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Frequently
Asked Questions
 What
is Tourette Syndrome?
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.
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
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.
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.
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 one 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.
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.
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.
©2001 TSAGW
All rights reserved.
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