Index:
- What
is Tourette Syndrome?
- What
are the symptoms?
- How
are tics classified?
- Can
people with TS control their tics?
- What
causes TS?
- What
disorders are associated with TS?
- How
is TS diagnosed?
- How
is TS treated?
- Is
TS inherited?
- What
is the prognosis?
- What
is the best educational setting for
children with TS?
- What
research is being done?
- Where
can I go for more information?
- Credits
Tourette syndrome
(TS) is an inherited, neurological disorder
characterized by repeated involuntary movements
and uncontrollable vocal (phonic) sounds called
tics. 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.
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.
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.
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.
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.
Not all people
with TS have disorders other than tics. However,
many people experience additional problems such
as obsessive compulsive behavior, where the
person feels that something must be done
repeatedly, such as hand washing or checking
that a door is locked; attention deficit
disorder, where the person has difficulty
concentrating and is easily distracted; learning
disabilities, which include reading, writing,
arithmetic, and perceptual difficulties;
problems with impulse control, which can result
in overly aggressive behaviors or socially
inappropriate acts; 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.
Generally, TS is
diagnosed by observing the symptoms 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.
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 the associated
behavioral disorders. Stimulants such as
methyphenidate, pemoline, and dextroamphetamine,
usually prescribed for attention deficit
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 increase
in tic symptoms.
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 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.
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.
Although students
with TS often function well in the regular
classroom, it is estimated that many may have
some kind of learning disability. When attention
deficit disorder, obsessive compulsive disorder,
and frequent tics greatly interfere with
academic performance or social adjustment,
students should be placed in an educational
setting that meets their individual needs. These
students may require tutoring, smaller or
special classes, and in some cases special
schools.
All students with
TS need a tolerant and compassionate setting
that both encourages them to work to their full
potential and is flexible enough to accommodate
their special needs. This setting may include a
private study area, exams outside the regular
classroom, or even oral exams when the child's
symptoms interfere with his or her ability to
write. Untimed testing reduces stress for
students with TS.
Within the
Federal Government, the leading supporter of
research on TS and other neurological disorders
is the National Institute of Neurological
Disorders and Stroke (NINDS). The NINDS, a part
of the National Institutes of Health (NIH), is
responsible for supporting and conducting
research on the brain and central nervous
system.
NINDS sponsors
research on TS both in its laboratories at the
NIH and through grants to major medical
institutions across the country. The National
Institute of Mental Health,the National Center
for Research Resources, the National Institute
of Child Health and Human Development, the
National Institute on Drug Abuse, and the
National Institute on Deafness and Other
Communication Disorders also support research of
relevance to TS.
Recent research
has led to several notable advances in the
understanding of TS. Already scientists have
learned that TS is inherited from a dominant
gene(s) that causes different symptoms from
patient to patient, and that the disorder is
more common than was previously thought.
Genetic studies.
Currently, investigators are conducting genetic
linkage studies in large multigenerational
families affected with TS in an effort to find
the chromosomal location of the TS gene(s).
Finding a genetic marker (a biochemical
abnormality that all TS patients might share)
for TS would be a major step toward
understanding the genetic risk factors for TS.
Once the marker is found, research efforts would
then focus on locating the TS gene(s).
Understanding the
genetics of TS will directly benefit patients
who are concerned about recurrence in their
families and will ultimately help to clarify the
development of the disorder. Localization of the
TS gene will strengthen clinical diagnosis,
improve genetic counseling, lead to the
clarification of pathophysiology, and provide
clues for more effective therapies.
Neurotransmitter
studies. Investigators continue to study certain
neurotransmitters to increase our understanding
of the syndrome, explore the role they play in
the disease process, and provide more effective
therapies.
Environmental
studies. Other research projects currently under
way include analyzing young unaffected children
at high risk for TS in order to identify
environmental factors such as life stresses or
exposure to certain medications that may
influence the expression of the disorder.
Scientists are
also conducting neuropsychological tests and
neuroimaging studies of brain activity and
structure to determine the extent to which
specific environmental exposures may affect the
emergence of tics and/or obsessive compulsive
symptoms.
For more
information about TS or other neurological
disorders, or about the NINDS and its research
programs, contact the NINDS Office
of Communications and Public Liaison.
The Tourette
Syndrome Association, Inc., is a voluntary,
nonprofit organization whose members include
people with TS, their families and friends, and
health care professionals. The Association funds
research, provides services to patients and
their families, and offers a variety of
publications, including a newsletter, brochures,
and fact sheets. For further information,
contact:
Tourette
Syndrome Association, Inc.
42-40 Bell Boulevard
Bayside, New York 11361-2861
(718) 224-2999
(800) 237-0717
* http://tsa.mgh.harvard.edu/
Credits:
This document was
originally prepared by the:
Office of
Communications and Public Liaison
National Institute of Neurological Disorders and
Stroke
National Institutes of Health
Bethesda, MD 20892
NIH Publication No. 95-2163
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