Tourette Syndrome Running head: TOURETTE SYNDROME

Literature Review of Tourette Syndrome with Particular Emphasis Towards Children and Treatments Jessica R. Dettmann Andrews University

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.

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

Tourette Syndrome 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

Tourette Syndrome 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

Tourette Syndrome 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

Tourette Syndrome 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).

Tourette Syndrome 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.

Tourette Syndrome 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).

Tourette Syndrome The characteristics of blocking (not being able to move certain muscles) experienced in TS must be differentiated from what is experienced in akinesia symptoms

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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).

Tourette Syndrome In a study conducted by Silva et al. (1995), that had 14 subjects, seventeen

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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).

Tourette Syndrome 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;

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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

Tourette Syndrome 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,

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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.

Tourette Syndrome Treatments The goals of treatment for individuals with TS are to eliminate or reduce tic

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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.

Tourette Syndrome Medications

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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

Tourette Syndrome 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

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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

Tourette Syndrome 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.

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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, selfmonitoring, cognitive therapy, laser therapy, acupuncture, surgery (Robertson, 2000) aerobic exercise (Simms, 2006) and electro convulsive therapy (Karadenizli, Dilbaz & Bayam (2005).

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

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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

Tourette Syndrome management is a common operant conditioning technique based on the theory that consequences influence the reoccurrence of behaviors. Because of the extreme degree of

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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

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.

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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

Tourette Syndrome This paper has briefly covered many aspects of TS, including its history, characteristics, effects on person and family and treatments. TS is a neurological

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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 at http://www.sfn.org/index.cfm?pagename=home.

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References American Psychiatric Association (2004). Diagnostic and statistical manual of mental disorders (4th ed. Text Revision). Washington, DC. Bawden, H. N., Stokes, A., Camfield, S. c., Camfield, P. R., & Salisbury, S. (1998). Peer relationship problems in children with Tourette disorder or Diabetes Mellitus. Journal Child psychology and Psychiatry, 39 (5), 663-668. Berecz, J. M. (1992). Understanding Tourette syndrome: Obsessive compulsive disorder & related disorders. New York: Springer Publishing Company. Blacher, J. (2002). The double whammy: Tourette syndrome and associated disorders as they impact families. Exceptional Parent Magazine, 72-77. Burd, L., Freeman, R. D., Klug, M. G. & Kerbeshian, J. (2006). Variables associated with increased tic severity in 5,500 participants with Tourette syndrome. Journal of Developmental and Physical Disabilities, 18 (1), 13-24. Canitano, R., & Vivanti, G. (2007). Tics and Tourette syndrome in autism spectrum disorders. Autism, 11 (1), 19-28. Abstract obtained from PsycINFO. Deckersbach, T., Rauch, S., Buhlmann, U., Wilhelm, S. (2006). Habit reversal versus supportive psychotherapy in Tourettes disorder: a randomized controlled trial and predictors of treatment response. Behavioral Research Therapy, 44 (8), 1079-1090.

Tourette Syndrome Dally, R., & Lev, B. (2004). Tourettes syndrome: Three year follow-up of a successful treatment outcome. Journal of Neurotherapy, 8 (2), 143-144.

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Martin, A., Scahill, L., Charney, D., Leckman, J. (2003). Pediatric psychopharmacology: Principles and practice. New York: Oxford University Press. Dornbush, M. P., Pruitt, S. K. (1995). Teaching the Tiger: A handbook fro individuals involved in the education of students with Attention Deficit disorders, Tourette syndrome or Obsessive-Compulsive disorder. Duarte, CA: Hope Press. EEG Info. (n.d.). Tourette Syndrome. Retrieved March 29, 2007 from http://www.eeginfo.com/therapeutic_applications/tourett_syndrome/. EEG Spectrum. (n.d.). EEG Biofeedback Training for Tourette Syndrome. Retrieved March 29, from http://www.eegspectrum.com/Applications/Tourette/TouretteIntro/. Faraone, S. V. (2003). Straight talk about your childs mental health: What to do when something seems wrong. New York: The Guilford Press. Gadoth, N., Mass, E. (2004). Hereditary neuropathies with self-mutilation. Journal of Pediatric Neurology, 2 (4), 205-211. Gilbert, D. L., & Buncher, R. (2005). Assessment of scientific and ethical issues in two randomized clinical trial designs for patients with Tourettes syndrome: A model for studies of multiple Neuropsychiatric diagnoses. The Journal of Neuropsychiatry and Clinical Neurosciences, 17 (3), 324-332. Johannes, S., Wieringa BM, Mantey, M., Nager, W., Rada, D., Muller-Vahl, KR., Emrich, HM, Dengler, R., Munte, TF., Dietrich, D. (2001). Altered inhibition of motor responses in Tourette syndrome and Obsessive-Compulsive disorder. ACTA Neurologia Scandinavica, 104, 36-43.

Tourette Syndrome Karadenzli, D., Dilbaz, N., & Bayam, G. (2005). Gilles De La Tourette syndrome: Response to Electroconvulsive therapy. Journal of ECT, 21 (4), 246-248. Leckman J. F., Cohen, D. J. (Eds.). (1999). Tourettes syndrome: Tics, obsessions, compulsions. New York: John Wiley & Sons. Lewis, M. (Ed.). (2002). Child and Adolescent Psychiatry (3rd ed.). Philadelphia, PA: Lippincott Williams & Wilkins. Lin, H., Katsovich, L., Ghebremichael, M., Findley, D. B., Grantz, H., Lombroso, P. J., King, R. A., Zhang, H., & Leckman, J. F. (2007). Psychosocial stress predicts future symptom severities in children and adolescents with Tourette syndrome

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and/or obsessive-compulsive disorder. Journal of Child Psychology and Psychiatry, 48 (2), 157-166. Morrison, J. (2006). DSM-IV made easy: The clinicians guide to diagnosis. New York: The Guilford Press. National Institute of Neurological Disorders and Stroke. (2007). NINDS Tourette syndrome information page. [WWW page]. http://www.ninds.nih.gov/disorders/tourette/tourette.htm. PDM Task Force. (2006). Psychodynamic diagnostic Manual. Silver Spring, MD: Alliance of Psychoanalytic Organizations. Piacentini, J., & Chang, S. (2005). Habit reversal training for tic disorders in children and adolescents. Behavior Modification, 29 (6), 803-822. Abstract obtained from PubMed. Plessen, K. J., Gruner, R., Lundervold, A., Hirsch, J. G., Xu, D., Bansal, R., Hammar, A., Lundervold, A. J., Wentzel-Larsen, T., Lie, S. A., Gass, A., Peterson, B. S., &

Tourette Syndrome Hugdahl, K. (2006). Reduced white matter connectivity in the corpus callosum of children with Tourette syndrome. Journal of Child Psychology and Psychiatry, 1013-1022. Preston, J., ONeal, J. H., Talaga, M. C. (2006). Child and Adolescent Clinical Psychopharmacology Made Simple. Oakland, CA: New Harbinger Publications, Inc. Preston, J. D., ONeal, J. H., Talaga, M. C. (2005). Handbook of Clinical Psychopharmacology for Therapists. 4th Edition. Oakland, CA: New Harbinger Publications, Inc. Robertson, M. M. (2006). Attention deficit hyperactivity disorder, tics and Tourettes syndrome: the relationship and treatment implications a commentary. European Child and Adolescent Psychiatry, 15 (1), 1-11. Robertson, M. M. (2000). Tourette syndrome, associated conditions and the complexities of treatment. Brain, 123, 425-462.

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Safer, J. (2002). The normal one: Life with a difficult or damaged sibling throughout life. New York: Bantam Dell. Shavitt, R. G., Hounie, A. G., Rosario, M. C., & Miguel, E. C. (2006). Psychiatric Clinics Of North America, 29, 471-486. Snijders, A., Robertson, M., Orth, M. (2006). Beck depression inventory is a useful screening tool for major depressive disorder in Gilles de la Tourette syndrome. Journal of Neurology, Neurosurgery & Psychiatry, 77 (6), 787-798. Abstract obtained from PsychINFO.

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Stewart, S., Illmann, C., Geller, D., King, R., Pauls, D. (2006). A controlled family study of attention-deficit/hyperactivity disorder and tourettes disorder. Journal of the American Academy of Child & Adolescent Psychiatry, 45 (11), 1354-1362. Abstract obtained from PsychINFO. Silva, R. R., Munoz, D. M., Barickman, J., & Friedhoff, A. J. (1995). Environmental factors and related fluctuation of symptoms in children and adolescents with Tourettes disorder. Journal of Child Psychology and Psychiatry, 36 (2), 305-312. Simms, P. (2006). Influence of aerobic exercise on the symptom severity of Tourette syndrome [abstract]. Dissertation abstracts International: Section B: The sciences and Engineering, 67 (3-B), 1743. Tourette syndrome association, inc. (n.d.). Official website of the national Tourette syndrome association (TSA). Retrieved March 16, 2007 from http://www.tsausa.org/. Neuroscience for Kids: Tourette syndrome. (n.d.). Retrieved March 16, 2007 from http://faculty.washington.edu/chudler/ts.html. Society for Neuroscience. (2006). Brain Facts: A primer on the brain and nervous system. Retrieved February 20, from http://www.sfn.org/index.cfm?pagename=brainfacts. Yoo, H. K., Kim, J. Y., & Kim C. Y. (2006). A pilot study of Aripiprazole in children and adolescents with Tourettes disorder. Journal of Child and Adolescent Psychopharmacology, 16 (4), 505-506.

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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)

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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