Tourette
Syndrome
What
is it?
Tourette syndrome (TS) is an
inherited disorder of the nervous system.
It is named for the French neurologist Dr.
George Gilles de la Tourette, who first described
it. TS
is characterized by motor and vocal tics, which
are involuntary, sudden repeated movements or
sounds. Symptoms
usually appear in early childhood.
Who
gets it?
TS is hereditary. If one
parent has TS, each child has a 50% chance of
inheriting the abnormal gene.
It is three times more common in males than in females.
What
causes it?
The exact cause of TS isn’t
known.
However, research has shown that it appears to be related to
a defect in the way the brain produces or uses the
neurotransmitters dopamine and serotonin.
Neurotransmitters control
how signals are sent along
the nerve cells. The
neurotransmitter dopamine sends signals to the
brain to initiate movement. Research
has shown that the receptors in the brain may be
exceptionally sensitive in people with TS, taking
any slight sign from dopamine as a reason to move.
This results in tics. Other
research indicates that tics are related to higher
than normal levels of dopamine production and use.
What
are the symptoms?
TS symptoms usually
begin around 7 years of age, and usually before
age 14. However,
symptoms may appear as early as one year of age.
TS symptoms usually begin with a
facial tic, such as rapid eye blinking or mouth
twitches. Other
initial symptoms often include sniffing, throat
clearing, or jerky movements of the arms,
shoulders, or legs. Tics
are classified as motor or vocal, and the range of
tics is very broad.
Motor tics can include head jerking, foot
stomping, shoulder shrugging, grimacing, rapid
finger movements, teeth clicking, rolling the
eyes, and twirling about.
In severe cases, patients may bang their
heads, writhe around, and bite.
Vocal tics can include barking, shrieking,
snorting, grunting, hissing, and tongue clicking.
Children may repeat phrases, such as
“okay, okay, okay.”
Echolalia is a vocal tic where people
repeat, or echo, the words spoken to them by
others. Less
than one-third of TS patients have an irresistible
urge to curse or use offensive words, which is
called coprolalia.
While tics are involuntary, people with TS
may be able to control their symptoms anywhere
from seconds to hours at a time. However, tics are
very strong urges, and eventually, there is an
uncontrollable outburst of tics.
Tics follow a cycle of waxing and waning, and tend to become
worse during stressful times.
This may explain why tics tend to increase
in children with TS during the school year, but
subside during the summer months.
Except in very severe cases, tics are
usually much less of a problem during sleep.
There are a number of additional disorders
that have been associated with TS.
These include Obsessive-Compulsive disorder (OCD), Attention Deficit Disorder with or without
Hyperactivity (ADD/ADHD), Oppositional Defiant
Disorder (ODD), learning disabilities, difficulty
controlling impulses, and sleep disorders.
Not all people with TS will have one of these associated
disorders. Generally,
TS does not impair thinking or intelligence.
How
is it diagnosed?
Your physician can diagnose
TS by observing the symptoms and asking questions
about any family history of TS or a related
disorder. He
or she may refer you to a pediatric neurologist, a
doctor who specializes in neurological disorders
in children. To qualify as TS, both motor and
vocal tics must be present for at least one year
and should begin before age 21. There
are no specific tests for TS, but some physicians
may order tests of the brain’s electrical
activity (EEG), magnetic resonance imaging (MRI)
of the brain, or certain blood tests to rule out
other causes. A diagnosis of Tourette’s is often
delayed because parents and physicians don’t
understand or recognize the symptoms.
Early blinking or sniffing tics are often
blamed on vision problems and allergies, or
dismissed as “just a habit.”
It is important for parents and teachers to
recognize the symptoms of TS so they do not to
punish children for behaviors that they cannot
control. It
is also important to look at any medication
history.
Certain stimulant drugs my cause tics or aggravate TS
symptoms. Once
TS is diagnosed, your physician will classify the
symptoms as mild, moderate, or severe, and
formulate a treatment plan. The progression and
types of symptoms are unpredictable.
What
is the treatment?
In mild to moderate cases,
many TS patients do not need any drug treatment.
Medications are recommended only when
symptoms interfere with the patients ability to
function normally on a day-to-day basis, or when
the symptoms have an effect on a child’s
self-esteem.
Drugs commonly used in the treatment of TS
include haloperidol (Haldol) or pimozide (Orap).
Each carries a risk of potentially serious side effects, and
is started at a very low dose, then increased
until the tics respond. Drugs
that are used to treat high blood pressure, such
as clonidine and guanfacine, seem to work well in
treating motor tics, but may not be as effective
with vocal tics.
Side effects are less of a problem with these drugs, and they
seem to help children with focus and anxiety
problems. Medications
for TS should never be stopped suddenly, but
tapered off gradually.
Your doctor may recommend increasing
medications when tics become more severe, then
decreasing in times when tics are quieter.
Most children with TS function well in a
regular classroom.
In fact, their classmates are usually
unaware of the condition.
However, children with severe TS may need to be in smaller,
specialized classes that can better meet their
needs.
While psychotherapy will not help to suppress tics, it is
often recommended to help the child cope with the
disorder.
Therapies that encourage relaxation, such as yoga and
biofeedback, may also be beneficial.
Some parents report improvements in
symptoms through vitamin and dietary changes.
You should consult your physician before
trying any therapies that involve supplements or
changes in diet, especially with children. While
there is no cure for TS, many patients find that symptoms
improve as they grow older.
In fact, a small percentage recover
completely after their teenage years.
Self-care
tips
At this time, there is no way
to prevent TS.
Couples in which one or both individuals
have TS may want to consider the odds of passing
the condition on to a child before deciding to
have children.
If you or your child has TS, it’s
important to develop a good support system, and to
be patient and understanding.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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