Overview of Tourette’s Syndrome
by Leslie E. Packer, PhD, 1998 (last Updated March 2011)
Article Table of Contents:
* What Is a Tic?
* Tic Spectrum and Tourette’s Syndrome
* How is Tourette’s Syndrome Diagnosed?
* What Other Conditions Can Produce Tics?
* Tics and Tourette’s Are Not Rare!
* How Does the Individual Experience Tics?
* Can Tics be Suppressed?
* How Does TS Usually Start?
* Tourette’s Over the Life Span
* What Other Conditions are Associated with TS?
* So What’s That Cursing Stuff?
* Psychosocial Consequences of TS and Quality of Life Issues
* Educational Consequences of Tourette’s Syndrome
* What Causes Tourette’s Syndrome?
* Stress, Emotions, and TS
What is a Tic?
A tic is usually defined as a brief, repetitive, purposeless, nonrhythmic, involuntary movement 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.” A child with an eye blinking tic, for example, will exhibit a bout of eye blinking, and not just one eye blink.
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, and neck region, such as eye blinking, head jerking, shoulder shrugging, mouth grimacing, etc. Simple vocal tics include throat-clearing sounds, grunting, sniffing, and coughing.
In contrast to simple tics, 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). A chart of common tics (pdf) is presented in a separate table.
In addition to tics that we can see and hear, some people report having “internal” or nonobservable tics of internal organs, but those reports are anecdotal and there has been no research to explore these reports.
Tics are generally understood to be “involuntary” or “unvoluntary.” The latter term may be more consistent with the experiences of adults with TS who report that on some level, they feel like they are choosing to release the tics or “let them out.” The majority of adults in one sample described their tics as a “voluntary response” to an “involuntary sensation.” Most young children would probably describe their tics as involuntary, however, and not being under their control. The issue of whether tics are really “Voluntary” or not has important implications for treatment as well as parenting and educational accommodations or interventions. At the present time, I think the safest approach is to view them as involuntary in children under the age of 8-10, and unvoluntary but possibly modifiable in those who are older. The fact that they may be modifiable, however, does not mean that we can assume that an older child, teen, or adult would be able to modify them on their own without training or support.
The Tic Spectrum and Tourette’s Syndrome
Tic conditions represent a spectrum ranging from a simple tics that lasts only weeks or a few months to situations in which there are many tics that change in their anatomic location and frequency over time and tics are present for more than a year. Within each type of tic condition, there is also a range of severity from mild and infrequent to severe and frequent.
A significant minority of all children will experience one, or even a few, tics at some point in their development. If tic(s) are present for less than a year and do not recur, we say that the child has 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, 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’s Syndrome.
As you read online or offline, you may see references to “Tourette’s Disorder” or “Gilles de la Tourette Syndrome.” These are just other names for TS, and there is no difference in meaning.
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 and would include obsessions, compulsions, and other features as being part of Tourette’s, for now, we will use the term as it is defined in the Diagnostic and Statistical Manual (DSM-IV-TR) since that is the primary reference that U.S. mental health professionals use in making diagnoses.
To simplify the diagnostic criteria for Tourette’s, let us focus on the criteria that most mental health professionals really do consider:
- Both multiple motor tics (i.e., more than two motor tics) and one or more vocal tics have been present at some time, although not necessarily at the same time or continuously;
- The tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than 1 year;
- Other causes of tics have been ruled out (see the next section for other conditions that can produce tics)
- Age of onset before age 18.
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 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.
What Other Conditions Can Produce Tics?
There are a number of medical conditions that can produce tics or mimic Tourette’s:
- Sydenham’s Chorea
- Wilson’s Disease
- tardive dyskinesia
- Lesch-Neyhan Syndrome
- cerebral palsy
- Huntington’s disease
- postviral encephalitis
- stimulant medications such as methylphenidate hydrochloride (Ritalin) may also produce tics or unmask tics in some children
- head injury
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.
Tics are also seen in association with other conditions such as Attention Deficit Hyperactivity Disorder, Autism Spectrum Disorders, including Asperger’s Syndrome, and Obsessive-Compulsive Disorder. If the tics are mild and infrequent, they may not warrant a separate diagnosis, but if the tics are problematic for the child, then they may warrant diagnosis and treatment.
Over the last 17 years, some intriguing but controversial studies have suggested that in some cases, streptococcal (“strep”) infections may trigger an acute-onset of TS or Obsessive-Compulsive Disorder or may trigger a dramatic worsening of symptoms in individuals who already have TS and/or OCD. For those who want to read more about this topic or who first noticed their child ticcing after a recent infection, see the “PANDAS” section of this web site. If the physician suspects that PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) is responsible, specialized blood tests may be ordered.
Tics and Tourette’s are Not Rare Conditions
For many years, TS was considered to be a rare disorder, but tics and TS are now recognized as fairly common childhood-onset conditions. There is still some controversy about its rate in the general population. 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 significantly underestimate rates in children), and whether the subjects were drawn from clinical settings or from the general community, to name but two factors. That said, a 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. These rates are somewhat higher than what was reported in UK schools.
Because symptoms of TS tend to diminish with maturation, the rates of TS are lower in older individuals. A recent review of data on TS from an international perspective and across the life span suggests that the prevalence of TS in the general population is slightly under 1% (Robertson, 2008).
Although there has been considerable variability in estimates of rates in children in mainstream schools, all recent studies have reported higher rates in special education settings than in mainstream settings. The disproportionate representation of children with TS in special education does not prove that it is the tics or TS that are leading to school problems, however, and parents of children with tics should not fear that the tics, by themselves, will lead to a placement in special education. At the present time, and based on limited available research, it may well be the case that children with uncomplicated TS are not generally in need of special education (as much as accommodations) although those who have TS in the presence of other conditions such as Attention Deficit Hyperactivity Disorder may be represent a population with increased need for special education.
Like many other conditions, TS is much more common in males than in females.
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. The sensations and internal events leading up to the expression of the tic are often referred to in the literature as “premonitory sensory phenomena,” “urges,” or by some other term. Prado and her colleagues (2007, 2008) review the literature on these experiences and note how they are reported in both TS and Obsessive-Compulsive Disorder patients, but moreso in TS. These urges or internal sensations or perceptions that something is “not just right” precede tics and compulsive behaviors.
Although one early study reported that 28% of patients with TS reported such urges or phenomena (Chee & Sachdev, 1997), a more recent study by Kwak et al. (2003) reported that over 90% of their sample reported premonitory urges prior to motor tics, with participants reporting that their most common sensation was an urge to move and an impulse to tic (“had to do it”).
Can Tics be Suppressed?
Tics can sometimes be suppressed, but most people’s experience is that the tics will eventually be released. Thus, if we were to ask someone who felt that the tics were consciously released to not tic, we might observe that they could suppress a tic for a while, but eventually, they would release it.
What happens if the individual tries to suppress the tics? 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, and recent research supports the notion that as the child matures, their awareness of their tics and their ability to suppress them increase.
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. Asking a young child who has tics to suppress them is generally not a good idea because the effort involved in suppressing the tics will distract the child from tasks requiring their attention and may decrease their accuracy on the task (cf, Conelea & Woods, 2008) .
Parents frequently report 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. For years, many of us considered these explosions of tics to represent an actual worsening of tics due to inhibition or suppression of tics during the school day. Recent research, however, casts some doubt on that interpretation. Data from some studies suggest that even when tics are being consciously and actively suppressed, there is no rebound effect (Himle & Woods, 2005; Verdellen et al., 2007).
Keeping in mind that each child is unique, the parents and child are often the teacher’s best source of information about a particular child’s tics and patterns. But if you are a parent or teacher who is thinking of discussing the child’s tics with them, also keep in mind that not all young children are aware of their tics. 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. And just talking about their tics may induce a temporary worsening of their tics (Woods, Watson, et al. 2001).
Banaschewski et al. (2003) conducted a survey on premonitory sensory phenomena (PSP) and suppressibility of tics in children and adolescents. Over a third of the children and adolescents in the sample reported experiencing PSP, and almost two-thirds reported being able to suppress their tics. Slightly more than one quarter of the sample (28%) reported both PSP and the ability to suppress tics, indicating that the majority of children and teens do not have awareness of sensory urges building up with the ability to suppress their tics. Changes in reports were noted at two ages developmentally. At age 10, there seemed to be an increased ability to suppress tics, and at age 14, there was more reporting of PSP. It is important to note that awareness of premonitory sensory urges did not precede ability to suppress tics, but rather, ability to suppress tics actually preceded awareness of (or experience of) premonitory sensory urges.
If tics are actively suppressed, what happens to any preceding urges? Do they diminish over time or do they intensify and then diminish, or what? Woods and colleagues found that suppressing tics when reinforcement was provided was associated with intensified urges during tic suppression for 5-minute periods (Himle & Woods, 2007). Their data provide some support for the notion that people tic to decrease or alleviate the unpleasant sensation that is building up. But what if we allow the period of tic suppression to go even longer? Would the urge continue to build or would it eventually extinguish? Verdellen et al. (2008) provide data indicating that the urge does decrease both within 2-hour sessions and between sessions using an Exposure-Response Prevention treatment. Exposure-Response Prevention for tics is the same approach used in treating Obsessive-Compulsive Disorder.
What factors predict ability to suppress tics or severity of sensory urges during training? Data provided by Woods, Himle, et al. (2008) suggest that errors of ommission on a continuous performance task correlated with ability to suppress tics, while Verdellen et al. (2008) found that tic frequency at baseline – but not tic severity – was correlated with reductions in frequency of tics and severity of sensory urges during training sessions.
So…. can tics be actively suppressed without any rebound effects or worsened sensory urges? The answer appears to be “Possibly, but not in children under 10, and not without risking distracting the person from attention-demanding tasks.” Hopefully, longer-term studies will shed light on whether suppression can become more automatic and less of a risk of distracting the individual. In any event, these studies offer hope and promise of nonmedication treatments that may be of value, and suggest that what we all described as “involuntary” may be more modifiable than we had recognized.
How Does Tourette’s Syndrome 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 (or tics), a few months may go by and then the tic may re-emerge or a new tic may appear. The tic (or tics) will increase in frequency and severity over weeks 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.
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 (PANDAS).
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 things, it used to take years before someone was properly diagnosed. In the last few years, however, we have seen evidence that children are being diagnosed sooner, but the delay depends on a number of factors, including awareness by doctors, presence of other disorders, and age of the child at time of onset of symptoms. For example, in Israel, the median delay to diagnosis was only 6 months, but it was longer in cases where vocal tics were the first presentation or where the child was older. The co-occurrence of other disorders resulted in a shorter delay to diagnosis (Shilon et al., 2008). In Denmark, the median delay to diagnosis was 2.8 years (Mol Debes et al., 2008), while in Poland, the delay in diagnosis was on the order of 3.9 years (Janik et al., 2007).
The Course of Tourette’s Over the Life Span
Most cases of TS are thought to be “mild,” meaning that the individual does not seek treatment and/or does not experience significant interference in their life from their tics. If tics become problematic, you may wish to consider treatment options (treatment options are discussed in the article, “Treatment of Tics and Tourette’s Syndrome,” also on this web site.
In the first years after tics first emerge, many people report that the waxing periods tend to worsen from one waxing cycle to the next. The child may experience more tics and/or more severe tics over time, and there seems to be a tendency for things to get worse before they get better. The good news is that for many 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 10 – 12 in the majority of cases, and that by age 18, half of the children may be virtually tic-free, with other children showing significant improvement.
In another study on changes over time, Burd, Kerbeshian, et al. (2001) followed 39 out of 73 patients who had been diagnosed with TS as children in 1984-1985. They reported that symptom severity decreased by 59%, global functioning improved by 50%, and the average number of comorbidities (other conditions also present) decreased by 42%. Similar to findings by Leckman, they reported that 44% of the patients were essentially symptom free at follow-up; 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.
Patient reports of symptoms improvement are often subjective, and a study by Pappert, Goetz, et al. (2003) suggests that many adults report being tic-free when they actually still have tics. In this study, the investigators compared videotapes of 31 adults with TS to the same individuals’ videotapes during childhood.They found that motor tic severity significantly decreased by adulthood, as did objective measures of overall tic disability. In contrast to previous studies that had relied on subjective reports, these investigators found that 90% of the adults still had tics. As a group, the adults were somewhat inaccurate in their self-assessment in the sense that those who reported having tics did, in fact, display tics, but 50% of those who reported being tic-free also displayed objective evidence of tics. The investigators suggest that tics may persist into adulthood more than studies using subjective reporting might suggest, but that when adults report improvement or being tic-free, it’s probably because of a significant improvement in motor tic severity.
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 or whose tics worsen in adulthood. As an example, Pappert et al. (2003) computed a “global tic impairment” score based on five tic factors (number of body areas with tics, motor tic frequency, motor tic severity, phonic tic frequency, phonic tic severity) and compared the childhood global tic impairment scores to the scores of the same individuals as adults. For their sample, they found that that 10 of the 31 adults in their study had worse objective global tic disability scores as adults. There was a reliable and moderately strong correlation between the severity of objective global tic impairment at childhood and the severity of objective global tic impairment in adulthood. Taken together, the data suggest that:
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 re-emergence of TS after decades of quiet.
Other Conditions Associated With Tourette’s Syndrome
Although there have been very few studies that have looked at this question in community-based studies, and there are certainly children and adults who have TS without any other conditions, a significant percentage of TS patients seen in clinical settings also have symptoms of other disorders or conditions. It is important, however, to keep this caveat in mind: we really don’t know what percentage of all people with TS will have other problems or disorders because what we see in clinical settings may represent only the more severe end of the spectrum and not the majority of individuals with TS.
As you read more about Tourette’s, you will probably encounter terms like “co-morbid” or “associated,” so we will begin by defining those terms as they are used on this web site. We say that two conditions are “comorbid” when we want to indicate that two conditions are present in the same individual. We say something is an “associated disorder” when there appears to be a significant probability that the two conditions will occur together at a higher rate than you would expect by chance.
When I first began talking to people about TS, I realized 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 Attention Deficit Hyperactivity Disorder or obsessive-compulsive symptoms. So to decrease confusion in our communication, in 1991, I introduced the term “TS+” to refer to individuals who have TS plus features of one or more other disorders such as Attention Deficit Hyperactivity Disorder (ADHD), Obsessive-Compulsive Disorder (OCD), anxiety, self-injurious behaviors, anger or rage outbursts, or depression, to name but some of the conditions that may be associated with or frequently comorbid with TS. The goal was to help people remember that not everything may be a tic of TS, and that the child may have other conditions that may be responsible for any impairment they are experiencing.
“TS+” is not a technical or diagnostic term, but rather a convenient way to remind ourselves that there is often other things affecting a child who has been diagnosed with TS. This is particularly evident when we examine school functioning. In the vast majority of cases I’ve dealt with over the years, it is seldom the tics that are the child’s or teen’s biggest problem.
Unfortunately, and despite my best efforts to remind people not to attribute everything to TS when it may be due to something else, all too many people continue to describe people with TS as having a variety of problems that may not be due to TS at all, but rather to some other condition. For example, one publication from the National Tourette Syndrome Association suggested that TS was linked with what used to be called Central Auditory Processing Disorder (CAPD), and yet there is not one study that shows any direct association between TS and what was called CAPD. Such imprecise writing does not further our understanding of TS. It is one thing to say that children with TS and Attention Deficit Hyperactivity Disorder may be more likely to have processing deficits, but it is quite another thing to say that children with TS are more likely to have processing deficits.
So, what other disorders tend to be associated with or frequently comorbid with TS? The answer depends on whom you ask and whether the research is based on a clinical sample or a community-based sample. That said, 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. [Note: some of these conditions are discussed in other articles on this web site.]
In a community-based study of school children that employed direct interviews and standardized rating scales for 1596 children, Kurlan and his colleagues found that OCD, ADHD, separation anxiety, overanxious disorder, simple phobia, social phobia, agoraphobia, mania, major depression, and oppositional defiant behavior occurred significantly more frequently in the children who had tics compared to their non-tic peers [Kurlan, Como, Miller, et al., 2002]. But are all of these conditions really associated with tics, or is it the case that some of them are just associated with each other? For example, could it be that you don’t find oppositional defiant behavior unless there is also ADHD? There is much that we don’t know about what is truly associated with TS and what conditions are only present if there is ADHD or OCD in the presence of TS. Additional research is clearly needed to help identify patterns.
Other research also suggests that children with Tourette’s tend to be shorter and weigh less than children with ADHD. Problems with conduct disorder or self-injurious behavior may be more common in those children with TS who are in the lowest quartile of height and weight. [Zelnik et al, 2002].
Some studies, such as that by Kwak et al. (2003) indicate that migraine headaches are significantly more common in those with Tourette’s than would be expected based on the general rate of migraine headaches in the population. The presence of migraine headaches did not appear to be linked with any obsessive-compulsive factors.
Overall, based on research and experience, the following are conditions parents of young children with TS need to be alert to (adapted from Packer & Pruitt, 2010):
- Attention Deficit Hyperactivity Disorder (ADHD)
- Obsessive-Compulsive symptoms or Obsessive-Compulsive Disorder (OCD)
- Visual-motor integration problems (including fine motor control, copying from the board, and handwriting)
- Anxiety disorders
- Speech dysfluencies (e.g., stuttering or cluttering)
- Sensory defensiveness
- Self-injurious tics
- Sleep problems
- PANDAS (when Strep infection worsens tics)
- Depression (especially if ADHD, OCD, or anxiety disorders are already present)
- Bipolar Disorder (especially if ADHD and/or OCD are already present)
- Learning disabilities
- Executive Dysfunction (if other comorbid conditions are present)
- Working memory deficits (especially when other comorbid conditions are present)
- “Rage Attacks” (especially when other comorbid conditions are present)
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, although adults with TS who are seen in clinics generally present because of problems in anger management. In one of the few studies that looked at the question of personality disorders, 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’s or rather to a comorbid condition — or whether it was the result of having been misunderstood during their childhood and adolescence.
So where does that leave parents of newly diagnosed children? From my perspective, parents of newly diagnosed children need to be aware that:
If your child is struggling at home or in school or socially and the behavior or symptom doesn’t really appear to be a tic, then be curious about what else might be going on, and find out about the other conditions described on this web site.
So What’s That “Cursing” Stuff?
Although Tourette’s has been stigmatized by the media as the “Cursing Disease,” only a minority of individuals who have Tourette’s experience unvoluntary outbursts of socially inappropriate or taboo expressions. A number of studies suggest that less than half of individuals seen in clinics that specialize in Tourette’s may have this type of symptom, and that it occurs in less than 10% of children with TS. 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 these complex vocal tics that are called “coprolalia.”
Coprolalia is not required for diagnosis of TS.
Some coprolalia is immediately obvious as a tic: the individual may have one obscene or socially unacceptable 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.
Psychosocial Consequences of Tourette’s
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 but the individual places himself or herself under a self-imposed stress that may actually exacerbate their tics.
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, 2005). Even young children may reject peers for their tics. Research by Stokes (1991), Friedrich et al. (1996) and Woods and his colleagues (2005) 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 some preliminary data (Marcks et al., 2007; Woods & Marcks, 2005) suggests that preventive disclosure and peer education may be helpful.
In light of the data on peer teasing and rejection due to tics, it is not surprising that some studies of quality of life (QOL) measures would indicate a difference between youth with TS and those without. Storch et al. (2007) found that QOL scores in children and teens with TS were significantly lower than non-TS controls, and that tic severity was moderately associated with QOL in children and adolescents. More recently, however, Pringsheim et al. (2008) compared children with TS-only, TS+ADHD, TS+OCD, and TS+ADHD+OCD to normal controls on a variety of measures of psychosocial functioning. For the TS-only children, only the family activities domain was significantly affected. The most significant predictor of psychosocial impairment was ADHD symptom severity. For children with TS-only, psychosocial health was not different from that of the general population in the majority of measures. The investigators suggest that treatment of ADHD and OCD should be the priority in children with multiple diagnoses.
Decreased QOL is also reported in studies of adults with TS. As one example, Elstner et al. (2001) assessed QOL in 90 adult patients with TS and found that scores of 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 factors 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%). More recently, Gorman et al. (2010) evaluated teens at age 18 after they had been evaluated as children and found that those with TS+ scored lower on measures on global psychosocial functioning. Preliminary analyses of their data suggest that ADHD contributed significantly to overall impairment, as did OCD, but even after these factors were accounted for, TS still predicted worse psychosocial functioning.
Although the findings described above may be cause for some concern, what none of the available studies address is whether treatment might change the psychosocial outcomes, and what factors in the child’s or teen’s life might be protective factors.
That said, one factor that has been identified as possibly predicting more severe impairment, however, is fine motor deficits and visual-motor integration deficits. Bloch et al. (2006) found that poor performance on measures of fine-motor coordination and visual-motor integration as children predicted worse adulthood tic severity for the former measure and worse adulthood tic severity and worse global psychosocial functioning for the latter measure.
Educational Consequences of Tourette’s Syndrome
Does the presence of tics or Tourette’s Syndrome impact educational functioning? All available research and clinical experience suggest that it does, but the assessment of its impact has been somewhat muddied by the presence of comorbid conditions.
Pappert, Goetz, et al. (2003) found that 52% of children seen in their clinic experienced social or educational dysfunction. Of these, 39% required special education placement, 10% had been retained at least 1 year in school, and 29% experienced disciplinary problems. Their findings are consistent with other published reports from tertiary clinics, but it is their follow-up data on these children as adults that is of special interest and concern here. Whereas half the sample experienced significant social and education dysfunction as children, (only) 32% of the same sample experienced social or education dysfunction as adults. On a positive note, all of the sample participants had completed high school and at least two years of college (adult sampling was conducted while the individuals were in their 20′s). The investigators found that 71% of the adults were currently employed or pursuing their education. Of the adults who had social or educational dysfunction as children, 50% had social or education dysfunction as adults, and 13% of children who had not experienced education or social dysfunction went on to develop social or education dysfunction. Out of their sample of 31, then, while the majority were doing well and were well-integrated in their communities, over one quarter of the adults were disabled with problems that included alcohol abuse, unemployment or criminal activity. That these measures did not correlate with tic severity measures but did correlate with early childhood dysfunction suggests that early and effective interventions for comorbid conditions (perhaps even more so than for tics) may impact on the prevention of dysfunction in adulthood.
When reading reports on the educational impact of TS, be careful to consider whether the sample consists of youth with TS-only or TS+. If your child only has tics without any other comorbid symptoms or conditions, they may do quite well in a regular classroom as long as appropriate accommodations are made for interference they may experience from their tics. Also consider whether your child suffers from fine motor control impairment or impaired visual-motor integration, as those two functions have a significant impact on academic functioning. The school’s occupational therapist and psychologist can screen for impairment in those domains.
What Causes Tourette’s Syndrome and Is There a Cure?
Tourette’s Syndrome (TS) appears to be familial (i.e., it “runs” in families) in the vast majority of cases, suggesting a genetic component or an inherited vulnerability to it, but our understanding of the mode of genetic transmission is incomplete. At the present time, it appears that there is likely to be a significant genetic factor, although prenatal, perinatal, autoimmune, and environmental factors may contribute to or modulate the severity of symptoms.
Gender is also linked to expression of TS: boys are significantly more likely to develop TS than are girls. One question adults often ask when they are thinking of starting a family is whether there are any tests such as amniocentesis that can screen or test a fetus for Tourette’s Syndrome. The answer is “not at this time.”
In some of the seminal research on this topic, Pauls et al. (1986) reported that male offspring would 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 had lower probabilities of showing symptoms of these disorders, but there was still a fairly high probability (.70) that a female child would have one of these disorders.
In more recent research, however, McMahon, Carter, Fredine, and Pauls (2003) approached the problem somewhat differently by looking at the onset of TS in children whose parents have TS and comparing that to the onset of TS in children whose parents do not have TS. Children who were considered “at-risk” and control children were enrolled between the ages of 3 and 6 years and followed with yearly structured assessments over intervals of 2-5 years. Of the 34 at-risk children who were tic-free at baseline, 10 (29%) subsequently developed a tic disorder; 3 of those 10 met criteria for TS. None of the 13 control children developed a tic disorder, supporting the hypothesis of a significant genetic or familial contribution to the onset of tics. Similarly, Obsessive-Compulsive Disorder (OCD) or features or OCD emerged in 11 of the at-risk cases, but not in any of the controls, while Attention Deficit Hyperactivity Disorder (ADHD) occurred in 14 at-risk children but not in any of the controls.
The data described above provide support for familial transmission of TS as well as an association between tics, obsessions and compulsions, and ADHD. The study also provided additional information on whether having two parents with TS increased the risk compared to cases where only one parent has TS. When the rates in offspring were compared, the investigators found that the rates of TS in children with two affected parents were three times the rate observed for children with one affected parent.
A second common question is whether there is any way to predict how severe a child’s symptoms might be based on what the parent experienced if the child does develop TS. Based on available reports, there is currently no clearly identified 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 yet 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. 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.
There is no cure for Tourette’s Syndrome at the present time.
Lest the preceding sound somewhat alarming, it is encouraging to note that family studies suggest that most cases of TS are “mild.”
In addition to what appears to be a genetic vulnerability or component, some research also suggests that prenatal events or factors (such as maternal smoking, drug use, exposure to toxins) and perinatal events (such as jaundice, infections) may be associated with severity of TS. While some factors do not necessarily cause TS, they may be correlated with symptom severity in children who do develop TS. Some of the environmental factors that may affect symptom severity are mentioned later in this primer in the section on stress.
While some investigators look to identify specific genetic contributions to TS, other investigators have focused on using neuroimaging techniques to try to identify whether TS is related to a specific abnormality in one or more brain structures or whether it is due to dysfunction in the way the brain’s circuitry functions. Some studies do report differences when comparing the brains of those with TS to those who do not have TS. A growing body of research also points to the role of structures in the brain known as the basal ganglia. A recent study by Sowell et al. (2008) used magnetic resonance imaging (MRI) to look at the cortex of individuals with TS. They found cortical thinning in the frontal and parietal lobes compared to non-TS control children. Of particular significance, the thinning was most prominent in the sensorimotor areas of the cortex that control the facial, orolingual and laryngeal musculature. Because facial tics and vocal tics are part of TS, their findings are particularly helpful in explaining some of the neurological bases for tics. Other researchers further expand our understanding by their study of white matter changes in the brain (Siebner et al., 2009).
Other research has addressed the neurochemical transmitters that might be involved in TS. As you read about Tourette’s Syndrome, you will also 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. Studies on dopaminergic function in individuals with TS has produced some fascinating results.
Stress and Tourette’s Syndrome
While stress does not cause TS, it would seem almost intuitively obvious that stressors might make the symptoms worse for at least some individuals with tics. Stressors might take the form of environmental or ambient weather factors such as temperature, illness factors such as infection, or might include potential psychosocial stressors such as hassles on the job or in school, demanding environments, breakup of romances or marriages, death in the family, or any major life event.
With respect to environmental factors, children or adults with TS may find that their symptoms are worse during allergy seasons, and 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). Environmental factors were also noted to be significantly correlated with tic severity in another report by Snider et al. (2002), who found that motor tics and problem behaviors were significantly higher during the winter months of November through February when compared to the spring months of March through June (a finding which does not match this author’s own clinical experience that suggests that although tics are likely to worsen in the winter, they also worsen in the spring and the behavior problems are often worse in the spring; these patterns correspond to allergy seasons in this geographic area).
Because correlations between physiological responses and ambient environmental factors may not be present for all individuals and because such correlations may also vary by region and direction of correlation in individuals exposed to the same environmental conditions (Wenger, 1959; Packer, 1979), more research is needed before we can truly understand the contribution of ambient environmental factors to variability in tic severity.
In the past few years, a few additional studies have shed new light on the question of the relationship between everyday types of stressors and tic severity. For example, Findley and her colleagues (2003) reported that children with TS and OCD reported greater psychosocial stress than did their control peers, and that the reported level of psychosocial stress correlated with self-reports of tic and obsessive-compulsive symptom severity.
Wood et al. (2003) attempted to determine if emotional state was related to tic factors, and if any changes in tics were mediated by changes in the autonomic nervous system. In their study, four children with TS were videotaped while they were watching a movie. The investigators found that tic severity was worst during periods associated with anticipation, resolution of emotional changes, and lower concentration, while tic severity was lowest during periods of anger and happiness, and intermediate in severity during periods of sadness and fear. Tic severity did not correlate with autonomic changes in heart or respiratory rate. It is important to note that both anger and happiness were associated with decreased tic severity, suggesting that it is not necessarily the positive emotional valence (happiness) that is associated with tic reduction.
Using a self-report method of stressors and tic severity over a 12-week period, Hoekstra et al. (2004) looked at the relationship between stressors and tic severity in an adult sample with TS and in a pediatric sample. Throughout the study, participants filled out weekly questionnaires about the occurrence of small life events (SLE) — the type of events most of us would consider “daily hassles” — and they rated their own tic severity. In looking at the group data, there were no correlations between SLE and tic severity measures in the pediatric group, and a weak but statistically significant (i.e., reliable) relationship between negative small life events and tic severity in the adult group. Overall, and keeping in mind the limitations of their design (including the lack of objective verification of tic severity), the expected relationship between SLE and tic severity was not confirmed.
Because group data and group analyses can sometimes obscure significant effects within individuals, the investigators also looked at individual correlations. They reported that a minority of the pediatric sample (21%) and adult sample (18%) did exhibit significant association between the reported frequency of small negative life events and reported tic severity. To complicate the picture even more, however, the direction of the correlation varied across individuals: in some cases, negative small life events were positively correlated with tic severity, but for other individuals, negative small life events were negatively correlated with tic severity. The investigators conclude, “Contrary to traditional views, in general, life events do not account for changes in tic severity. Only in a minority of tic disorder patients do fluctuations in symptom severity appear to be associated with possibly stressful small life events.” Since their study was limited to small life events and not major stressors or what might be perceived as major stressors, their findings may actually be more applicable to the kinds of everyday stressors children and adults with TS encounter in school or on the job. Hopefully, their research will inspire more research on this important topic. The investigators appropriately noted the limitations in their design and methodology, but in my opinion, this exploratory study is quite important for a number of reasons, not the least of which is that it reminds us that sweeping generalizations and “common knowledge” or “common sense” may not always lead to an accurate understanding of phenomenon.
The relationship between infections (such as strep infections) and tics and OCD has been studied by a number of researchers; that research is summarized in the PANDAS section of this site. Interestingly, a new study by Lin et al. (2010) suggests that for a subset of children with tics and early-onset Obsessive-Compulsive Disorder, the presence of an active infection may not predict an increase in tics in the immediate future, but magnifies the impact of psychosocial stressors in predicting future tic and OCD severity.
One other stressor that has recently been investigated is the presence of allergic diseases. A study by Chang et al. (2011) using a large Taiwanese database found a significant correlation between allergic diseases and TS whereby children with allergic rhinitis were twice as likely to have TS as non-allergic controls. The greater the number of allergic comorbidities and the older the child, the greater the risk of TS. Their study did not correlate tic severity or frequency with the presence or absence of specific allergic symptoms, however. Thus, my hypothesis has yet to be tested and it would make a great doctoral dissertation for someone. If we find that allergy symptoms correlate with tic symptom worsening, then does treating the allergic condition reduce tic symptom severity? Instead of just suffering mild seasonal allergy symptoms, should we be treating them more aggressively? As I said, it’s an area that needs to be researched.
If stress does make the symptoms worse for a particular child or adult, does relaxation make the symptoms 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.