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Tourette Syndrome
Sunday, 14 August 2005
Sunday, 14 August 2005
Gilles de la Tourette syndrome, GTS

What

Tourette syndrome is a neurologic disorder characterized by multiple motor and vocal tics. Motor tics are involuntary and repetitive movements resembling a purposeful movement. Motor tics usually involve the face and shoulders. Vocal tics are similar to motor tics but involve involuntary sounds or speech. In many cases, there are a variety of associated disorders (referred to as co-morbid conditions) associated with Tourette syndrome. The two most common behavioral and emotional disorders that can be seen in children who have Tourette Syndrome are attention-deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). In some cases ADHD or OCD can be the most serious part of this syndrome.

Who

The onset of Tourette syndrome is typically between 2 and 15 years of age, although the diagnosis is sometimes delayed until early adulthood in some cases. Having tics alone does not indicate a diagnosis of Tourette syndrome because studies have shown that tics can occur in 4-24% of school children. Most of these children do not have Tourette syndrome. In order to make the diagnosis of Tourette Syndrome a child must meet specific criteria including multiple motor tics and at least one vocal tic for a year. For reasons that are not clear, males are affected more often than females. Tourette Syndrome is found in all social classes, cultures, and ethnic groups.

Signs and Symptoms

Tics in Tourette syndrome can be simple or complex. Simple tics include; blinking, facial grimacing, shoulder shrugging, and head jerking. Many children have complex sequences of coordinated movements including; unusual walking, kicking, jumping, body gyrations, scratching, touching, or licking. The first commonly reported motor tic is eye blinking or squinting and the most common initial vocal tics are sniffing and throat clearing. Coprolalia (involuntary uttering of obscenities) occurs in about one-third of people who have Tourette syndrome, but is less common in children. The average age of onset for coprolalia is usually 13-15 years and in some cases disappears over time. Echolalia (imitation of sounds or words of others) and echopraxia (imitation of actions of others) can occur in 10-40% of people with this syndrome. Palilalia (repetition of the last word or phrase in a sentence or the last syllable of a word) occurs in about 10% of people with this syndrome. Motor tics and vocalizations are made worse by stress, boredom, fatigue, and excitement. Sleep, relaxation, or concentrating on an enjoyable task often reduces symptoms. Premenstrual stress and stimulants (caffeine, methylphenidate, and amphetamines) may make tics worse. Children with this disorder describe an irresistible urge before and relief after a tic. The symptoms wax and wane and may be worse for periods of days or weeks, then be less severe for other periods. The tics tend to increase to maximum severity during prepubescent years but will decline in frequency and severity in about two-thirds of cases by the beginning of adulthood.

Possible Causes

While the hereditary nature of Tourette syndrome has been recognized from the time of original description, the exact genetic mechanism underlying the disorder is still unknown. It has been suggested that the basis for Tourette syndrome involves an imbalance of neurotransmitters in the central nervous system (the brain and the spinal cord). Neurotransmitters are chemicals that travel between the cells of the central nervous system (neurons) and transmit signals that tell the body how to function and what to do. It is also thought that environmental factors may be involved in the expression of Tourette syndrome but no specific factors have yet been identified.

Diagnosis

In order to diagnose Tourette syndrome, the physician will take a thorough family history and perform a physical exam. The physician will want to distinguish tics in Tourette syndrome from other disorders. Brain imaging, such as MRI and PET scans, may show abnormalities characteristic of Tourette syndrome. It may be difficult to make an accurate diagnosis of Tourette syndrome because many of the symptoms of Tourette syndrome can also be found in other disorders, including the disorders commonly associated with Tourette syndrome. Currently the most important criteria for diagnosing Tourette syndrome are the identification of multiple motor tics and at least one vocal tic lasting at least a year.

Treatment

Treatment of Tourette syndrome is guided to trying to control the most severe and troublesome symptoms. The most disabling symptom may not always be tics and could be associated disorders such as ADHD, OCD, or other behavior problems. When tics are interfering with normal function they should be treated. Most children with mild tics can avoid using medications. Educating the children, family members, peers, and school personnel, restructuring the educational environment, and supportive counseling can all be used and be sufficient to avoid medications. If the tics are functionally disabling, medications may be used to help suppress or control them. Currently, medications called dopamine agonists and dopamine antagonists are considered to be the most effective in reducing the frequency and intensity of tics. Other medications can also be helpful in specific cases. There is currently no cure for Tourette syndrome, but research to determine the cause of the disease is ongoing in the hope of finding the cure.

Prognosis

In most cases, Tourette syndrome appears as a mild, nondisabling condition that does not require extensive medical attention or therapy. Children with Tourette syndrome usually have normal intelligence and, provided they get the treatment needed for their specific symptoms and the appropriate assistance from family members and school personnel, should be able to lead happy and normal lives.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Tourette Syndrome Association, Inc
This website has information, tips for parents of children who have recently been diagnosed, and links to current research.

Tourette Syndrome Foundation of Canada
This website has a good question and answer section providing information as well as a chat board for support and a “kid’s korner” for children to share their experiences.

Tourette-Syndrome.com
This site is an online community devoted to children and adults with Tourette syndrome. It provides a chat room for support as well as information about the disorder.

Google Search for Tourette Syndrome

References and Sources

Hyperkinetic Movement Disorders www.utdol.com/application/topic.asp?file=ped_neur/8748&type=P&selectedTitle=1~4 Watts, RL, Koller, WC (2004). Movement Disorders: Neurologic Principles & Practice. 2nd ed. New York: The McGraw-Hill Companies, Inc. Dewey, D, Tupper, DE (2004). Developmental Motor Disorders: A Neuropsychological Perspective. New York: The Guilford Press Joseph, AB, Young, RR (1999). Movement Disorders in Neurology and Neuropsychiatry. 2nd ed. Massachusetts: Blackwell Science, Inc. Robertson, MM, Eapen, V (1995). Movement and Allied Disorders in Childhood. England: John Wiley & Sons Ltd