work is revealing a more complex pattern. Mutations have been identified in the SLITRK1 gene on chromosome 13q31.1 and, more rarely, on the HDC gene on chromosome 15q21-q22.
If a parent has Tourette’s syndrome, the risk of having a child with Tourette’s syndrome is 22%. The siblings of an affected child have an 8% chance of developing Tourette’s syndrome.
PANDAS, a movement disorder felt to be caused by untreated strep throat, is no longer assumed to be a direct cause of Tourette’s syndrome.
Other non-genetic, post-infectious, environmental and psychosocial factors can affect the severity of Tourette’s syndrome.
What treatments are available for Tourette’s syndrome?
Patients with mild Tourette’s syndrome symptoms that do not interfere with their daily activities of living may not require specific treatment. However, if your child is having difficultly with the normal activities of school, or at social gatherings, or with friends; or if your child is the victim of teasing or bullying; or if your child is having difficulty performing every day tasks, then you might consider treatment options.
Certain medications are available which decrease the frequency and severity of tic behaviors. In particular, the alpha-adrenergic agonist class of drugs may prove beneficial. The most commonly used medications are clonidine (Catapres and Kapvay) and guanfacine (Intuniv and Tenex). These medications may also benefit the attentional disorders associated with Tourette’s syndrome.
Some antipsychotic medications, in certain settings, have also been shown to decrease motor and vocal tics: risperidone (Risperdal), aripiprazole (Abilify) and other related compounds. Drugs that block dopamine receptors such as fluphenazine (Prolixin), pimozide (Orap) or tetrabenazine (Xenazine) may also be considered.
Botulinum toxin (Botox) may be considered in severe cases. Its use should only be considered by a specialist very familiar with its use.
Selective Serotonin Reuptake inhibitors (SSRI’s) like Zoloft, Prozac, and Luvox may be used to treat the anxiety and OCD symptoms associated with Tourette’s syndrome.
Some medications may exacerbate (make worse rather than cause) tic behaviors and should be avoided, when feasible, in children with Tourette’s syndrome. In particular, the stimulant class of medications used to treat attention deficit disorder (ADD, ADHD) may fall into the category of unmasking tics. In such cases, a non-stimulant such as Intuniv (improves tics) or Strattera (largely tic neutral) should be considered.
Habit reversal training is a therapy shown to have some success in Tourette’s syndrome patients. Habit reversal training requires a therapist trained in this specific technique as applied to patients with Tourette’s syndrome.
Will my child outgrow Tourette’s syndrome?
There is no cure for this disorder. However, about one half of children with Tourette’s syndrome have greatly reduced tic behaviors as adults. Occasionally, the tics may return late in life.
In Summary
The proper management of Tourette’s syndrome includes the accurate and timely diagnosis of the ailment; education of patients, parents, teachers and friends; genetic counseling; behavioral and pharmacologic treatments if indicated; and generous support and understanding by the community.
