Tourette syndrome is an inherited neurological
disorder with onset in childhood, characterized by the presence
of multiple physical (motor) tics and at least one vocal tic; these
tics characteristically wax and wane. It is a co-morbid disorder
with Autism Spectrum Disorders such as autism
Tourette’s is defined as part of a spectrum of
tic disorders, which includes transient and chronic tics. It is
also called Tourette’s syndrome, Tourette’s disorder, Gilles de
la Tourette syndrome, GTS or the more common Tourette’s or TS.
Tourette’s was once considered a rare and bizarre
syndrome, most often associated with the exclamation of obscene
words or socially inappropriate and derogatory remarks (coprolalia).
However, this symptom is present in only a small minority of people
with Tourette’s. Tourette’s is no longer considered a rare condition,
but it may not always be correctly identified because most cases
are classified as mild cases.
Since the incidence may be as high as one in a hundred people, up
to 530,000 U.S. school-age children may have Tourette’s, with the
more common tics of eye blinking, coughing, throat clearing, sniffing,
and facial movements. People with Tourette’s have normal life expectancy
and intelligence. The severity of the tics decreases for most children
as they pass through adolescence, and extreme Tourette’s in adulthood
is a rarity. Notable individuals with Tourette’s are found in all
walks of life.
Genetic and environmental factors each play a
role in the etiology of Tourette’s, but the exact causes are unknown.
In most cases, medication is unnecessary. There is no effective
medication for every case of tics, but there are medications and
therapies that can help when their use is warranted. Explanation
and reassurance alone are often sufficient treatment; education
is an important part of any treatment plan.
Characteristics of Tourette syndrome
Tics are sudden, repetitive, stereotyped, non-rhythmic,
involuntary movements and utterances that involve discrete muscle
groups. Motor tics are movement-based tics, while vocal tics are
involuntary sounds produced by moving air through the nose, mouth,
The most typical age of onset is from five to seven. The ages of
highest tic severity are eight to twelve (average ten), with tics
steadily declining for most patients as they pass through adolescence.
The most common, first-presenting tics are eye blinking, facial
movements, sniffing and throat clearing. Initial tics present most
frequently in midline body regions where there are many muscles,
usually the head, neck and facial region. This can be contrasted
with the stereotyped movements of other disorders (such as stims
and stereotypes of the Autism Spectrum Disorders), which typically
have an earlier age of onset, are more symmetrical, rhythmical and
bilateral, and involve the extremities (e.g., flapping the hands).
Tics that appear early in the course of the condition are frequently
confused with other conditions, such as allergies, asthma, and vision
problems: pediatricians, allergists and ophthalmologists are typically
the first to see a child with tics.
Unlike tics in other disorders, the tics of Tourette’s
are temporarily suppressible and preceded by a premonitory urge.
Immediately preceding tic onset, most individuals with Tourette’s
are aware of an urge that is similar to the need to sneeze or scratch
an itch. Individuals describe the need to tic as a buildup of tension
which they consciously choose to release, as if they “had to do
it”. Children may be less aware of the premonitory urge associated
with tics than are adults, but their awareness tends to increase
Causes of Tourette syndrome
The exact cause of Tourette’s is unknown, but
it is well established that both genetic and environmental factors
are involved. Genetic studies have proved that the overwhelming
majority of cases of Tourette’s are inherited, although the exact
mode of inheritance is not yet known, and no gene has been identified.
Non-genetic, environmental, infectious, or psychosocial factors
— while not causing Tourette’s — can influence its severity. Autoimmune
processes may affect tic onset and exacerbation in some cases. The
unproven and contentious hypothesis that Pediatric Autoimmune Neuropsychiatric
Disorders Associated with Streptococcal infections plays a role
in the onset of tic disorders and OCD is a current focus of research.
Diagnosis of Tourette syndrome
According to the fourth edition of the Diagnostic
and Statistical Manual of Mental Disorders (DSM-IV-TR), Tourette’s
Disorder may be diagnosed when a person exhibits both multiple motor
and one or more vocal tics (although these do not need to be concurrent)
over the period of a year, with no more than three consecutive tic-free
months. Previous editions of the DSM included a requirement for
“marked distress or significant impairment in social, occupational
or other important areas of functioning”, but this requirement was
removed in the most recent update of the manual, in recognition
that clinicians see patients who meet all the other criterion for
Tourette’s, but do not have distress or impairment.
Treatment of Tourette syndrome
The treatment of Tourette’s focuses on identifying
and helping the individual manage the most troubling or impairing
symptoms. Most cases of Tourette’s are mild, and do not require
pharmacological treatment; instead, psychobehavioral therapy, education,
and reassurance may be sufficient. Relaxation techniques, such as
exercise, yoga or meditation, may be useful in relieving the stress
that may aggravate tics, but the majority of behavioral interventions
(such as relaxation training and biofeedback, with the exception
of habit reversal) have not been systematically evaluated and are
not empirically supported therapies for Tourette’s. Educating a
patient, family, and surrounding community (such as friends, school,
and church) is a key treatment strategy.
Treatments, where warranted, can be divided into those that target
tics and comorbid conditions, which, when present, are often a larger
source of impairment than the tics themselves. Not all people with
tics have comorbid conditions, but when those conditions are present,
such as Aspergers syndrome, they often take treatment priority.
There is no cure for Tourette’s and no medication that works universally
for all individuals without significant adverse effects. Clonidine
(or the clonidine patch) is one of the medications typically tried
first when medication is needed for Tourette’s. Haloperidol is an
antipsychotic medication sometimes used to treat severe cases of
Tourettes . Medication is available to help when symptoms interfere
The classes of medication with the most proven
efficacy in treating tics include typical and atypical neuroleptics,
antihypertensive agents and stimulants may be useful in treating
when it co-occurs with tic disorders.
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