What is Tourette syndrome?
Tourette syndrome (TS) is an inherited, neurological disorder
characterized by multiple involuntary movements and
uncontrollable vocalizations called tics that come and go over
years. In a few cases, such tics can include inappropriate words
and phrases. The disorder is named for Dr. Georges Gilles de la
Tourette, the pioneering French neurologist who first described
an 86-year-old French noblewoman with the condition in 1885.
The symptoms of TS generally appear before the individual is 18
years old. TS can affect people of all ethnic groups; males are
affected 3 to 4 times more often than females. It is estimated
that 100,000 Americans have full-blown TS, and that perhaps as
many as 1 in 200 show a partial expression of the disorder, such
as chronic multiple tics or transient childhood tics.
The natural course of TS varies from patient to patient.
Although TS symptoms range from very mild to quite severe, the
majority of cases fall in the mild category. What are the
symptoms? The first symptoms of TS are usually facial
tics--commonly eye blinking. However, facial tics can also
include nose twitching or grimaces. With time, other motor tics
may appear such as head jerking, neck stretching, foot stamping,
or body twisting and bending.
TS patients may utter strange and unacceptable sounds, words,
or phrases. It is not uncommon for a person with TS to
continuously clear his or her throat, cough, sniff, grunt, yelp,
bark, or shout. People with TS may involuntarily shout
obscenities (coprolalia ) or constantly repeat the words of
other people (echolalia). They may touch other people
excessively or repeat actions obsessively and unnecessarily. A
few patients with severe TS demonstrate self-harming behaviors
such as lip and cheek biting and head banging against hard
objects. However, these behaviors are extremely rare. Tics
alternately increase and decrease in severity, and periodically
change in number, frequency, type, and location. Symptoms may
subside for weeks or months at a time and later recur. How are
tics classified?
There are two categories of tics: simple and complex. Simple
tics are sudden, brief movements that involve a limited number
of muscle groups. They occur in a single or isolated fashion and
are often repetitive. Some of the more common examples of simple
tics include eye blinking, shoulder shrugging, facial grimacing,
head jerking, yelping, and sniffing. Complex tics are distinct,
coordinated patterns of successive movements involving several
muscle groups. Complex tics might include jumping, smelling
objects, touching the nose, touching other people, coprolalia,
echolalia, or self-harming behaviors. Can people with TS control
their tics?
People with TS can sometimes suppress their tics for a short
time, but the effort is similar to that of holding back a
sneeze. Eventually tension mounts to the point where the tic
escapes. Tics worsen in stressful situations; however they
improve when the person is relaxed or absorbed in an activity.
In most cases tics decrease markedly during sleep.
What causes TS?
Although the basic cause of TS is unknown, current research
suggests that there is an abnormality in the gene(s) affecting
the brain's metabolism of neurotransmitters such as dopamine,
serotonin, and norepinephrine. Neurotransmitters are chemicals
in the brain that carry signals from one nerve cell to another.
What disorders are associated with TS?
Not all people with TS have disorders other than tics. However,
many people experience additional problems such as obsessive
compulsive behavior, characterized by an intense need to act
repeatedly, such as hand washing or checking that a door is
locked; attention deficit-hyperactivity disorder, characterized
by difficulty concentrating and staying on task; learning
disabilities, which include reading, writing, and arithmetic
difficulties; or sleep disorders, which include frequent
awakenings or talking in one's sleep. The wide range of
behavioral symptoms that can accompany tics may, in fact, be
more disabling than the tics themselves. Patients, families, and
physicians need to determine which set of symptoms is most
disabling so that appropriate medications and therapies can be
selected.
How is TS diagnosed?
Generally, TS is diagnosed by obtaining a description of the
tics and evaluating family history. For a diagnosis of TS to be
made, both motor and phonic tics must be present for at least 1
year. Neuroimaging 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. However, TS is
a clinical diagnosis. There are no blood tests or other
laboratory tests that definitively diagnose the disorder.
Studies show that correct diagnosis of TS is frequently delayed
after the start of symptoms because many physicians may not be
familiar with the disorder. The behavioral symptoms and tics are
easily misinterpreted, often causing children with TS to be
misunderstood at school, at home, and even in the doctor's
office. Parents, relatives, and peers who are unfamiliar with
the disorder may incorrectly attribute the tics and other
symptoms to psychological problems, thereby increasing the
social isolation of those with the disorder. And because tics
can wax and wane in severity and can also be suppressed, they
are often absent during doctor visits, which further complicates
making a diagnosis. In many cases, parents, relatives, friends,
or even the patients themselves become aware of the disorder
based on information they have heard or read in the popular
media. How is TS treated?
Because symptoms do not impair most patients and development
usually proceeds normally, the majority of people with TS
require no medication. However, medications are available to
help when symptoms interfere with functioning. Unfortunately,
there is no one medication that is helpful to all persons with
TS, nor does any medication completely eliminate symptoms; in
addition, all medications have side effects. Instead, the
available TS medications are only able to help reduce specific
symptoms. Some patients who require medication to reduce the
frequency and intensity of the tic symptoms may be treated with
neuroleptic drugs such as haloperidol and pimozide .
These medications are usually given in very small doses that are
increased slowly until the best possible balance between
symptoms and side effects is achieved. Recently scientists have
discovered that long-term use of neuroleptic drugs may cause an
involuntary movement disorder called tardive dyskinesia .
However, this condition usually disappears when medication is
discontinued. Short-term side effects of haloperidol and
pimozide include muscular rigidity, drooling, tremor, lack of
facial expression, slow movement, and restlessness. These side
effects can be reduced by drugs commonly used to treat
Parkinson's disease. Other side effects such as fatigue,
depression, anxiety, weight gain, and difficulties in thinking
clearly may be more troublesome.
Clonidine , an antihypertensive drug, is also used in the
treatment of tics. Studies show that it is more effective in
reducing motor tics than reducing vocal tics. Fatigue, dry
mouth, irritability, dizziness, headache, and insomnia are
common side effects associated with clonidine use.
Fluphenazine and clonazepam may also be prescribed to help
control tic symptoms. Medications are also available to treat
some of he associated behavioral disorders. Stimulants such as
methyphenidate, pemoline , and dextroamphetamine , usually
prescribed for attention deficit-hyperactivity disorders,
although somewhat effective, have also been reported to increase
tics; therefore their use is controversial. For obsessive
compulsive behaviors that significantly disrupt daily
functioning, fluoxetine , clomipramine , sertraline , and
paroxetine may be prescribed.
Other types of therapy may also be helpful. Although
psychological problems do not cause TS, psychotherapy may help
the person better cope with the disorder and deal with the
secondary social and emotional problems that sometimes occur.
Psychotherapy does not help suppress the patient's tics.
Relaxation techniques and biofeedback may be useful in
alleviating stress which can lead to an icrease in tic symptoms.
Is TS inherited?
Evidence from genetic studies suggests that TS is inherited in a
dominant mode and the gene(s) involved can cause a variable
range of symptoms in different family members. A person with TS
has about a 50-50 chance of passing on the gene(s) to one of his
or her offspring. However, that genetic predisposition may not
necessarily result in full-blown TS; instead, it may express
itself as a milder tic disorder or as obsessive compulsive
behaviors or possibly attention deficit-hyperactivity disorder
with few or no tics at all. It is also possible that the
gene-carrying offspring will not develop any TS symptoms. A
higher than normal incidence of milder tic disorders and
obsessive compulsive behaviors has been found in families of
individuals with TS.
Gender also plays an important role in TS gene expression. If
the gene-carrying offspring of a TS patient is male, then the
risk of developing symptoms is 3 to 4 times higher. However,
most people who inherit the gene(s) will not develop symptoms
severe enough to warrant medical attention. In some cases of TS,
inheritance cannot be determined. These cases are called
sporadic and their cause is unknown.
What is the prognosis? There is no cure for TS; however, the
condition in many individuals improves as they mature.
Individuals with TS can expect to live a normal life span.
Although the disorder is generally lifelong and chronic, it is
not a degenerative condition. TS does not impair intelligence.
Tics tend to decrease with age, enabling some patients to
discontinue using medication. In a few cases, complete remission
occurs after adolescence. Although tic symptoms tend to decrease
with age, it is possible that neuropsychiatric disorders such as
depression, panic attacks, mood swings, and antisocial behaviors
may increase.
For more information on neurological disorders or research
programs funded by the National Institute of Neurological
Disorders and Stroke, contact the Institute's Brain Resources
and Information Network (BRAIN) at: BRAIN P.O. Box 5801 Bethesda
, MD 20824 (800) 352-9424 http://www.ninds.nih.gov
Information also is available from the following organizations:
Tourette Syndrome Association
42-40 Bell Boulevard Suite 205 Bayside, NY 11361-2820
ts@tsa-usa.org http://tsa-usa.org
Tel: 718-224-2999 888-4-TOURET ( 486-8738 ) Fax: 718-279-9596
National Institute of Mental Health (NIMH) National Institutes
of Health, DHHS 6001 Executive Blvd. Rm. 8184, MSC 9663
Bethesda , MD 20892-9663
nimhinfo@nih.gov http://www.nimh.nih.gov
Tel: 301-443-4513/301-443-8431 (TTY) 866-615-NIMH (-6464) Fax:
301-443-4279
National Institute on Deafness and Other Communication Disorders
(NIDCD) National Institutes of Health, DHHS
31 Center Drive , Rm. 3C35 MSC 2320 Bethesda , MD 20892-2320
nidcdinfo@nidcd.nih.govhttp://www.nidcd.nih.gov Tel:
301-496-7243 301-241-1055 (TTD/TTY