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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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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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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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Tourette Syndrome

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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Tourette Syndrome

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.

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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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