Primer on Tourette s Syndrome - - Prepared by Leslie E. Packer, PhD

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1 Primer on Tourette s Syndrome - - Prepared by Leslie E. Packer, PhD In 1885, French physician Georges Gilles de la Tourette wrote an article that described patients with a cluster of symptoms including involuntary movements and sounds, and compulsive behaviors. The condition was named in his honor , Leslie E. Packer, PhD. All rights reserved. Published electronically on Tourette Syndrome "Plus" This primer may be reproduced for personal, noncommercial use only. Last updated January 3, 2002.

2 Primer on Tourette s Syndrome - - Prepared by Leslie E. Packer, PhD Notes: This primer attempts to address some of the commonly asked questions about Tourette s Syndrome. It does not provide information on treatment or school-related issues. For those topics, see the author's web site at Use the bookmarks in the left frame of your Adobe reader to jump to particular topics. What is a Tic? A tic is a brief, repetitive, purposeless, nonrhythmic involuntary or sound. Tics that produce movement are called motor tics, while tics that produce sound are called vocal tics or phonic tics. Tics tend to occur in bursts or bouts. Tics are often characterized by whether they are "simple" or "complex." A simple tic involves one muscle group or one simple sound. Many simple motor tics are associated with the face/head/neck region, such as eye blinking, head jerking, shoulder shrugging, mouth grimacing, etc. Simple vocal tics include throatclearing sounds, grunting, sniffing, and coughing. A complex tic involves a coordinated movement produced by a number of muscle groups (complex motor tic) or a linguistically meaningful utterance or phrase (complex vocal tic). As examples, complex motor tics can involve touching objects or other people, jumping up and down, spinning around, or even more complex motor sequences such as imitating someone else's actions (echopraxia) or exhibiting inappropriate or taboo gestures or behaviors (copropraxia). Complex vocal tics may involve having to repeat one phrase over and over, whether it is something one heard (echolalia) or one's own last words (palilalia). The chart on the following page lists some of the common tics of Tourette s Syndrome: , Leslie E. Packer, PhD. All Rights Reserved. 2

3 Common Tics of Tourette Syndrome* Type of Tic: Motor Vocal/Phonic Simple Eye Blinking Eye Rolling Shoulder Shrugs Head Jerks Brushing or Tossing Hair Out of Eyes Mouth Opening Arm Extending Facial Grimaces Nose Twitching Lip-licking Squinting Throat Clearing Grunting Yelling or Screaming Sniffing Barking Snorting Coughing Spitting Squeaking Humming Whistling Honking Laughing Complex Pulling at Clothes Touching People Touching Objects Smelling Fingers Smelling Objects Jumping or Skipping Poking or Jabbing Punching Kicking Hopping Kissing Self or Others Flapping Arms Twirling Around Tensing Muscle Groups Thrusting Movements of Groin or Torso Twirling Hair Walking on Toes Copropraxia: Sexually Touching Self Sexually Touching Others Obscene Gestures Echo Phenomena: Others' Actions One's Own Actions Self-Injurious Behaviors (e.g., Biting, Hitting, Picking Skin Or Scabs) Making Animal-like Sounds Unusual Changes in Pitch or Volume of Voice Stuttering Echo Phenomena: Own Words or Sounds Others' Words or Sounds Coprolalia: Obscenities Socially Taboo Phrases *Note: This list is not all-inclusive. Furthermore, any behavior or symptom may have alternative explanations in a child who has TS and other diagnoses such as Obsessive-Compulsive Disorder and/or Attention Deficit Hyperactivity Disorder. Table adapted from Educating children with Tourette Syndrome: Understanding and educating children with a neurobiological disorder. I: Psychoeducational implications of Tourette Syndrome and its associated disorders, by L. E. Packer, published by the NYS Education Department, Albany, NY. Copyright 1995 by L. E. Packer , Leslie E. Packer, PhD. All Rights Reserved. 3

4 What is Tourette s Syndrome? A significant percentage of all children will experience one, or even a few, tics at some point in their development. For most children, the tic will emerge without any warning or explanation, remain a few weeks, and then disappear slowly. If tic(s) are present for less than a year and do not recur, we say that it is a "transient" tic condition. The transient tic condition observed in children is generally benign and usually does not require treatment. If there is a history of a number of tics that have been present -- even if not continuously -- for more than a year, we say that there is a "chronic" tic condition. A chronic motor tic condition is one in which the individual has one or several motor tics on and off for more than a year. A chronic vocal tic condition is one in which the individual has one or a number of vocal tics on and off for more than a year. If the individual has a history of a number of motor tics and at least one vocal tic, and tics have been present on and off for more than a year and there has been a pattern where the tics emerge, get worse over weeks, then abate, then the individual may have Tourette s Syndrome (TS). The word "may" is important, because there are other conditions that could produce multiple tics without the individual having Tourette Syndrome. How is Tourette s Syndrome Diagnosed? Currently, there is no brain test or laboratory test to determine if someone has Tourette s. The diagnosis is a clinical one, which means that it's based on the professional taking a careful history, observing the patient, and arranging for any tests that might be necessary to rule out other conditions that could look like Tourette s or that could cause tics. Although many people who have Tourette s Syndrome or who are in the professional field feel that there is more to Tourette s Syndrome than just the tics or movement disorder aspect, for now, we will use the term as it is defined in the Diagnostic and Statistical Manual DSM-IV or DSM-IV-TR, the revised version) since that is the primary reference that physicians use in making diagnoses. To simplify the diagnostic criteria for Tourette s, let us focus on the criteria that most physicians really do look at: A. Both multiple motor and one or more vocal tics have been present at some time, although not necessarily concurrently or continuously; B. The tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than 1 year; C. Other causes (such as medication-induced tics, substance-induced tics such as what you might see with Ritalin, and other neurological conditions that can produce tics) have been ruled out. What other conditions can produce tics? There are a number of medical conditions that can produce tics or mimic Tourette: Sydenham's Chorea, Wilson's Disease, tardive dyskinesia, Lesch-Neyhan Syndrome, cerebral palsy, Huntington's disease, postviral encephalitis and neuroacanthocytosis, to name but some. Stimulant medications such as Ritalin may produce tics, and tics may also be among the sequelae of head injury or encephalitis. Depending on the patient's history, the clinician's observations, and the physical examination, the physician may order some tests to rule out some of these other conditions. Years ago, it was common for physicians to put patients through a lot of neurological testing such as EEGs before making the diagnosis of TS. Today, many physicians do not order any tests unless there is some indication that other conditions can only be ruled out that way. Do not be surprised, therefore, if the diagnosis is made after the physician takes a detailed history and performs a neurological examination in , Leslie E. Packer, PhD. All Rights Reserved. 4

5 the office. A neurological examination involves checking the cranial nerves and asking the patient to perform certain tasks or activities -- no sophisticated tests or instruments are required for that. Over the last eight years, some intriguing but controversial studies have suggested that in some cases, streptococcal ("strep") infections may trigger an acute-onset TS or Obsessive-Compulsive Disorder or a dramatic worsening of symptoms in individuals who already have TS and/or OCD or in those who are constitutionally vulnerable to it. For those who want to read more about this topic, studies on the potential relationship between these antineuronal antibodies and TS are referred to the "PANDAS" section of this site. If the physician suspects that PANDAS is responsible, specialized blood tests will be ordered. Is cursing part of Tourette s or required for diagnosis? Although Tourette has been stigmatized by the media as the "Cursing Disease," only a minority of individuals who have Tourette experience unvoluntary outbursts of socially inappropriate or taboo expressions. This type of complex vocal tic (coprolalia) is not required for diagnosis. Some coprolalia is immediately obvious as a tic: the individual may have one word or phrase that they blurt out repetitively and inappropriately in the middle of speech. Other coprolalia may be more difficult to recognize as a symptom since it is often triggered by cues in the immediate environment. As an example, an individual who has coprolalia may just blurt out whatever is inappropriate or unacceptable in a particular situation. Walking down the street, the sight of a buxom woman may lead to "Big boobs!" As another example, the sight of someone of a different race may trigger a racial epithet -- although the person may have no racial prejudice and feels mortified by what just popped out of his or her mouth. A number of studies suggest that less than 30% of individuals seen in clinics that specialize in Tourette s may have coprolalia. Since specialty clinics tend to see the more severe cases, this suggests that an even smaller percentage of individuals with Tourette s in the general population will have coprolalia. How Does the Individual Experience Tics? Even though tics are considered to be "involuntary" or "unvoluntary," many individuals report a sensory basis for their tics -- they feel the need to tic building up as a kind of tension in a particular anatomical location, and they feel that they consciously choose to release it. For many individuals with Tourette s, there is also a sense that the tic needs to be performed or released until they achieve a "just so" kind of sensation or experience. The sensory phenomena and the "just so" or "just right" kind of experience seem to be more prevalent in patients who have Tourette s or Tourette s plus Obsessive-Compulsive Disorder than in patients who have just Obsessive-Compulsive Disorder (see Miguel et al., 2000). Not all individuals are aware of their tics. As but one example, a student may be sitting at her desk humming tunelessly and be quite surprised (and frustrated) when her peers repeatedly tell her to stop the humming. "What humming?" she asks. When a child or adult denies ticcing it may not mean that they are in "denial." They may really be unaware of their movements or sounds. Can Tics Be Suppressed? Is the individual really choosing to release the tic? If so, does that mean that they could stop ticcing or suppress ticcing if they just wanted to badly enough? For example, a man may report that his shirt collar is irritating the back of his neck and that his head/neck movements are a conscious attempt to get rid of the uncomfortable feeling. His experience is that he is releasing it or "doing it on purpose," but in actuality, if he tried to not do it, he'd find himself eventually doing it anyway. Tics can sometimes be suppressed, but they will eventually be released. As noted earlier, tics tend to come in bursts or "bouts." The child or adult may have a bout of eye blinking, followed by a period of calm, and then another bout of eye blinking. Dr. James Leckman talks about this pattern as being one of "bouts within bouts within bouts," whereby bursts of tics may be experienced throughout the day, over days, over weeks and months. When the number of bouts within the day is , Leslie E. Packer, PhD. All Rights Reserved. 5

6 increasing, we say that the individual is in a "waxing" cycle. As the number of bouts within the day or over days decreases, we say that they are in a "waning" cycle. How does a bout end, however? As suggested earlier, many individuals report that their tics continue until some "just right" sensation is achieved. An analogy might be to think of the last time that you had a mosquito bite. You became aware of the itch and need to scratch. You may have been able to delay scratching or to try to substitute scratching near the bite instead of directly on it, but you continued doing something until the itch was relieved or you got some "just right" sensation -- either a feeling in the skin or the sight of blood let you know that you could stop. What happens if the individual tries to suppress or interrupt the bout? Some individuals have no control at all over their tics, while others have varying degrees of control. Most adults report that their ability to modify or suppress their tics improved as they matured. With young children, it is important to remember that the child may not be aware of their tics, and even if they are aware, they may have no ability to suppress them. More importantly: Asking an individual who has tics to suppress them is generally not a good idea because: the effort involved in suppressing the tics will distract the individual from whatever else is going on that they should be paying attention to, the effort spent in suppressing tics is stressful and can produce fatigue and/or irritability, and when the tics are eventually released, they will be more explosive. It is a common phenomenon that children or adolescents who try to suppress their tics in school all day (with varying degrees of success) will come home from school, walk in the door, and explode in tics -- often accompanied by a lot of emotional behaviors. But he stopped ticcing when he was playing Nintendo! One phenomenon that has often been reported is that under some conditions, people may not tic at all. As just one example, children who might be ticcing quite a bit at may not tic at all in the doctor's office. Similarly, they may exhibit very few tics in school, due to either involuntary suppression or voluntary attempts to suppress their tics. When someone who has Tourette s is totally and constructively engrossed in something, the tics may stop altogether. This is not generally experienced as a stressful form of suppression, and suggests an important strategy for working with students in the classroom: If they are ticcing a lot, and you wait until they stop ticcing to try to teach, they will probably continue ticcing. If you temporarily ignore their tics and present something that is novel and fascinating to them, their tics will probably just stop as their neurochemistry shifts in response to the novel situation or activity. Because children who tic actually tend to tic less when they are fascinated with classroom tasks or activities, the author has often encouraged schools to put the student in enrichment or gifted programs that are more likely to enable them to focus constructively. Should I point out the tics to the child? Talking with children about their tics is likely to lead to increased ticcing, particularly in the case of vocal tics (Woods et al., 2001). Parent reports also suggest that students may find it aversive (unpleasant) when their teachers signal them (even discreetly) that they are ticcing (Packer, 2002, in press) , Leslie E. Packer, PhD. All Rights Reserved. 6

7 This does not mean that a parent or teacher should never discuss the child s tics with them. It merely means that any conversation about tics is likely to produce increased ticcing during the conversation. How Does TS Usually Start? In the majority of cases, the first tics are usually simple motor tics of the head, face, neck, and shoulder region or simple phonic tics. Eye blinking is the most common 'first tic,' but it is important to remember that having this tic does not necessarily indicate that the child will develop Tourette s, as approximately 1 in every 5 children will have a tic at some point in their development. The first tics of TS are often erroneously thought to be "nervous habits," allergies, or unexplained colds. For example, a child who suddenly starts sniffing may be thought to have a cold or allergies, but the pediatrician may find no evidence of a cold and no clear allergy symptoms such as rhinitis. Similarly, a child who suddenly starts blinking their eyes a lot may be thought to have some vision problem or allergies, but on examination, there will be no evidence to support the notion that the blinking is allergy related. The tic increases in frequency and severity for a few weeks to a month or so, then starts to subside and eventually disappears. Unless the parents or teachers are already aware of a history of tic disorders in the family or are familiar with tics or Tourette s Syndrome from other sources, the first tics are not likely to be recognized as tics. Following the disappearance of the first tic, a few months may go by and then the tic may re-emerge or a new tic may appear. That tic will also increase in frequency and severity and then subside and disappear. The pattern of new tics emerging and worsening ("waxing") followed by a symptom decrease ("waning"), a relative "lull," and then new tics emerging and starting to increase in severity and frequency (repeat cycle) is characteristic of TS. Indeed, this variability is the hallmark of TS, and is frequently a source of confusion for parents and educators. While the average age of onset of TS is 6-7 years old, there are many cases where parents later realized that their child's tics had actually started much younger. In almost all cases, TS emerges before age 18, but there are exceptions (see Chouinard, 2000, for a discussion of adult-onset tic disorders). In some children, TS may emerge more forcefully or explosively. A child with no recognized history of tics may suddenly erupt in a number of tics within a very short period, or the child may present with complex tics instead of simple tics. Another situation in which severe tics or symptoms may emerge is those cases that appear to be related to infections. Because of the changing anatomical location of the tics, the long tic-free periods between bouts of tic worsening, and because tics are often misunderstood as being due to other medical conditions, it used to take years before someone was properly diagnosed. In the last few years, however, we have seen evidence that children are being properly diagnosed sooner. What Causes TS? Can We Predict Severity or Future Outcome? Note: while a full discussion of the genetics and neurology is beyond the scope of a primer, the following is included just to give some perspective on the current state of our understanding and because so many parents are concerned about whether their children will inherit TS , Leslie E. Packer, PhD. All Rights Reserved. 7

8 TS appears to be familial (i.e., it runs in families) in the vast majority of cases, suggesting a genetic component, but our understanding of the mode of transmission is incomplete and controversial. At the international scientific symposium on Tourette in June 1999, researchers announced that they had found evidence suggesting a link between chromosomes 4 and 8 and TS. More recently, other investigators have pointed to a gene located in the breakpoint region at 7q31 that may be involved (Kroisel et al., 2001) or susceptibility loci on chromosomes 2p11, 8q22 and 11q23-24 (Simonic et al., 2001). In contrast to studies identifying or attempting to identify specific loci, Comings (2001) argues that the failure of linkage studies and existing data support a polygenic model of TS. Are there neurological (brain) correlates or predictors? While some scientists search for the gene(s) involved in TS, other scientists use neuroimaging techniques to identify what areas of the brain are involved in TS and what specific neurotransmitters are involved. As you read about Tourette s Syndrome, you will see frequent reference to dopamine, and the symbol "D2," which refers to a particular type of dopamine receptor in the brain. Dopamine has been implicated in other movement disorders, such as Parkinson's Disease, in which there is too little dopamine and movement slows considerably. In one of the most significant research reports, scientists discovered that in pairs of monozygotic twins, the twin who had the more severe tics showed greater sensitivity to dopamine in the caudate nucleus in the brain (Wolff et al., 1996). Since the caudate nucleus has been implicated in the cognitive activities of motor planning, the results suggest that the increased sensitivity to dopamine releases the "brake" that the caudate nucleus would normally exert on movement, leading to increased movements. More recently, using PET scans, Ernst et al. confirmed the finding of dopaminergic dysfunction in the caudate nucleus of children with Tourette s Syndrome. While some investigators had hypothesized that mutations in another neurotransmitter system (norepinephrine) might be linked to TS, other published research suggests that genetic variants in the norepinephrine system may not be linked to TS (Stober et al., 1999). In terms of anatomical structures and functions, current research implicates structures in the basal ganglia and frontocortical circuits in the brain. Bradley Peterson and his colleagues (2001) used neuroimaging techniques to compare the brains of children and adults with TS to their non-ts peers. They found significant differences in regional ventricular volumes between TS subjects and the controls and reduced sex differences in the parieto-occipital regions of the brains of adults with TS (when compared to non-ts adults). Regional cerebral volumes were significantly associated with the severity of tic symptoms in orbitofrontal, midtemporal, and parieto-occipital regions. Practical Implications for planning parenthood At the present time, all we can say is that there appears to be a genetically transmitted susceptibility to TS, and that boys are more likely to develop TS than girls are. Research by Pauls et al. (1986) suggests that male offspring will have almost a 50% chance of having symptoms of TS, and a.99 (almost certain) probability of having either TS, a chronic motor tic disorder, or Obsessive-Compulsive Disorder. Female offspring have lower probabilities of showing symptoms of these disorders, but there is still a fairly high probability (.70) that a female child will have one of these disorders. One question potential parents often ask is whether there is any way to predict how severe a child's symptoms might be based on what the parent experienced. Based on available reports, there is currently no known relationship between the severity of tics experienced by a parent in their childhood and the severity of tics experienced by their child(ren). A parent who has mild symptoms might have a child who has severe systems (Kurlan et al., 1988) and vice versa. Lichter and his colleagues (1999) also found that the presence of family history does not predict the severity of motor symptoms or Attention Deficit Hyperactivity Disorder in Tourette's Syndrome. While we cannot predict the severity of tics based on family, family history appears to be correlated with severity of obsessive-compulsive behaviors that are often seen in conjunction with Tourette s Syndrome , Leslie E. Packer, PhD. All Rights Reserved. 8

9 Lichter et al. (1999) reported that more severe obsessive-compulsive symptoms were associated with bilineal transmission (i.e., the presence of probable or definite tics or obsessive-compulsive behaviors in both parents or relatives on both sides of the family) than in unilineal transmission (family history on only one side of the family). Children who had a family history of tics or obsessive-compulsive behaviors on both sides of the family were not only more likely to have more severe obsessive-compulsive behaviors, but they were also more likely to exhibit self-injurious behaviors. Lest the preceding sound somewhat alarming, it is encouraging to note that family studies suggest that most cases of TS are "mild, and that prenatal events or factors such as maternal smoking and perinatal events may be associated with severity of TS. While a small percentage of children may need hospitalization for their tics, it is usually not the tics that lead to hospitalization. Coffey et al. (2000) looked at factors predicting whether children and adolescents with Tourette are hospitalized. While tic severity was marginally significant as a predictor, the presence of mood disorders such as depression and Bipolar Disorder were strong ("robust") predictors. Thus, there is a growing body of data to suggest that it is not the tics of TS that are often the most significant problem, but other conditions that may co-occur with TS. Many people report that the waxing periods tend to worsen over the first years after onset of symptoms, but for many (but not all) individuals, the tics will ease up significantly or go into remission in the teen years. A report by Leckman et al. (1998) suggests that tics seem to reach their peak severity between the ages of in the majority of cases, and that by age 18, half of the children are virtually tic-free, with other children showing significant improvement. More recently, Burd, Kerbeshian, et al. (2001) reported on a follow-up of 39 out of 73 patients who had been diagnosed as children in They reported that symptom severity decreased by 59%, global functioning improved by 50%, and the average number of comorbidities decreased by 42%. Similar to findings by Leckman, they reported that 44% of the patients were essentially symptom free at followup; only 22% were on medication as adults. In their sample, improvement in males was more variable than improvement in females, but overall, males demonstrated more improvement than females. Don't panic if your child's tics get worse before they get better: the odds are that your child's tics will eventually disappear or improve in time! While the majority of individuals seen in clinics appear to have a significant remission or improvement in tics, there are people who do not show improvement with age, and there is really no reliable way to predict how severe or frequent someone's tics will be as an adult from how frequent or severe they are as a child. A number of adults have reported that either their TS re-emerged after decades of being tic-free or that they are beginning to suffer adverse physical effects after years of severe ticcing. Unfortunately, while most clinicians seem to be aware of this, no one seems to be doing any long-term research to address these compelling adult needs and only a handful of published articles even refer to the reemergence of TS after decades of quiet. [For a recent literature-based review of the course of Tourette over the life span, see Coffey et al. (2000).] Stress and TS While stress does not cause TS, stressors can make the symptoms worse. Environmental, family, workor school-related stresses, fatigue, and infection may all cause exacerbations (worsening) of symptoms in , Leslie E. Packer, PhD. All Rights Reserved. 9

10 patients who already have these disorders. Preliminary data suggest that a subset of individuals with TS may also experience tic worsening associated with increased heat or room temperature (Scahill et al., 2001). Anything that increases arousal levels or emotional levels may produce an increase in tic symptoms -- including "positive" or exciting events such as upcoming birthdays or vacations, marriage, etc. The author has often observed a predictable worsening of students' symptoms at particular times of the school year: tics worsen right before or as school reopens in September (which is also hay fever season on the east coast), they worsen right before Christmas vacation, and they worsen right around the Easter/Passover/Spring break (which is also when grasses and trees pollinate). School may be a very stressful environment for children with Tourette. There are many resources available on possible modifications that can be used in the classroom to "work around" a child's tics if they're interfering with performance or learning. Some of these materials are available on this site in the Education section; others are available as links or by specific resources you can obtain. Unfortunately, less attention has been paid to the needs of adults who may be experiencing interference in the job setting, although many of the problems and potential solutions appear similar. If stress makes the symptoms worse, does relaxation make them better? While relaxation may lead to less tics, some people tic more as they start to relax and let all the tics out. Similarly, a child or adult may come home from school or work and seemingly "explode" in tics for the first hour or so. Under such circumstances, the most likely explanation is that they are "letting it all out." Tics generally decrease significantly or disappear completely during sleep. As noted previously in this primer, it has been reported anecdotally by many people that when they are nonanxiously or constructively engaged or focused on a task, their tics tend to lessen or stop. The key word there is "nonanxiously." If you demand that the child focus, it will be stressful. If the child is just genuinely interested or has self-selected the activity, they are more likely to be nonanxiously engrossed, and you may see the tics stop. "Do Mild Tics Mean Mild Tourette? I'm So Confused!" While there are clinical scales for assessing the severity of Tourette, there is generally much confusion among the public when someone says "mild" Tourette, because it's not clear whether the individual means "mild and infrequent" tics, "frequent but mild" tics, infrequent tics without any comorbid conditions, or mild and infrequent tics but there are comorbid conditions that aren't mild (comorbid conditions are discussed below). If you're trying to communicate with someone else, try to be precise about the frequency and severity of tics and the presence or absence and severity of any comorbid conditions. Is There a Cure for TS? There is no cure for TS at this time. Nor do all cases require treatment. If symptoms are severe or seriously interfering with functioning, a combination or accommodations and judicious use of medication can offer the individual relief in many cases , Leslie E. Packer, PhD. All Rights Reserved. 10

11 How Common is TS? A recent study by Kurlan, McDermott, et al. (2001) indicates that over 19% of children in regular education classes have tics and over 23% of children in special education classes have tics. Their study also indicated that almost 4% of children in regular education meet diagnostic criteria for Tourette s Syndrome, while over 7% of children in special education have TS. In most cases, tics and/or TS were usually undiagnosed. The incidence and prevalence of tics and TS is controversial. The estimates you may read in the literature depend, in part, on how old the subjects were in the study (studies of adults tend to underestimate rates in children), and whether the subjects were drawn from clinical settings or from the general community, to name but two factors. Comorbidity and TS = TS+ A significant percentage of TS patients who seek medical attention also have symptoms of other disorders. We say something is an "associated disorder" when there appears to be a significant probability that the two conditions will occur together. We say that two conditions are "comorbid" when we want to indicate that two conditions are present in the same individual. When the author first began talking to people about TS, she used the definition of TS provided in the DSM-III-R (the previous edition of the DSM IV). She discovered, however, that when some people would say "That's a symptom of my son's TS," they weren't talking about tics but about features or symptoms of disorders such as hyperactivity or compulsions. So to decrease confusion in our communication, in 1991, she introduced the term "TS+" to refer to patients who have TS plus features of one or more other disorders such as Attention Deficit Hyperactivity Disorder (ADHD), Obsessive-Compulsive Disorder (OCD), anxiety, sleep disorder, anger or rage outbursts, or depression, to name but some. The goal was to help people remember that not everything may be a tic of TS, and that the child may have other problems that are actually impacting their lives more. "TS+" is not a technical or diagnostic term, but rather a convenient way to remind ourselves that there may be more than "just TS" affecting the individual. And for many of us, it saves us an awful lot of time from saying or typing out, 'My child has TS- ADHD-OCD," a common pattern of comorbidity. So, what other disorders tend to be "comorbid" with TS? The answer depends on whom you ask. This is an extremely controversial topic. The majority of clinical settings report that a significant percentage of individuals who have TS also have obsessive-compulsive behaviors and Attention Deficit Hyperactivity Disorder. Mood disorders such as depression and Bipolar Disorder are also commonly reported. Some researchers, including David Comings, MD, have suggested that there are many other disorders that are also linked to TS, including sleep disorders, oppositional defiant or conduct disorder, "rage attacks," anxiety disorders, alcohol and substance abuse problems, and autism, to name but some. Other researchers have reported either some support or total disagreement with Dr. Comings' findings. Out of all the conditions that are discussed as being comorbid with, or associated with TS, the most frequently observed are Obsessive-Compulsive Disorder, Attention Deficit Disorder, sleep disorders, learning disabilities, and mood disorders. In adults who have TS, the primary reason for seeking professional help seems to be anger management problems , Leslie E. Packer, PhD. All Rights Reserved. 11

12 So where does that leave parents of newly diagnosed children? From the author's perspective, parents of newly diagnosed children need to be aware that: (1) these other disorders generally don't emerge at the same time as the TS if they're going to emerge at all, (2) a child can have features of another disorder without meeting all the criteria for diagnosis of another disorder, and (3) when push comes to shove, the research on whether something is or is associated with TS or comorbid with TS is not as important as understanding what is going on with your child and what you can do about it. Knowing more about ADHD, OCD, "rage attacks," depression, anxiety, etc., may help you understand your child. Is there any indication that children or adults with TS are more likely to have personality disorders or other problems? Unfortunately, there has been very little "hard" research on this topic. In one of the few studies that looked at this question, Robertson et al. (1997) compared adult clinic patients with TS to undergraduate students and hospital staff. They reported that the TS patients were significantly more likely to have a personality disorder, and that many of the patients had more than one personality disorder. Their data indicate that about half of the adult TS patients had Borderline Personality Disorder and were also significantly more likely to have other personality disorders: Avoidant, Depressive, Obsessive-Compulsive, Paranoid, and Passive-Aggressive. However, one cannot tell from their study whether the personality disorder was causally linked to the Tourette or rather to a comorbid condition -- or whether it was the result of having been misunderstood during their childhood and adolescence. Perhaps the most conservative thing we can say is that children with Tourette s Syndrome who have other conditions as well may be at increased risk for a whole host of other problems, including rage attacks, but that many of those problems may be avoidable or not due to the TS but to the other conditions. Those interested in the comorbidity issues for scientific reasons or curiosity will be interested in finding out about the ongoing multi-site study known as TIC. As the data come in from sites all over the world, our understanding of what percentage of patients may have different comorbid problems -- or NO comorbid problems -- increases. So far, it seems that those individuals who have TS-only generally do not experience significant functional impact in their daily lives, although they may still need understanding, support, and accommodations. Psychosocial Consequences of TS and Quality of Life Issues TS is not a fatal disorder, but it can be a stigmatizing one for those who live in environments that do not or cannot support or tolerate anyone who might appear a bit different. The media has created a public misperception of what most people with TS can do and what they are like. As a direct consequence, many people are embarrassed to admit that they or their child has TS. This is extremely unfortunate as by attempting to "hide" any TS, we perpetuate the myths. In the author's experience, parents are often most concerned about the psychosocial impact of TS, and worry about "How will other children view my child? Will teachers think he's crazy? Will he be teased because of his tics?" Teenagers who are already struggling with the agonies of adolescence may feel particular concerns as to how their symptoms may affect their attractiveness to others or their ability to form friendships and romantic relationships. Even children or adults with extremely mild tics may experience psychosocial consequences. Two surveys of parents of children with TS indicate that many children are teased because of their tics, and a subset are actually rejected because of their tics (Hagin et al., reported in Silver & Hagin, 1990; Packer, 2002). Even young children may reject peers for their tics. Research by Stokes (1991), Friedrich et al. (1996) and ongoing research by Dr. Doug Woods and his colleagues suggest that parents do have cause to be concerned about the child being viewed as less socially acceptably because of their tics. To date, however, there has been little research that has addressed whether an adequate peer education program could eliminate any negative peer evaluations, although a preliminary study (Woods, 2002, in press) suggests that peer education may be helpful , Leslie E. Packer, PhD. All Rights Reserved. 12

13 Because a child sees him/herself through the parents' eyes, parents need to understand, accept, and support their child. They help their child achieve acceptance in school and among peers not only by educating others, but also by providing the kinds of successful and positive experiences that form the basis for age-appropriate interactions. Perhaps one of the saddest things the author sees is when a parent can't accept their child's symptoms. In some cases, it may be that a child's symptoms violate some religious belief or doctrine. In other cases, it may be that the parent feels embarrassed in front of family or strangers. Whatever the reason, under such conditions the parent tends to put pressure on the child to suppress or stop symptoms that the child often cannot suppress or stop. In addition, in putting that pressure on the child, the parent invalidates the child's experience of being out of control of their body. The child gets the message, "You can stop this if you really want to," when the child can't stop it. Elstner et al. (2001) assessed quality of life (QOL) in 90 adult patients with TS. The QOL scores in TS patients were significantly lower than those of members of the general population, with greatest impact due to mental health, social functioning, and role limitation from emotional and physical problems. The features patients identified as having the greatest impact on quality of life were motor tics (25% of participants rated their tics as having a severe impact), concentration (23%), depression (25%), memory (17%), vocal tics (11%), and anger/aggression (15%). While a third of patients had coprolalia, it had an impact on QOL in only 7% of patients, and only 2% said it had a severe impact. Their data provide partial replication of a Canadian study by Wand et al. (1993). In a recent survey, Packer (2002) asked parents or guardians of children and adolescents with Tourette s to assess the impact of tics on academic functioning, peer relationships, and other activities. While the majority reported impact on academic functioning and peer interactions, the greatest impact of tics appeared to be on the child s self-esteem about school and on family functioning; the child s self-esteem about school and family functioning were significantly correlated. Is There Anything Good About Having TS? In terms of "hard research," there is no known "benefit" of having TS or TS+, other perhaps, than the enhanced fine motor speed that children with "just TS" may have. But if you ask people who live with it or ask parents of young children who have TS+, you may often hear things like: "I think that children with TS+ are more creative," or "I think it's increased my sensitivity to others," or "My racing mind enables me to be 'way ahead' of others," "My child is a voracious reader," or "My child can spend hours and hours with his latest hobby and has become quite an expert on it." Whether these presumed "benefits" are actually linked to TS, ADHD, OCD, Bipolar Disorder, or simply are unique to the individual and not the disorder(s) is uncertain, although after you see something often enough, you begin to suspect that there may well be a connection. That said, and while it is always helpful to find something positive about what might otherwise be just a hindrance, there really is no evidence at this time to support any claims of the superiority of people with TS+. Similarly, despite claims that children with ADHD might be more creative than non-adhd peers, research has not confirmed that claim (see, for example, Funk et al. 1993). So how do we reconcile what so many people experience with the "hard research?" We recognize that group designs that only study individuals in discrete periods of time or on very specific measures often lose the 'flavor' of the whole individual. And in that respect, the author agrees wholeheartedly with clinical neurologist, Oliver Sacks, MD. By getting to know people with Tourette s Syndrome, by spending time with them, you often discover a wonderful exuberance that may be characterized by wild creativity and , Leslie E. Packer, PhD. All Rights Reserved. 13

14 flights of humor, extraordinary sensitivity, and an overall intensity that escapes most people. Always in motion, always reacting to the world in immediate and observable ways, the child or adult with TS or TS+ may make their non-tourette peers seem pale and stuffy. To watch Canadian artist Shane Fistel work or to even walk down the street is to be transported to a whole other dimension of the human experience. The exuberance, energy, and creativity of so many people with TS/TS+ has led a number of people to challenge the notion that TS is a "disorder." Some think that it is a social disorder or that society has the disorder, while the individual with TS has a "condition." To find out about some famous people who have/had Tourette, see the national Tourette Syndrome Association web site. Where Can I Get More Information or Help? The national TSA (TSA) publishes articles and materials for families, educators, and professionals, as well as producing videotapes on TS, and organizing conferences on TS. Tourette Syndrome Association, Inc Bell Blvd. Bayside, NY Telephone: ts@tsa-usa.org Web site at Another TS organization in the U.S. is the: Tourette Spectrum Disorder Association, Inc. Web site at: Other Tourette support organizations and chapters of the U.S. TSA that are online are listed in the "Support" file on the author s web site: , Leslie E. Packer, PhD. All Rights Reserved. 14