Tourettes Syndrome
Tourette's syndrome (TS) is a neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics. The disorder is named for Dr. Georges Gilles de la Tourette, the pioneering French neurologist who in 1885 first described the condition in an 86-year-old French noblewoman. Tics are abrupt, purposeless, and involuntary vocal sounds or muscular jerks. The first symptoms of TS are almost always noticed in childhood.
Tourette syndrome involves both motor tics, which are uncontrolled body movements, and vocal or phonic tics, which are outbursts of sound. Some motor tics are simple and involve only one muscle group. Simple motor tics, such as rapid eye blinking, shoulder shrugging, or nose twitching, are usually the first signs of Tourette syndrome. Motor tics also can be complex (involving multiple muscle groups), such as jumping, kicking, hopping, or spinning.
In addition to frequent tics, people with Tourette syndrome are at risk for associated problems including attention deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), anxiety, depression, and problems with sleep.
Symptoms of Tourette’s:
Symptoms of TS usually begin between the ages of 5 and 10 years of age, and usually begin with mild, simple tics involving the face, head, or arms. With time, tics become more frequent and increase in variety, involving more body parts such as the trunk or legs, and often become more disruptive to activities of daily living. Tics usually appear in childhood, and their severity varies over time. In most cases, tics become milder and less frequent in late adolescence and adulthood.
TS may be found in people from all ethnic groups; males are affected about three to four times more often than females. It is estimated that 200,000 Americans have the most severe form of TS, and as many as 1 in 100 exhibit milder and less complex symptoms such as chronic motor or vocal tics or transient tics of childhood. Although TS can be a chronic condition with symptoms lasting a lifetime, most people with the condition experience their worst symptoms in their early teens, with improvement occurring in the late teens and continuing into adulthood.
Tic behaviors seen in TS change over time, and vary in frequency and complexity. The following are the most common tic behaviors associates with TD. However, each child experiences symptoms differently. Symptoms may include:
• Involuntary, purposeless, motor movements (may involve different parts of the body, such as the face, neck, shoulders, trunk, or hands)
• head jerking
• squinting
• blinking
• shrugging
• grimacing
• nose-twitching
• any excessively repeated movements (i.e., foot tapping, leg jerking, scratching)
Some of the more complex tic behaviors associated with TD may appear purposeful, and may include the following:
• kissing
• pinching
• sticking out the tongue or lip-smacking
• touching behaviors
• making obscene gestures
In addition to some, or all, of the above symptoms, TD is also characterized by one or more vocal tics (meaningless sound), in order for a diagnosis of TD to be made, including the following:
• grunting or moaning sounds
• barking
• tongue clicking
• sniffing
• hooting
• obscenities
• throat clearing, snorting, or coughing
• squeaking noises
• hissing
• spitting
• whistling
• gurgling
• echoing sounds or phrases repeatedly
Tics are classified as either simple or complex. Simple motor tics are sudden, brief, repetitive movements that involve a limited number of muscle groups. Complex tics are distinct, coordinated patterns of movements involving several muscle groups.
The most dramatic and disabling tics include motor movements that result in self-harm such as punching oneself in the face or vocal tics including coprolalia (uttering swear words) or echolalia (repeating the words or phrases of others). Some tics are preceded by an urge or sensation in the affected muscle group, commonly called a premonitory urge. Some with TS will describe a need to complete a tic in a certain way or a certain number of times in order to relieve the urge or decrease the sensation.
Tics are often worse with excitement or anxiety and better during calm, focused activities. Certain physical experiences can trigger or worsen tics, for example tight collars may trigger neck tics, or hearing another person sniff or throat-clear may trigger similar sounds. Tics do not go away during sleep but are often significantly diminished.
Forty percent of children and adolescents who have TS also have attention problems and 30 percent have academic difficulties. However, most have normal intelligence and do not usually have primary learning disabilities. Twenty-five percent to 30 percent also experience symptoms of obsessive-compulsive disorder (OCD), which is an anxiety disorder in which a person has an unreasonable thought, fear, or worry (obsession) that he/she tries to manage through a ritualized activity (compulsion) to reduce the anxiety.
The symptoms of TS may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
Possible causes Tourette's disorder:
The cause of TS is unknown, research points to abnormalities in certain bran regions. Given the often complex presentation of TS, the cause of the disorder is likely to be equally as complex. A variety of genetic and environmental factors likely play a role in causing Tourette syndrome. Most of these factors are unknown, and researchers are studying risk factors before and after birth that may contribute to this complex disorder. They believe that tics may result from changes in brain chemicals (neurotransmitters) that are responsible for producing and controlling voluntary movements. Tourette syndrome is a relatively common disorder. Although its exact incidence is uncertain, it is estimated to affect 1 to 10 in 1,000 children.
Tourette’s disorder is one where both males and females are affected. A parent with TD or a parent who has the gene for TS has a 50/50 chance, with each pregnancy, to pass the gene on. Tourette’s particular gene does not manifest its symptoms in everyone. In other words, if a parent passes the gene on to a child, the child may not have any symptoms of the disorder. If a daughter inherits the gene, there is a 70 percent chance that she will have at least one of the signs of TS. On the other hand, if a son inherits the gene, there is a 90 percent chance that he will have at least one of the signs of TS. Tourette’s is also associated with a non-genetic cause in 10 to 15 percent of children with this condition. Complications of pregnancy, low birth weight, head trauma, carbon monoxide poisoning and encephalitis are thought to be associated with the onset of non-genetic TS.
Finally, dominant disorders such as Tourette’s can also exhibit something known as variable expressivity. This means that there are differences in the expression of the TS gene in different people. For example, one person with TS may have obsessive-compulsive disorder, while another has a chronic tic disorder, while another has full-blown TS. In addition, there are differences in expressivity between males and females: males are more likely to have full-blown TS or chronic tics, while females are more likely to have obsessive-compulsive disorder.
Diagnosing Tourette's disorder:
A pediatrician, child psychiatrist, or a qualified mental health professional usually identifies TS in children and adolescents. A comprehensive evaluation of the child or adolescent's psychological, social, and educational status is recommended, as well as a thorough medical, developmental, and family assessment. A detailed history of the child's behavior from parents and teachers, in addition to observations of the child's behavior, contribute to making the diagnosis.
TS is a diagnosis that doctors make after verifying that the patient has had both motor and vocal tics for at least 1 year. Most commonly used for diagnosis are neuron-imaging studies, such as magnetic resonance imaging (MRI), computerized tomography (CT), and electroencephalogram (EEG) scans, or certain blood tests may be used to rule out other conditions that might be confused with TS.
It is not uncommon for patients to obtain a formal diagnosis of TS only after symptoms have been present for some time. The reasons for this are many. For families and physicians unfamiliar with TS, mild and even moderate tic symptoms may be considered inconsequential, part of a developmental phase, or the result of another condition. For example, parents may think that eye blinking is related to vision problems or that sniffing is related to seasonal allergies.
Treatment for Tourette's disorder:
Specific treatment for Tourette's disorder will be determined by your child's physician based on:
• your child's age, overall health, and medical history
• extent of disruption caused by tic behavior
• your child's tolerance for specific medications or therapies
• expectations for the course of the disorder
• your opinion or preference
The effect of symptoms on the child's or adolescent's self-concept, family and peer relationships, and classroom participation determines what needs are to be addressed in treatment. In many cases, TS is not disabling. Development may proceed normally, and there is no need for treatment. However, when tics interfere with functioning or school performance, and/or if there are other disorders also present (i.e., obsessive-compulsive disorder, attention-deficit/hyperactivity disorder), some effective medications are available. Children with TS can generally function well at home and in a regular classroom. If they have accompanying emotional or learning problems, they may require special classes, psychotherapy, and/or medication.
Due to tic symptoms not often causing impairment, the majority of people with TS require no medication for tic suppression. However, effective medications are available for those whose symptoms interfere with functioning. Unfortunately, there is no one medication that is helpful to all people with TS, nor does any medication completely eliminate symptoms. In addition, all medications have side effects.
Other medications may also be useful for reducing tic severity, but most have not been as extensively studied or shown to be as consistently useful. Effective medications are also available to treat some of the associated neurobehavioral disorders that can occur in patients with TS. Scientists hope that future studies will include a thorough discussion of the risks and benefits of medication in those with TS or a family history of TS and will clarify this issue.
Psychotherapy may also be helpful. Although psychological problems do not cause TS, such problems may result from TS. Psychotherapy can help the person with TS better cope with the disorder and deal with the secondary social and emotional problems that sometimes occur. More recently, specific behavioral treatments that include awareness training and competing response training, such as voluntarily moving in response to a premonitory urge, have shown effectiveness in small controlled trials.
Although there is no cure for TS, the condition in many individuals improves in the late teens and early 20s. As a result, some may actually become symptom-free or no longer need medication for tic suppression. Although the disorder is generally lifelong and chronic, it is not a degenerative condition. Individuals with TS have a normal life expectancy. Although tic symptoms tend to decrease with age, it is possible that neurobehavioral disorders such as depression, panic attacks, mood swings, and antisocial behaviors can persist and cause impairment in adult life.
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