[Editor's note: This article is from 2007. Some newer treatments and current statistics are not included here. See further information on BrainWeb]
Sections include: tics, behavioral symptoms, diagnosis and treatment, treating tics
Tourette’s syndrome (TS) is a neurological disorder manifested by motor and vocal tics, usually starting in childhood. It is often accompanied by obsessive-compulsive disorder (OCD), attention deficit/hyperactivity disorder (ADHD), poor impulse control, and other behavioral problems. Once considered a rare psychiatric curiosity, TS is now recognized as a relatively common and complex neurobehavioral disorder affecting up to 3 percent of the general population. Many notable historical figures, including Samuel Johnson and possibly Mozart, are thought to have been afflicted with TS, like some accomplished individuals today.
The French neurologist Georges Gilles de la Tourette first described the disorder in 1885. He had noticed nine patients who shared one feature: they all exhibited brief involuntary movements he called tics. In addition, six made noises, five shouted obscenities (coprolalia), five repeated other people’s words (echolalia), and two mimicked others’ gestures (echopraxia). Although Tourette considered the disorder hereditary, for nearly a century afterward, most physicians felt the behavior had psychological causes. The perception of TS began to change in the 1960s, when doctors recognized that people with the symptoms benefited from drugs that block dopamine receptors in their brains.
The cause of TS is still unknown, but most people with the disorder appear to have inherited it. Many studies have found that people often inherit a genetic susceptibility from both parents. How the genetic defect surfaces in behavior varies from one person to another, even within families. In addition, individuals’ symptoms often fluctuate.
The tics that characterize TS are of two sorts: motor and vocal. Motor tics are abrupt, brief, rapid, repetitive movements. They may fluctuate in distribution and severity, and a person can often suppress them for a while. Common motor tics include eye blinking, eye deviation, facial grimacing, neck popping or stretching, and shoulder shrugging.
Tics that produce sound from air moving through the nose or mouth are called vocal or phonic. The most common include sniffing, coughing, grunting, screaming, and squealing.
We also classify tics as either simple or complex. Simple motor tics involve the repetitive movement of one muscle group, such as shoulder shrugging, eye blinking, or neck popping. Complex motor tics involve the integration of learned, sequenced movements, such as touching someone or something, tapping, jumping, or socially inappropriate gestures.
The most overemphasized TS symptom is a complex vocal tic: coprolalia, the shouting of obscenities or profanities. Only about a third of all people with the disorder ever actually have this problem. Other complex vocal tics include palilalia (repeating the last syllable, word, or phrase in a sentence) and echolalia (repeating someone else’s words or phrases).
Motor and vocal tics may persist during all stages of sleep. They are usually exacerbated by stress and by suggestion. People with TS, especially children, often exert energy to suppress their tics in order to avoid embarrassment or social ridicule. This can reduce the attention students can give to their schoolwork, and cause their symptoms at school to appear different from their symptoms when alone with family members.
People with TS often feel premonitory sensations before tics; they describe these as increased tension or discomfort, a need to stretch the muscle, or an urge to tic until it “feels just right.” The “just right” desire may also relate to the compulsive behaviors of OCD. Frequently, TS patients engage in these activities until things look “just right” or their bodies feel “just right.”
ADHD and OCD commonly accompany tics in TS, as do such other behavioral problems as poor impulse control, anxiety, mood and conduct disorders and self-injurious behavior. These behavioral symptoms may contribute to poor academic, social, and work performance. For many people, they are more troubling and debilitating than the motor tics.
About half of all people with TS have symptoms of ADHD sometime during the course of the disease. Although attention deficit is certainly one of the most common and disabling symptoms of TS, for many individuals the inability to pay attention is due not only to a coexistent ADHD but also to poor motivation, unwanted intrusive thoughts, the mental concentration needed to suppress tics, sedation from anti-TS medications, and associated migraine headaches.
Some people with TS cannot pay attention because of a compulsive fixation of gaze. While they are sitting in a classroom or a theater, for instance, their gaze can become fixed on a particular object. Despite concentrated effort, they are unable to break the fixation. As a result, they might miss a teacher’s lesson or a particular action in a play. This can also happen during conversations, leading others to think they are choosing not to listen.
That OCD is a part of the spectrum of neurobehavioral manifestations in TS is now well accepted. Most people with OCD have been found to have a lifetime history of tics, suggesting a link to TS.
Diagnosis and Treatment
The Tourette Syndrome Association, scientific programs, and the media have increased professional and public awareness of TS. As a result, more people with tics have consulted with their doctors about these symptoms, and doctors are correctly diagnosing TS earlier than in the past. Many individuals, however, still wrongly attribute their symptoms to such things as habits, allergies, asthma, dermatitis, hyperactivity, and nervousness.
Diagnosing TS depends on a careful evaluation of a person’s symptoms and signs by an experienced clinician. Tics do not automatically point to TS, though that is their most likely cause. Tics appear in other neurological disorders, and there are other symptoms of TS to look for.
Once a doctor has confirmed that a person has TS, the next task is for the doctor and patient to discuss what sort of treatment seems most useful. The goal should not be to completely eliminate all symptoms. Often the side effects of the drugs or other therapies needed to achieve that goal outweigh the benefits. Rather, treatment should be aimed at suppressing tics and other symptoms so that each individual can function at his or her best. Doctors must also consider which component of TS is causing the most difficulty. For instance, a person might have an obsession that triggers a tic; treatment might target the obsession, the tic, or both. Similarly, when an individual with TS has an attention deficit, the doctor cannot assume the problem is due to ADHD but must consider other possible causes before selecting the best therapeutic approach.
TS is a chronic condition that people must learn to manage. It does not have to be debilitating. Even people with severe TS tics, such as touching the faces of people with whom they speak—normally a social taboo—have been able to adapt and enjoy happy, successful lives. Especially for children with TS, the best treatment requires the integrated experience of educators, physicians, psychologists, and social workers.
Most physicians believe that the treatment of choice for reducing the frequency and severity of tics involves medications that act by blocking dopamine receptors or by depleting dopamine. The neuroleptic (dopamine receptor–blocking) drugs include fluphenazine (Prolixin), haloperidol (Haldol), risperidone (Risperdal), and pimozide (Orap). While they have proved effective in reducing tics, they can also bring a variety of side effects: gastrointestinal upset, sedation, restlessness, and weight gain. Face and neck spasms, lockjaw, or involuntary eye deviation may occur with all these drugs, but these side effects can be reversed with anticholinergic medications such as benztropine (Cogentin) or diphenhydramine (Benadryl). People taking pimozide are advised to have regular electrocardiogram (ECG) tests. In rare cases, dopamineblocking agents may cause involuntary repetitive movements that usually involve the lower face and mouth, called tardive dyskinesia.
Another antidopaminergic drug, tetrabenazine, may be effective in treating tics without the risk of tardive dyskinesia, but this drug has not yet been approved by the Food and Drug Administration. Other drugs occasionally found useful in the treatment of tics include clonidine, guanfacine, and clonazepam.
Yet another alternative for treating tics is the use of botulinum toxin injections in the area of the most problematic tic. The botulinum temporarily paralyzes or weakens the muscles. Such injections are safe and effective in reducing the severity and frequency of the tic, as well as the premonitory sensation or tic urge, without the side effects of systemic medications. Depending on the site of injection, the most common side effects include drooping eyelids, weak neck, and low vocal volume (hypophonia).
When TS and ADHD are both present, behavioral modification, school and classroom adjustments, and stress management may provide important emotional support for the person with TS and family members, helping to improve self-esteem and motivation. But while these behavior strategies may be useful in ADHD, for most people they are rarely fully effective by themselves. Pharmacological therapy is usually required as well. The central nervous system (CNS) stimulants used to treat ADHD, such as methylphenidate (Ritalin), controlled-release methylphenidate (Concerta), dextroamphetamine (Dexedrine), mixture of amphetamine salts (Adderall), and pemoline (Cylert), can exacerbate or precipitate tics in up to 25 percent of people with TS. If the symptoms of ADHD are the most troublesome for an individual, however, it is reasonable to use these stimulants while keeping the dosage at the lowest effective level. Furthermore, many people report that their tics return to baseline levels several days or weeks after they start taking a stimulant medication. Alpha-2 agonists and tricyclic antidepressants are also useful in treating ADHD, particularly if people cannot tolerate CNS stimulants. Clonidine (Catapres) improves symptoms of ADHD and of impulse-control problems but only occasionally has been found to be effective against tics.
The most effective drugs for OCD are the selective serotonin reuptake inhibitors (SSRIs). These drugs usually do not relieve typical motor or phonic tics, but they may be beneficial in controlling the premonitory urges and compulsions (that is, “compulsive tics”). It may take two or three months for people’s symptoms to respond, and about a third of all people show no response to one or another SSRI.
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