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TOURETTE'S SYNDROME
Judith Margolin,
Psy.D and Lew Gantwerk,
Psy.D
Center for Applied Psychology
Graduate School of Applied and Professional Psychology
Rutgers University
In 1885, George Gilles de la Tourette's
clinical description of a condition exhibited by a French noblewoman and a
few other patients launched the first neurological investigation into a syndrome
that later came to bear his name. A few years later, Sigmund Freud described
a case whose multifaceted psychological disorder included tic symptoms. It
wasn't until the 1970's, however, that the two perspectives merged in the
neuropsychiatric study of the relationship between
brain and behavior. Tourette's Syndrome (TS) has
become "the model neuropsychiatric disorder for
studying the interacting contributions of genetic vulnerability and varied
experiences, from gestation through the course of the first years of life,
in the shaping of the lines of personal development of the individual and
the nature and severity of a psychiatric disorder" (Mayes & Cohen, 1996b,
in Leckman & Cohen,
1999, p.3). Today, TS is viewed as a neuropsychiatric,
neurobehavioral disorder typified by involuntary movements and vocal sounds
called tics.
Diagnostic Criteria
For a diagnosis of TS, both motor
and vocal tics must be present at some time during the course of the disorder,
although not necessarily concurrently. Although at times resembling purposeful
behavior, a tic is actually an involuntary, sudden, rapid, stereotypic, non-rhythmic
motor movement or vocalization. The symptoms first become evident in childhood,
before the age of 18, with onset most commonly occurring around 6 or
7 years of age. Tics may occur from many times daily, nearly everyday to
intermittently throughout the period of one year, with no tic free
period lasting for more than three consecutive months. Within the same individual
there may be a waxing and waning of symptoms, with the number, frequency,
complexity, type and severity of tics changing over time. The disturbance
causes marked distress or significant impairment in social, occupational,
or other areas of functioning, and can be explained by no other medical condition
(DSM-IV). Environmental circumstances, such as stress, anxiety, excitement
or fatigue, often cause an exacerbation of the symptoms, and a decrease in
the child's ability to suppress the tics. Tics can be suppressed for varying
periods of time, but suppression may reduce the ability to concentrate and
focus on other things. Suppression often results in more intense outbursts
of tics.
Tics are described as either simple
or complex. Examples of simple motor tics include, but are not limited to,
blinking, eye rolling, grimacing, head jerks, shoulder shrugs, finger tapping,
licking lips, and scratching. Eye blinking, head jerking and shoulder shrugging
are the most common simple motor tics. More complex motor tics may include
jumping, repetitive touching, smelling, rubbing, shaking, echopraxia,
or multiple sequential movements. Simple vocal tics include, but are not
limited to, throat clearing, grunting, sniffing, snorting, barking, clicking,
and moaning, with the most common being sniffing and throat clearing. More
complex vocal tics include singing, whistling, humming, echolalia, palilalia, or coprolalia. Echolalia
(repetition of other's words), palilalia (repetition words or phrases with increasing rapidity),
and coprolalia (the offensive use of remarks or
obscenities) are erroneously considered primary symptoms for the diagnosis
of TS. In fact, these complex tics are generally quite rare, and occur in
less than 10% of the TS population.
Co-Morbid Disorders
TS is a neuropsychological disorder that is frequently accompanied
by other disorders. Attention Deficit Hyperactivity Disorder
(ADHD), Obsessive Compulsive Disorder (OCD), Learning Disabilities
(LD), and Repeated anger generated episodes (RAGE) occur
most often with TS. Less common co-morbid disorders include
non- OCD anxiety disorders, mood disorders, personality disorders,
sleep disorders and self-injurious and antisocial behavior.
Delays have also been noted in the development of peer relations
and social adaptation.
ADHD
Individuals with TS frequently present with symptoms of distractibility,
impulsivity, and hyperactivity. Co-morbid presentation of
attention deficit hyperactivity disorder often exceeds 50%
in the clinical population. ADHD may best be viewed
as a delay in the development of the inhibition of behavior
(Barkley, 1995). The symptoms of ADHD and their impact on
learning in the classroom, and functioning in the world, may,
in fact, be more disabling for persons with TS than even the
tics themselves (Walkup, et al., 1999). The prevalence of
learning disabilities in those with TS may be more closely
associated to ADHD and other factors, rather than to the TS
itself. "The literature on LD in Tourettes syndrome suggests
there is an increased risk for school related problems, but
the nature of these problems is multifactorial" (Walkup, et
al., 1999, p. 76).
OCD
Obsessive-compulsive behaviors, anxiety and mood disorders
may be an equaling distressing source of impairment for those
with TS. "Obsessive-compulsive symptoms can accompany TS,
or be present in family members, even in the absences of tics.
These symptoms may blend with the tics, making the boundary
between these symptom domains indistinct and arbitrary" (Leckman
& Cohen, 1999). OCD is characterized by recurring thoughts,
impulses or images that are experienced as intrusive, inappropriate
and cause distress, and are not simply excessive worries about
real life problems, and repetitive behaviors or mental acts
that a person feels compelled to perform according to a rigid
set of rules in order to reduce distress or prevent some dreaded
event (DSM IV). Persons with TS often have a subclinical
level of OCD, with significant obsessions and compulsions
that do not cause enough impairment or distress to meet diagnostic
criteria. In addition, certain groupings of obsessive-compulsive
symptoms are more common in individuals with TS (worries with
violent and aggressive themes or about symmetry and exactness,
and compulsions involving ordering, arranging, counting touching,
doing/redoing). Individuals with TS only occasionally exhibit
contamination worries or cleaning compulsions (King, et al.,
1999). Both tics and compulsions are associated with a preliminary
perceptual awareness and the Ôjust right' phenomena. Something
in the environment is perceived as Ônot right', and a behavior
must be performed until that something sounds, looks or feels
Ôright'. In attempting to distinguish between a tic and a
compulsion, tics have been described as a movement prompted
by a bodily sensation (an itch or the buildup of physical
energy), while a compulsion is preceded by a mental sensation
(a want or the buildup of emotional energy) (King, 1999).
Both phenomena present a challenge to the individual's sense
of self, and sense of control.
Repeated anger generated episodes (RAGE)
Repeated anger generated episodes (RAGE) occur in a significant
number of individuals with TS. These unpredictable, primitive
and extreme explosive outbursts are preceded by a sense of
increasing tension, accompanied by loss of control, and followed
by a feeling of relief once the attack has ended. Children
who exhibit these explosive outbursts often are characterized
as rigid (limited capacity for cognitive flexibility), concrete
(limited repertoire of adaptive skills), reactive (extremely
low frustration tolerance) and overwhelmed (easily overstimulated).
These RAGE attacks, as compared to temper tantrums, occur
in older children (the behavior is no longer age appropriate),
are more severe in magnitude and may be accompanied by significant
damage to property, and are not triggered by an obvious frustration
or precipitant stressor (Budman, et. al., 2000).
Peer relationships and Social Adaptation
Peer relationships and social adaptation may be impaired
in individuals with TS. Many children exhibit delays in their
social-emotional development. Factors within the child (tics,
ADHD, OCD, self esteem, temperament) and in the environment
(home, school, community) reciprocally influence one another
over the course of their development. If children are the
objects of prejudice, stigmatization, and teasing, peer acceptance
and social relationships can be compromised. As with most
children, children with TS do best when they feel good about
themselves, have warm and supportive relationships with their
family, have friendships and a sense of humor, and are not
encumbered by obstacles to learning and social interaction
(Dykens, et al., 1999). The existence of TS, and/or TS and
one or more co-morbid disorders may increase the risk of developing
social-emotional difficulties.
Treatment
A diagnosis of Tourette's Syndrome has implications for the
child's functioning in the physical, emotional, cognitive
and social domains, and impacts on relationships at home,
in school, and in the wider community. It is essential to
address all these needs in a multifaceted, multidisciplinary
fashion once intervention is indicated.
Once persistent symptoms appear,
formal diagnosis by a physician may be required and desired. Pharmacological
treatments are available to help manage tics and co-morbid symptoms and improve
the quality of the child's life. The use of medication is a very individual
and personal decision for many families may have side effects and should be
evaluated carefully with the physician.
Schools are becoming increasingly
more aware of Tourette's Syndrome, and are beginning to address the impact on the child's
learning. "Understanding TS is the first step to dealing with it appropriately
in the classroom settingÉand is the key to demystifying it" (Connors, 2002).
Educating school personnel, faculty, and the child's peers about Tourette's Syndrome and its associated disorders aids in reducing the
prejudice, stigmatization and teasing which often arise out of ignorance about
the disorder, and helps the child have a successful school experience. School
in-service programs, educational materials, films are available to assist
in this process. Specific interventions and accommodations may need to be
implemented in the classroom in order to facilitate the child's learning and
promote appropriate behavior. Individual strategies and accommodations depend
upon the degree to which the tics and/or associated behaviors interfere with
the child's ability to learn, and are similar to those used in any classroom.
Suggestions for school based interventions are available on the Tourette's Syndrome Association website (www.tsa-usa.org).
The emotional and social needs
of the individual with TS and the family must also be addressed, beginning
with the initial period of diagnosis, and continuing through the daily management
of the TS and its associated disorders. There is wide variability in the
degree of impairment. TS can run the gamut from mild tics only to TS plus
co-morbid disorders plus RAGE behaviors, or anywhere in between (Cimring,
2003). As the goal of treatment is to facilitate the successful navigation
of developmental tasks, within the context of the TS diagnosis, it is necessary
to consider the degree to which TS interferes with the child's normal development
or effects the child and family when choosing treatment
options.
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This page was last updated
February 28, 2006
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