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Transcript of Tourette Syndrome[1]
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Tourette Syndrome
Running head: TOURETTE SYNDROME
Literature Review of Tourette Syndrome with Particular Emphasis Towards Children and
Treatments
Jessica R. Dettmann
Andrews University
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Tourette Syndrome
Abstract
Tourette syndrome is a common childhood onset neurological developmental
disorder, consisting of multiple motor tics and one or more vocal tics. The disorder affects
around 1% of school-aged children with males being considerably more likely to have the
disorder than females. In the disorder, there is a high rate of comorbidity with other
psychopathologies, especially attention deficit disorder and obsessive-compulsive disorder.
The predominant treatment for this disorder is medication; however, other treatments such
as family therapy, neurofeedback and awareness training are helpful in the treatment
process. Because Tourette syndrome affects multiple aspects of the child and their
families lives, the best treatment plan utilizes a multimodal approach.
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Tourette Syndrome
Literature Review of Tourette Syndrome with Particular Emphasis Towards Children and
Treatments
Tic disorders include chronic motor or vocal tic disorders and Tourettes disorder
(syndrome). According to the contemporary view, tic disorders result from developmental
neurological abnormalities. The characteristics of tic disorders include simple to complex
motor movements and or vocalizations. Tourette Syndrome (TS) is the most serious of
these tic disorders. TS usually begins in early childhood with the presentation of simple
motor tics (e.g. eye blinking or head jerking) and progresses with age towards the inclusion
of vocal tics (e.g. throat clearing or barking) and complex motor tics (e.g. brushing hair).
The tics occur intermittently and can change in their presentation throughout the course of
the disorder. Generally, the tics start out as only a mild disturbance and increase in
intensity and frequency as the child reaches middle school age. TS not only affect the
individual with the condition, it also affects their family and other contacts.
History
Tourette syndrome was first defined by French neurologist Jean-Marc Itard who
worked with a noblewoman in 1825 (Karadenizli, Dilbaz & Bayam, 2005; Neuroscience
for Kids, 2007). Later in 1885, Gilles de la Tourette became fascinated with the syndrome
and wrote a detailed clinical account of several patients including Itards work with the
noblewoman. Gilles de la Tourettes work launched the neurological investigation into the
nature of tics. Around this same time, Freud investigated the nature and origin of tic
symptoms in hysteria patients. He wrote a detailed report on his treatment of Frau Emmy
von N whose disorder included dramatic tics. Freuds work created interest in the
psychoanalytic investigation of the psychology of the inner world. Gilles de la Tourettes
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neurological view, focusing on the brain and Freuds psychological view focusing on the
mind, were predominate historical views on TS. Despite the contact of these views, they
were hard to integrate because of their divergent philosophies (Leckman & Cohen, 1999).
Around the 1970s the neurological and psychological views started to integrate
into the new field of neropsychiatric disorders (Leckman & Cohen, 1999). Most of the
current research on TS utilizes brain imaging, and heredity studies. The current view of TS
is that it is an inherited neurobiological disorder resulting from an abnormality in brain
structure, genes and/or neurotransmitters.
Diagnosis and Characteristics
The following is a description, taken from Tourette Syndrome (2007), of the
symptoms experienced by a gentleman who suffered from TS.
When I was nine-years old, an imp took up residence in me. One afternoon
he prodded the left side of my face from the inside, causing my lips to purse
and curl askew toward my squinting left eye. Without yet knowing why, I
rapidly blinked and shrugged. I grunted. I threw back my head and
squeaked while my fists smacked my bruised abdomen.
This description gives us an idea of how the tic symptoms of TS present
themselves. It also gives a glimpse into how children can easily obtain misconceptions
concerning the reasons for their disorder.
Diagnosis
In the diagnosis of TS, it is very important to understand the characteristics of the
disorder. The diagnosis of TS can be complicating because the symptoms can vary from
case to case and can continually change throughout an individuals lifetime. To date, there
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are not any imaging abnormalities, neurophathological lesions at post-mortem or genetic
tests that have proven themselves reliable in aiding symptom based diagnosis (Robertson,
2000). However, researchers are making advances in this area of research.
According to the American Psychiatric Association (2004), the criteria for the
diagnosis of TS states that a person must have both multiple motor and one or more vocal
tics, these tics do not have to occur concurrently. However, they must occur many times a
day, nearly every day or intermittently throughout a period of more than a year, during
which there has not been a tic free period of more than 3 consecutive months. The onset
for the disorder must be before the age of 18 (previous versions of the DSM had initial
onset age as 21 years and under (Robertson, 2000)). In addition, physiological effects
occurring from substance abuse or a general medical condition must not be the reason for
the problem. Finally, the individual with TS must be experiencing significant distress
from the symptoms before the diagnosis is received (American Psychiatric Association,
2004).
Life Course of TS
Typically, TS begins in early childhood with the average age of onset being 6-7
years of age (Tourette Syndrome, 2007). Berecz (1992) proposed that TS does not become
evident in children until they leave home for school or daycare, thus according to his theory
the age of onset would continue to decrease as children start school at younger ages. Thus,
it appears that the stress and expectations of a new environment evoke the TS symptoms.
During late adolescence to early adulthood, tic disorders usually improve. Motor
tics often decrease in both frequency and number and vocal tics become infrequent with the
possibility of disappearing altogether (Lewis, 2002). In most cases, the individual with this
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disorder will experience few symptoms from the disorder during their adulthood.
However, a few individuals continue to experience significant distress from the disorder
during adulthood.
The first sign of TS is usually the display of motor tics. A motor tic involves a
sudden, rapid, recurrent, nonrhythmic, and stereotyped motor movement (American
Psychiatric Association, 2004). The repertoire of motor tics can be vast, with the
incorporation of almost any voluntary movement by any portion of the body. (For
examples of motor tics, refer to appendix A). Leckman and Cohen (1999) suggest that
there is a rostal-caudal progression (i.e. tics progress from head neck shoulders
arms torso); however, this course progression is not predictable according to Lewis
(2002). As the course of TS progresses, complex motor tics may emerge. Recognition of
some complex is by their repetitive nature, this is because their appearances can be
disguised (e.g. brushing hair away from the face with an arm) (Lewis, 2002).
Trichotillomania, which involves pulling hair out, and Copraxia which involves making
obscene gestures, may be signs of obsessive-compulsive behaviors in the person with TS
(Dornbush & Pruitt, 1995).
Vocal tics usually do not manifest themselves until 1 to 2 years after the first
appearance of motor tics (Lewis, 2002). A vocal tic involves a sudden, rapid, recurrent and
nonrhythmic vocalization (American Psychiatric Association, 2004). (For examples of
vocal tics, refer to appendix B). Many individuals associate TS with the uttering of random
obscenities (copralalia), however, only 10-30% of individuals with TS suffer from
coprolalia (Blacher, 2002; Morison, 2006). Coprolalia, echolalia which involves
repeating what others say, and Palilalia which involves an individual repeating their own
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words, may be verbal indicators of the presence of obsessive compulsive behaviors in
individuals with TS (Dornbush & Pruitt, 1995).
For a comprehensive understanding concerning the nature of tics, the issue of
whether tics are voluntary or involuntary is addressed. According to research, tics
experienced are involuntary. This means that people with tics cannot decide to have their
tics stop or not occur (Tourettes Syndrome, 2007). Many individuals get confused over
this issue because some individuals with TS can control their tics for 1-2 hours, or in
certain situations. Not all individuals with TS can achieve the controlling of the tic impulse
for a short time-period. Even individuals who exhibit this control in most situations do not
have the control in all situations. Furthermore, the longer the individual suppresses the
urge the more serious the tics will be when they occur. Many individuals with TS report
experiencing ideational and/or somatic sensory symptoms prior to the urge to tic (Blacher,
2002; Leckman & Cohen, 1999). Premonitory urges such as needs, prickly feelings and
tension are present for 75-90% of adults with TS, with the onset of these premonitory urges
starting several years after the initial onset of tics (Leckman & Cohen).
Etiology
Current understandings of TS hold that it is a neurological disorder, rather than a
psychological disorder. The basis for this etiology is its receptiveness to medication. The
neurological problem in TS appears to result from a dysfunction in the dopaminergic
pathways (Preston, ONeal & Talaga, 2005).
Brain Imaging
New brain imaging studies (both functional and structural) have added to experts
knowledge of neural plasticity and the adaptation of the brain (Plessen, et al., 2006).
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Plessen, et al., states that studies of abnormalities of the brain in individuals with TS have
focused attention on the basal ganglia and its role in the cortico-striato-thalamo-cortical
circuits. These circuits appear to play a role in the underlying pathophysiology and
severity of tics. The cortical region appears to play a part in the modulation and
suppression of tics (Plessen, et. al.). The prefrontal regions are larger and the corpus
callosum area smaller in children and young adults who have TS. These differences in
anatomical features seem to reflect neural plasticity, which is involved in the severity of
tics (Plessen, et. al.).
Besides neurological problems in the etiology of TS, autoimmune mechanisms
appear to be related to the development of TS. The most common is strep infections and
viruses (Preston, ONeal & Talaga, 2006). In a study on the factors associated with
increased tic severity, Burd, Freeman, Klug and Kerbeshian (2006) found that perinatal
problems affect between 14.8% and 17.5% of children with TS.
Genetic Research
Genetic research has shown that TS has a greater prevalence among relatives of
individuals with TS and ADHD. This connection comes from research using twin adoption
and family studies.
Twin and family studies have shown that genetic factors play a role in the vertical
transmission within families of a vulnerability to TS and related tic disorders. Lewis
(2002) stated that the monozygotic twin partner of a twin with TS has a 50% chance of
suffering from TS. However, with dizygotic twins there is only a 10% chance that the
other twin will also have TS. These twin studies show that there is a definitely a genetic
connection involved in the transmission and acquisition of TS.
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Geneticists searched in hope of locating, a specific gene or genes that are involved
with TS. So far traditional linkage studies have not been able to pinpoint a specific
chromosome and gene, however, it has been suggested from research that there is a
linkage of TS to chromosomes 4q and 8p (Lewis, 2002).
Statistics and Comorbidty
Prevalence
Throughout most of the previous century TS was, considered a very rare disorder.
Clinicians had observed TS only a couple of times during their practice (Leckman &
Cohen, 1999). However, according to the Society for Neuroscience (2006) TS is currently
one of the most common neurobiological disorders. Currently estimates maintain that 1
million people suffer from tic symptoms, with 100,000 to 200,000 meeting the full criteria
for TS (Neuroscience for Kids, 2007). The Society for Neuroscience (2006) says that
roughly 200,000 Americans suffer from TS. Shavitt, Hounie, Campos and Miguel (2006)
reported that 1% of school aged children have TS, with 4 to 18% having some form of tic
disorder. Preston, ONeal, & Talaga (2006), say that TS affects 1 out of every 1000 to
2000 children. Males are three to four times more likely to have TS than females
according to (Robertson, 2000; Shavitt et al., 2006). According to Robertson (2000), there
is a high rate of TS in children receiving special education. In a two-year study conducted
in California, an estimated 12% of special education students had TS with 28% suffering
from tic disorders (Robertson, 2000). TS and its prevalence does not limit itself to a single
society, rather it exhibits itself across cultures (Robertson, 2006).
Comorbidity
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According to Gilbert and Buncher (2005), around 85% of individuals with TS have
one or more comorbid psychiatric disorder. Robertson (2006) found similar results in a
worldwide investigation of 3500 individuals with TS. He found that 88% of his
participants with TS also suffered from comorbid psychiatric disorders. Besides comorbid
psychiatric disorders children and adults with TS also suffer from behavioral problems and
negative thoughts and feelings.
Common comorbid disorders, behaviors and feelings that people with TS deal with
include; attention deficit hyperactivity disorder (ADHD) (Stewart, Illmann, Geller, King &
Pauls, 2006), obsessive compulsive behaviors (Robertson, 2000), Obsessive compulsive
disorder (OCD), Major depressive disorder (MDD) (Snijders, Robertson & Orth, 2006),
conduct disorder, oppositional defiant disorder, aggression and rage, antisocial behaviors,
sever temper outbursts, schizoid symptoms, inappropriate sexual behaviors (Robertson,
2000), learning disorders (Society for Neuroscience, 2006), autism (Canitano & Vivanti,
2007), sleep disorders, neurological disorders, mental retardation, social skill problems
(Burd et al., 2006), anxiety, anxiety disorders (Robertson, 2006) and self-mutilation
(Gadoth & Mass, 2004). ADHD and OCD are the most common of these comorbid
disorders (Robertson, 2006). The combination of comorbid disorders and TS has serious
implications for the implementation and outcome of treatments.
Researchers are searching to find the nature of the relationship between these
disorders and TS. Twin and family studies have shown that there is a higher prevalence of
TS in close family members of individuals with OCD (Johannes et al., 2001). Thus they
believe that there is a genetic relation between the two disorders. Research has also been
promising in showing a genetic relation between ADHD and TS. Depression has had
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inclusive evidence, as some studies claim that there is a genetic basis for the relation while
others have not reported this relation. Depression and anxiety related may stem from the
stigma and ostracism experienced from others and the inner turmoil that they experience
from trying to stop their TS behaviors (Robertson, 2006).
Differential Diagnosis
Common differential diagnoses for tic disorders are, Chorea, Dystonia, Myoclonus,
Dyskinesias, Akinesia, and Stereotypy (Leckman, Cohen, 1999; Lewis, 2002). There are
definitive differences though in the presentation of these disorders and tic disorders, which
are, discerned from by taking a careful case history.
The movements in TS are un-rhythmic; therefore, they differ from the movements
in Chorea, Dystonia, and Myoclonus. Other differences between the previously mentioned
disorders and TS are displayed by the changing pattern of tics in TS, the fact that tics do
not always stop during sleep in TS and the fact that individuals with TS usually over time
begin to experience premonitory senses before experiencing tics (Leckman & Cohen,
1999).
The most distinguishing difference between TS and Dyskinesias is that the
movements in Dyskinesias dramatically increase with distraction, whereas the movements
in TS decrease with distraction (Leckman & Cohen, 1999).
Stereotypy and TS are quite similar in their presentation; however, stereotypy does
not have simple clonic, muscular spasms in which contraction and relaxation rapidly
alternate or abrupt movements, both of which are common in TS (Leckman & Cohen,
1999).
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The characteristics of blocking (not being able to move certain muscles)
experienced in TS must be differentiated from what is experienced in akinesia symptoms
from Parkinsons disease. People with akinesia always have trouble initiating movement;
however, movement gets easier with progression (Leckman & Cohen, 1999). Individuals
with TS are unable to move their muscles no matter how hard they try, although, this
difficulty in movement does not last indefinitely.
Factors that Exacerbate Symptoms and the Effects of TS
Exacerbating Factors
According to Lin et al. (2007) the severity of the symptoms experienced by children
who suffer from TS can be predicted by the level of psychosocial stress the child
experiences. With the greater levels of stress being related to increased symptom severity.
Anxiety producing experiences were found to be major contributors to tic severity (Berecz,
1992; Silva, Munoz, Barickman & Friedhoff, 1994). Berecz (1992) says that the moods of
shame and anger lead to increased tic symptoms. He also suggested that overattentive and
anxious parents present an increased risk factor for tic severity.
Comorbidity of disorders is bound to have some effect on TS symptom severity.
Looking at the two most common comorbid disorders ADHD and OCD, we find that the
presence of ADHD does not appear to be associated with tic severity; however, the
presence of OCD is associated with increased tic severity (Burd et al., 2006). Despite Burd
et al. findings that ADHD comorbidity does not have an effect on tic severity, its presence
causes increased family stress as well as increased attention and behavior problems for the
child in school (Blancher, 2002).
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In a study conducted by Silva et al. (1995), that had 14 subjects, seventeen
environmental factors were found to increase tic severity and eleven environmental factors
were found to decrease tic severity. The most frequently experienced factors, which
increased tic severity, in order of ranking were events that made the participant anxious or
upset, emotional trauma and fatigue, watching TV, being alone, and social gatherings (5
out of twelve participants). The most common of the factors, associated with a decrease in
tic severity, in order of ranking were sleeping, doctor visits, social gatherings (3 out of
twelve participants), talking to friends and reading for pleasure. It is interesting how the
environmental factor of social gatherings served to both increase and decrease tic severity
in the participants. Reasons for this could be attributed to differences in the levels of
acceptance, of the childs behaviors, from the people attending the social gatherings.
Effects on Emotions and Relations
The literature is contradictory on the issue of children with TS and the quality of
their peer relationships. In a survey, parents of children with TS were asked about their
childs peer relationships. Some parents reported significant problems, whereas others did
not report problems (Leckman & Cohen, 1999). Bawden, Stokes, Camfield, Camfield &
Salisbury (1998) reported that children with TS had poorer peer relationships than controls.
Most children with TS report having some friends, with a big percent also saying that they
have trouble getting along with their classmates (Leckman & Cohen, 1999). Children with
TS are reported by their teachers to be more withdrawn and less popular compared to their
peers (Blacher, 2002). Leckman & Cohen (1999) found similar results with teachers
reporting that students with TS were significantly more withdrawn and aggressive than
peers (Leckman & Cohen, 1999).
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Are most children who suffer from a physical or mental illness less popular and
more withdrawn than their peers are? Blacher (2002) seems to think that this is the case;
however, Bawden et al. (1998) did not find this to be the case. Bawden et al. conducted a
study to see if peer relationship problems were the same for children with TS and Diabetes
Mellitus (a chronic physical condition). To test the severity of TS symptoms the Yale
Global Tic Severity Scale (YGTSS) was used. The results of their study showed that
children with TS and comorbid ADHD were rated as more aggressive, withdrawn and less
likable than the controls. Children with TS without ADHD were still rated as more
withdrawn than the children that had Diabetes Mellitus.
Children with tic disorders suffer in their affective states, thoughts, fantasies and
relationship patterns. Because of their frequent tics, they often experience embarrassment.
Leckman & Cohen (1999) reported that both children and their parents report that teasing
leads to discomfort and hurt feelings. These children sometimes get anxiety from the
teasing and from trying to stop their TS symptoms voluntarily. When teasing occurs often
and these children do not find a way to cope with it, their anxiety and hurt feelings can lead
to depression. These feelings can end up dominating the childs social relationships.
Children with TS are confused about why they have their problem. This confusion
can lead them to personalize the negative comments and experiences they have had, so that
they end up blaming themselves for the disorder. In addition, children with TS may
become so self-conscious of their behaviors that they withdraw from their peers and
become isolated (PDM Task Force, 2006).
Effects on Family
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Motor and vocal tics in TS are directly associated with difficulties in self-esteem,
family life, social acceptance and school or job performance (Lewis, 2002; see also
Faraone, 2003). The strain on the family of a person with a psychological or medical
disorder can be extreme. The parents are called upon to put forth more effort, patience,
time and usually money in the care for their children with this disorder. The strains of this
condition can lead to unsatisfactory marriages, neglect of other children, stress, anger and
frustration for all participants.
The siblings of a TS child must deal with many issues. Some of these issues are the
fear of contracting the disorder themselves, having to mature prematurely, less attention
from their parents, having to choose between sticking up for their sibling or pretending not
to know them when they are ridiculed and survivor guilt (Safer, 2002).
Effects on School performance
Students with TS have trouble in both their education and socialization with peers.
Because of experiencing failure in school, social relationships and being ridiculed, TS
students may learn to hate school and try their hardest to come up with excuses of why they
should not have to attend school (Dornbush & Pruitt, 1995). Parents need to recognize the
hardships their children are experiencing and sympathize with them while at the same time
encouraging their children not to give up.
Besides the social issues children face in the academic realm, children with TS can
also experience perceptual and cognitive difficulties/deficits. The most common of these
deficits are in tasks requiring visual-motor or visual graphic integration (Lewis, 2002).
Thus, Children with TS have many obstacles to overcome to have a successful and
fulfilling educational experience.
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Treatments
The goals of treatment for individuals with TS are to eliminate or reduce tic
symptoms. Help the individual and family learn how to accept the handicap of the disorder
and learn how to deal with the disorder in a healthy manner. Help the individual with TS
to diminish their embarrassment, frustration, depression and anger over their disorder and
teach them how to advocate for themselves (PDM Task Force, 2006).
The majority of the information concerning the effectiveness of TS treatments has
been gathered from sample sizes of 60 or fewer participants and usually targets single
symptoms, thus not allowing for comorbid treatment (Gilbert & Buncher, 2005). Thus
more research needs to be conducted on the disorder with larger sample sizes and aimed at
the treatment of comorbid disorders along with TS. This is particularly needed considering
that, treating TS can exacerbate the comorbid disorders and treating the comorbid disorders
can exacerbate the symptoms of TS.
In some cases, it is more important to treat the comorbid disorders before the
treatment of TS. However, when the TS symptoms are severe and interfere with activities
and are embarrassing, these symptoms should be treated immediately (Preston, ONeal &
Talaga, 2006). As with most disorders, a multimodal form of treatment has shown to be
very effective. Nonetheless, at present the most common form of treatment for TS is
medication. However, multiple side effects can arise due to some medications. Besides
medication, treatments there are other treatments some of which have proven effective and
others that are in the investigation stages. These include behavioral interventions,
neurological and cognitive feedback, electroconvulsive therapy, dietary measures,
psychotherapy, and counseling.
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Medications
Multiple classes (categories) of medications are effective for treating TS. Some of
these classes of medication have more side effects than other classes while at the same time
other classes yield superior results in the treatment of comorbid disorders. Thus, it is
helpful to be familiar with the different classes of medication used in the treatment of TS.
In many cases, medication does not eliminate the symptoms. Rather it makes the
symptoms more bearable. As a result, the person with TS should receive a dosage with the
intention and expectation of decreasing the symptoms, not eliminating them. The primary
classes of medications used are typical and atypical neuroleptics, nonneurolptics, alpha 2
agonists, and other tic suppressing agents (Martin, Scahill, Charney & Leckman, 2003).
Of these groups of medications, the typical neurolptics have received the most
research and are used the most widely used. The most commonly used medications in this
class are pimozide and haloperidol. In trial studies, patients have needed smaller doses of
pimozide than haloperidol, which resulted in fewer negative side effects. In a 1 and 15
year follow up study patients were significantly more likely to continue on pimozide than
they were on haloperidol (Martin, et al., 2003). With the use of pimozide there is concern
of QT elongation resulting from the calcium blocking properties in pimozide. Thus, the
recommendation is that patients on pimozide receive electro cardio gram (EKG) scanning
to monitor QT elongation.
The preference for atypical neuroleptics stems from there reduced risk of tardive
dyskinesia compared to that in typical neurolpetics. This group of medications combines
both serotonergic and dopaminergic antagonists. In this group of medications, risperidone
has received the most attention in research and has shown promising results in tic
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reduction. Olanzapine and Ziprasidone have also shown promising results in tic reduction;
however, clozapine has not proven effective in tic reduction (Martin, et al., 2003).
Antidopaminergic agents researched include, Tetrabenazine, Tiapride, Sulpiride
and Pergolide. Tetrabenazine is only available for research investigation purposes in the
United States. In a clinical trial of Tetrabenazine it was found to have a mild effect on the
reduction of tics in 11 (65%) of the 17 subjects (Martin, et al., 2003). Tiapride and
sulpiride are not available for use in the United States; however, Europe uses these
medications. In a clinical trial tiapride showed a 30-44% decrease in tics, according to
videotaped accounts (Martin, et al., 2003). Sulpiride showed positive results in 60% of the
63 subjects used in a retrospective study (Martin, et al., 2003). Pergolide is the routine
medication for Parkinsonss disease. In a clinical placebo study with 32 participants aged
7-19 years of age, 75% of the subjects reported a minimum of 50% reduction in tic
severity. A second study with children also found a significant reduction in tics, with no
significant difference in side effects between the treatment and placebo group (Martin, et
al.).
Alpha 2 Agonists, clonidine and guanfacine, were originally developed as
antihypertensive agents. Guanfacine causes less sedation, has a longer half-life, and does
not have increases in blood pressure following abrupt withdrawal compared with clonidine.
A benefit of clonidine is that it comes in two forms a patch and a pill (Martin, et al., 2003).
Other agents that have shown some beneficial effects in tic reduction include
nicotine, mecamylamine, androgen modification, botulinum, and baclofen. In a study,
children given nicotine chewing gum alone, received a modest effect, compared to children
given haloperidol in conjunction with nicotine chewing gum. The bitter taste of the gum
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combined with gastrointestinal symptoms was unpleasant (Martin, et al, 2003.).
Mecamylanine, a nicotine antagonist, significantly reduced tic severity in 22 out of 24
subjects, and decreased negative mood and irritability. The length and concomitant
medications and treatment were unclear for the study (Martin, et al.). Because of the
higher prevalence of TS in males than females, steroid manipulation is recommended as a
possible treatment; however, significant effects have not been found to date (Martin, et al.).
Botulinum showed some success. In a study with 45 subjects, 8 to 69 years of age, 39
showed moderate improvement. Common side effects were neck weakness, ptosis and
mild transient dysphagia. Direct injection at the place affected with tics showed the best
results. Baclofen, in an open-label trial with 264 children, found significant improvement
in 95% of the children in both motor and vocal tics. The most common side effect reported
was sedation (Martin, et al.).
Medication treatment for comorbid ADHD.
The symptoms of the comorbid ADHD are usually more impairing than the
symptoms from TS. This fact raises a challenge considering that commonly stimulant
medications have been believed to increase tics, although, one study investigating this issue
did not find this result (Martin, et al., 2003). As a result, in general, it is better to try and
treat the comorbid ADHD with nonstimulant medications, such as the alpha adrenergic
agents, clonidine and guanfacine. Tricyclic antidepressants such as desipramine and
nortriptyline, newer antidepressants such as bupropion, venlafaxine, and atomoxetine, the
beta-blocker pindolol; and the selective monoamines oxidase inhibitor and deprenyl have
been suggested for use in individuals with both TS and ADHD. Of these medications, only
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deprenyl and desipramine have research backing the efficacy of their use with children
having both TS and ADHD (Martin, et al.).
Medication treatment for comorbid OCD.
Individuals with TS and OCD respond differently to treatment than individuals with
only TS or OCD. Studies using specific serotonin-uptake inhibitors (SSRIs) for the
treatment of TS with OCD have found less responsiveness than in the treatment of OCD
alone. For the most part SSRIs are well tolerated in children with TS, with the most
common side effect being behavioral activation. Some studies have shown SSRIs to
exacerbate the symptoms of TS (Robertson, 2000). However, according to Martin, et al.
(2003) SSRIs usually does not have an effect on tic severity (Martin, et al., 2003).
Side effects for Antipsychotic medications
When prescribing medication for children and adults one must be careful of the
possibility of negative drug interactions. Thus making sure that the prescribed medication
will not have a negative interaction with a medication the person is currently taking is
necessary.
The following are common side effects for medications used in the treatment of TS
corresponding to the organ systems they affect in the body. In the cardiovascular system,
dizziness, hypotension, QT interval prolongation and tachycardia are possible side effects.
In the dermatological system, allergic reactions, alopecia, photosensitivity and skin
discoloration are reported side effects. In the endocrine system, prolactin elevation,
gastrointestinal problems, constipation, jaundice, steatohepatitis and weight gain are
possible side effects. In the neurological system, extrapyramidal effects, sedation and
seizures are possible side effects. In the ocular (vision) system, acute angle closure,
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blurred vision, cataracts, keratopathy and pigment deposits are possible side effects. In the
oral system, dry mouth and cavities are side effects. In the reproductive system,
anorgasmia, ejaculatory dysfunction, impotence, priapism and reduced libido are possible
side effects. In the urinary system, urinary retention and thermoregulatory dysfunctions are
possible side effects. Also in the immune system, agranulocytosis, leucopenia and
neutropenia are possible side effects. (Martin, et al., 2003).
These side effects are possible for all of the antipsychotic medications. No one
medication will have all of these possible side effects. You should carefully research the
medication you are considering to find out which of these side effects, are common for
your particular medication and the risk factor percentages for these side effects. Even if
these are the side effects for your prescribed medication you, there are recommendations
for diminishing or even eliminating these negative effects. By keeping, a careful watch and
following directions carefully concerning what you should and should not do while on
medication can help reduce side effects.
Non-pharmacologic Approaches to Treatment
Besides treating TS with medication, there are multiple alternative approaches,
which are effective when used separately or when combined with medication. Some of the
approaches that have been used and researched include, education, counseling, behavioral
techniques (Shavvitt, et al., 2006), neurofeedback (Daly, 2004), assertiveness training, self-
monitoring, cognitive therapy, laser therapy, acupuncture, surgery (Robertson, 2000)
aerobic exercise (Simms, 2006) and electro convulsive therapy (Karadenizli, Dilbaz &
Bayam (2005).
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According to Faraone (2003), both the child and their parents should receive
education concerning TS. This education should include the nature of the disorder and
treatment options. Shavitt et al. (2006) also recommends education on the disorder for the
individual with TS and their family. Lewis (2002) says that education and reassurance
with the individual, their family, and school is sometimes a sufficient without the addition
of other treatments.
Counseling and psychotherapy helps the individual and their family deal with
adjustment and emotional issues that are comorbid with TS (Shavitt et al., 2006). These
techniques have helped in learning to deal with the stressors and emotions that come with
the disorder; however, they do not affect tic severity. According to Deckerbach, Buhlmann
& Wilhelm (2006) supportive psychotherapy is effective in improving life-satisfaction and
psychosocial functioning in individuals with TS.
Behavioral techniques have proven effective in the treatment of TS symptoms. The
most common of form of these interventions, for use in treating TS, is habit reversal
therapy (HRT) (Deckerbach et al., 2006; Piacentini & Chang 2005; Shavitt et al., 2006).
HRT consists of a variety of techniques that focus on increasing the individuals awareness
to the onset of and preceding feelings of tics, and the development of competing responses
to replace the tics (Shavitt et al.). According to Piacentini & Chang HRT is proving itself
an effective treatment without side effects. Other behavioral interventions, which are used,
include massed negative practice, contingency management and self-monitoring (Leckman
& Cohen, 2002). Massed negative practice involves the person with TS deliberately
performing tics for a specified time-period with brief break periods in-between. This
technique has not proven itself very effective (Leckman & Cohen). Contingency
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management is a common operant conditioning technique based on the theory that
consequences influence the reoccurrence of behaviors. Because of the extreme degree of
structure needed for implementing this technique it may not be useful outside of controlled
settings, such as schools (Leckman & Cohen). Self-monitoring is a form of discrimination
training, in this technique the individual records each of their tics as soon as they occur.
This technique has yielded some positive results in the reduction of tics (Leckman &
Cohen).
Neurofeedback or EEG Biofeedback training (also called neurotherapy and brain
wave therapy) is effective in treating TS symptoms (EEG Biofeedback Training for
Tourette Syndrome, 2007; EEG Info: Neuroscience for Kids, 2007). Neurofeedback
attacks TS symptoms by dealing with one class of symptoms at a time. Attention problems
are one class of symptoms. The class of symptoms treated first depends on the severity of
the symptoms in each of the symptom classes involved. Usually the effect of
neurofeedback training is permanent; however, sometimes an individual may need booster
sessions or maintenance training (EEG Biofeedback Training for Neuroscience for Kids,
2007).
In a research study conducted in Russia, laser therapy was effective in treating TS.
In a study, using laser therapy the researchers found that individuals treated with laser
therapy needed lower doses of medications than the controls who did not receive the
treatment. Laser therapy is only experimental in nature (Robertson, 2000).
Acupuncture proved effective for a researcher in China. In his research, there were
156 participants with TS aged 6 to 15 years of age. The researcher reported that this
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intervention helped in 92% of the cases. This approach is only experimental in nature
(Robertson, 2000).
Strategies for Educators of students with TS
Children with TS can have a difficult time in the classroom; this is because of
multiple issues, which include stigma of the disorder and difficulty concentrating.
Children, however do not obtain low IQs because of the disorder (Lewis, 2002). Students
with TS work best in an emotionally safe environment. Educators can take steps so to
make the school environment an emotionally safe place for the student. A number of
strategies are useful in helping students with TS obtain academic success. (Strategies for
educators working with students who have TS are in appendix C).
The Future for Children with TS
Many people suffering from TS have lived productive lives. Tourette Syndrome
(2007) gives a few examples of famous people who have had TS. Some of these examples
include, Jim Eisenreich a professional baseball player, Mahmoud Abdul-Rauf a
professional basketball player, Samuel Johnson a famous British writer in the1700
hundreds, and speculations that Mozart also suffered from TS.
In about two-thirds of the cases, tic disorders stop during or after adolescence with
it relatively rare for a person to have the disorder for their entire life (Faraone, 2003).
During childhood treatment, usually helps reduce the tic disturbances to a bearable level.
Considering the success that many people with TS have experienced, and the treatments
available do not become discouraged when one of your relatives or acquaintances gets the
disorder.
Summary
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This paper has briefly covered many aspects of TS, including its history,
characteristics, effects on person and family and treatments. TS is a neurological
developmental disorder that affects approximately 1% of school aged children with males
being 3 or more times likely to have the disorder than females. Children who have TS can
suffer from being embarrassed about their tics, which can lead to withdrawal from social
interaction with others. The disorder also affects the family and creates additional
stressors. Learning to deal with the complications the disorder brings can create
considerable challenges for the childs parents, siblings, peers, and teachers. Learning
about the factors that exacerbate the childs symptoms is a helpful for treatment. There
multiple treatment approaches that can be utilized for treating TS. Medication is the most
common treatment used and is often used alone. However, other treatments are helpful
used with medication or alone, with the majority of them not having side effects. The best
form of treatment consists of a multimodal form of treatment that includes teaching the
family and child with TS and how to deal with its symptoms and effects.
For current up to date information concerning TS, you can contact the national
tourette syndrome association at http://www.tsa-usa.org/; the national institute of
neurological disorders and strokes at
http://www.ninds.nih.gov/disordres/tourette/tourette.htm and the society for neuroscience
athttp://www.sfn.org/index.cfm?pagename=home.
25
http://www.tsa-usa.org/http://www.ninds.nih.gov/disordres/tourette/tourette.htmhttp://www.sfn.org/index.cfm?pagename=homehttp://www.sfn.org/index.cfm?pagename=homehttp://www.sfn.org/index.cfm?pagename=homehttp://www.tsa-usa.org/http://www.ninds.nih.gov/disordres/tourette/tourette.htmhttp://www.sfn.org/index.cfm?pagename=home -
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Appendix A
Motor Movements (Dornbush & Pruit, 1995)
Ankle flexing/moving; Arm
flailing, flexing
Blowing on hands/fingers
Body jerking/tensing/posturing
Chewing clothes/paper/hair;
Spitting
Eyeblinking, rolling & squinting
Facial contortions & grimacing
Finger/foot tapping, moving;
clapping
Foot dragging, shaking, tapping
Hair patting/tossing/twisting
Hand clenching/unclenching
Head jerking/rolling
Hitting others/self
Inhaling/exhaling
Jaw/mouth moving
Joint/knuckle cracking
Jumping; kicking; hopping
kissing hand/others
Nose twitching
Picking at things; Pinching
Pulling clothes scratching
shoulder shrugging/rolling;
shivering
Skipping smelling fingers/objects
Squatting; stooping
Stepping backwards; Stomping
Table banging; Tapping objects
tearing books/paper teeth
clenching/unclenching
Muscle tensing/untensing
Throwing things
Toe walking
Tongue thrusting,
Twirling in circles; Twirling
objects
Leg bouncing/jerking
Lip licking/smacking/ pouting
Knee, deep bending/ knocking
Trichotillomania (pulling out hair)
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Copraxia (making obscene
gestures)
Appendix B
Vocalizations (Dornbush & Pruitt, 1995)
Barking or other animal noises,
blowing sounds
Belching, coughing, hiccupping
Calling out
Clicking/clacking
Grasping
Grunting, Gurgling, throat clearing
Hissing, Honking, Humming
Laughing
Making tsk, pft, and guttural
sounds
Making motor/jet noises
Screaming, shouting, screeching,
shrieking, squealing
Sniffing, snorting
Syllable: hmm, oh, wow,
uh, yeah
Talking in character voices
Whistling, Yelping
Unintelligible noises
Moaning
Noisy breathing
Saying hey hey, ha ha
Unusual speech patterns
(Accenting words
peculiarly/stammering or
stuttering/using unusual vocal
rhythms)
Coprolalia (obscenities)
Echolalia (repeating others words)
Palilalia (repeating own words)
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Appendix C
Strategies for Educators of Students With TS (Dornbush and Pruitt, 1995)
Model respectful behaviors
Educate the students and workers in the class concerning TS
Be flexible, respect the students limitations while at the same time encouraging
the students to strive for their goals
Try to discern what is causing the behavior, ask who, what, when where, how and
why questions
Recognize that some behaviors may be a result of neurological impulses or
medication side effects, rather than a direct result of the TS.
Enhance a sense of mastery and control over events (students with TS who
constantly deal with failure have a tendency towards learned helplessness)
Make a Strategy Book with the student (this is a collaborative effort from the
student and teacher concerning what works, and what does not work. Cues for such
things as leaving the room and asking for help should be included in the book).
Do not ask the students with TS to suppress their tics. This will divert their
attention and concentration from learning, and cause stress, which exacerbates tics.
Provide a safe place for students to go when they need to release their tics
Collaboration between teachers, counselors, psychologists and parents
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