A brief description
Tourette Syndrome is an inherited, neurological disorder
characterized by repeated and involuntary body movements
(tics) and uncontrollable vocal sounds. In a minority of
cases, the vocalizations can include socially inappropriate
words and phrases -- called coprolalia. These outbursts are
neither intentional nor purposeful. Involuntary symptoms
can included eye blinking, repeated throat clearing or
sniffing, arm thrusting, kicking movements, shoulder
shrugging or jumping.
These and other symptoms typically appear before the age of
18 and the condition occurs in all ethnic groups with males
affected 3 to 4 times more often than females. Although the
symptoms of TS vary from person to person and range from
very mild to severe, the majority of cases fall into the
mild category. Associated conditions can include
obsessivity, attentional problems and impulsiveness.
Most people with TS lead productive lives and participate
in all professions. Increased public understanding and
tolerance of TS symptoms are of paramount importance to
people with Tourette Syndrome.
Questions and Answers about Tourette Syndrome
Q. What is Tourette Syndrome?
A. Tourette Syndrome (TS) is a neurological disorder
characterized by tics -- involuntary, rapid, sudden
movements or vocalizations that occur repeatedly in the
same way.
The symptoms include:
Both multiple motor and one or more vocal tics present at
some time during the illness although not necessarily
simultaneously;
The occurrence of tics many times a day (usually in bouts)
nearly every day or intermittently throughout a span of
more than one year; and
Periodic changes in the number, frequency, type and
location of the tics, and in the waxing and waning of their
severity. Symptoms can sometimes disappear for weeks or
months at a time.
Onset before the age of 18.
The term, "involuntary," used to describe TS tics is
sometimes confusing since it is known that most people with
TS do have some control over their symptoms. What is not
recognized is that the control, which can be exercised
anywhere from seconds to hours at a time, may merely
postpone more severe outbursts of symptoms. Tics are
experienced as irresistible and (as the urge to sneeze)
eventually must be expressed. People with TS often seek a
secluded spot to release their symptoms after delaying them
in school or at work. Typically, tics increase as a result
of tension or stress, and decrease with relaxation or
concentration on an absorbing task.
Q. How would a typical case of TS be described?
A. The term typical cannot be applied to TS. The
expression of symptoms covers a spectrum from very mild to
quite severe. However, the majority of cases are in the
mild category.
Q. What causes the symptoms?
A. The cause has not been established, although current
research presents considerable evidence that the disorder
stems from the abnormal metabolism of several brain
chemicals (neurotransmitters) such as dopamine and
serotonin.
Q. How is TS diagnosed?
A. A diagnosis is made by observing symptoms and by
evaluating the history of their onset. No blood analysis or
other types of neurological tests exist to diagnose TS.
However, some physicians may wish to order an EEG, MRI, CAT
scan, or certain blood tests to rule out other ailments
that might be confused with TS.
Q. What are the first symptoms?
A. The most common first symptom is a facial tic such as
rapidly blinking eyes or twitches of the mouth. However,
involuntary sounds such as throat clearing and sniffing, or
tics of the limbs may be initial signs.
Q. How are tics classified?
A. There are two categories of tics: motor and vocal. Both
of these are then subdivided into simple and complex.
Simple:
Motor-- Eye blinking, head jerking, shoulder shrugging and
facial grimacing.
Vocal-- Throat clearing, yelping and other noises, sniffing
and tongue clicking.
Complex:
Motor-- Jumping, touching other people or things, smelling,
twirling about, and only rarely self-injurious actions
including hitting or biting oneself.
Vocal-- Uttering words or phrases out of context and
coprolalia (vocalizing socially unacceptable words).
The range of tics is very broad. Some symptoms are often so
complex that family members, friends, teachers and
employers may find it hard to believe that the movements
and vocalizations are involuntary.
Q. How is TS treated?
A. The majority of people with TS are not significantly
disabled by their tics or behavioral symptoms, and
therefore do not require medication. However, there are
medications available to help control the symptoms when
they interfere with functioning. The drugs include
haloperidol (Haldol), clonidine (Catapres), pimozide
(Orap), fluphenazine (Prolixin, Permitil), and clonazepam
(Klonopin). Stimulants such as Ritalin, Cylert, and
Dexedrine that are prescribed for ADHD may increase tics.
Their use is controversial. For obsessive compulsive traits
that interfere significantly with daily functioning,
fluoxetine (Prozac), clomipramine (Anafranil), sertraline
(Zoloft), fluvoxamine (Luvox) and paroxetine (Paxil) are
prescribed. Risperidone (Risperdal) is a newer medication
that is also being prescribed.
Dosages which achieve maximum control of symptoms vary for
each patient and must be gauged carefully by a doctor. The
medicine is administered in small doses with gradual
increases to the point where there is maximum alleviation
of symptoms with minimal side effects. Some of the
undesirable reactions to medications are weight gain,
muscular rigidity, fatigue, motor restlessness and social
withdrawal, most of which can be reduced with specific
medications. Side effects such as depression and cognitive
impairment can be alleviated with dosage reduction or a
change of medication.
Other types of therapy may also be helpful. Psychotherapy
can assist a person with TS and help his/her family cope,
and some behavior therapies can teach the substitution of
one tic for another that is more acceptable. The use of
relaxation techniques, biofeedback and excercise can reduce
the stress that often exacerbates tic symptoms.
Q. Is it important to receive a TS diagnosis early in life?
A. Yes, especially in those instances when the symptoms
are viewed by some people as bizarre, disruptive and
frightening. Sometimes TS symptoms provoke ridicule and
rejection by peers, neighbors, teachers and even casual
observers. Parents may be overwhelmed by the strangeness of
their child's behavior. The child may be threatened,
excluded from activities and prevented from enjoying normal
interpersonal relationships. These difficulties may become
greater during adolescence -- an especially trying period
for young people and even more so for a person coping with
a neurological problem. To avoid psychological harm, early
diagnosis and treatment are crucial. Moreover, in more
serious cases, it is possible to control the symptoms with
medication.
Q. Do all people with TS have associated behaviors in
addition to tics?
A. No, but many do have one or more additional problems
which may include:
Obsessions which consist of repetitive unwanted or
bothersome thoughts.
Compulsions and Ritualistic Behaviors are when a person
feels that something must be done over and over and/or in a
certain way. Examples include touching an object with one
hand after touching it with the other hand to "even things
up" or repeatedly checking to see that the flame on the
stove is turned off. Children sometimes beg their parents
to repeat a sentence many times until it "sounds right."
Attention Deficit Disorder with or without Hyperactivity
(ADD or ADHD) occurs in many people with TS. Children may
show signs of hyperactivity before TS symptoms appear.
Indications of ADHD may include: difficulty with
concentration; failing to finish what is started; not
listening; being easily distracted; often acting before
thinking; shifting constantly from one activity to another;
needing a great deal of supervision; and general fidgeting.
Adults too may exhibit signs of ADHD such as overly
impulsive behavior and concentration difficulties and the
need to move constantly. ADD without hyperactivity includes
all of the above symptoms except for the high level of
activity. As children with ADHD mature, the need to move is
more likely to be expressed by restless, fidgety behavior.
Difficulties with concentration and poor impulse control
persist.
Learning Disabilities may include reading and writing
difficulties, arithmetic disorders and perceptual problems.
Difficulties with impulse control which may result, in rare
instances, in overly aggressive behaviors or socially
inappropriate acts. Also, defiant and angry behaviors can
occur.
Sleep Disorders are fairly common among people with TS.
These include frequent awakenings or walking or talking in
one's sleep.
Q. Do students with TS have special educational needs?
A. While school children with TS as a group have the same
IQ range as the population at large, many have special
educational needs. It is estimated that many may have some
kind of learning problem. That condition, combined with
attention deficits and the problems inherent in dealing
with frequent tics, often call for special educational
assistance. The use of tape recorders, typewriters, or
computers for reading and writing problems, untimed exams
(in a private room if vocal tics are a problem), and
permission to leave the classroom when tics become
overwhelming are often helpful. Some children need extra
help such as access to tutoring in a resource room.
When difficulties in school cannot be resolved, an
educational evaluation may be indicated. A resulting
identification as "other health impaired" under federal law
will entitle the student to an Individual Education Plan
(IEP) which addresses specific educational problems in
school. Such an approach can significantly reduce the
learning difficulties that prevent the young person from
performing at his/her potential. The child who cannot be
adequately educated in a public school with special
services geared to his/her individual needs may be best
served by a special school.
Q. Is TS inherited?
A. Genetic studies indicate that TS is inherited as a
dominant gene (or genes) causing different symptoms in
different family members. A person with TS has about a 50%
chance of passing the gene to one of his/her children with
each separate pregnancy. However, that genetic
predisposition may express itself as TS, as a milder tic
disorder or as obsessive compulsive symptoms with no tics
at all. It is known that a higher than normal incidence of
milder tic disorders and obsessive compulsive behaviors
occurs in the families of TS patients.
The sex of the child also influences the genetic expression
of the condition. The chance that the gene-carrying child
of a person with TS will have symptoms is at least three to
four times higher for a son than for a daughter. Yet only
about 10% of the children who inherit the genetic
predisposition will have symptoms severe enough to ever
require medical attention. In some cases TS may not be
inherited, and is identified. Those cases are called
sporadic TS and the cause is unknown.
Q. Is there a cure?
A. Not yet.
Q. Is there ever a remission?
A. Many people experience marked improvement in their late
teens or early twenties. Most people with TS get better,
not worse, as they mature, and those diagnosed with TS have
a normal life span. As many as 1/3 of patients experience
remission of tic symptoms in adulthood.
Q. How many people in the U.S. have TS?
A. Since many people with TS have yet to be diagnosed,
there are no absolute figures. The official estimate by the
National Institutes of Health is that 100,000 Americans
have full-blown TS. Some genetic studies suggest that the
figure may be as high as one in two hundred if those with
chronic multiple tics and/or transient childhood tics are
included in the count.