ALL ABOUT TOURETTE'S SYNDROME

"Because there are so few anatomic (by imaging) or neurobehavioral abnormalities associated with pure Tourette syndrome, it is important to inform the parents of these children, who comprise 40% of all children with Tourette syndrome, that their future is not burdened with the same issues as those of the remaining 60% of children who have comorbid ADHD with Tourette syndrome."  Martha Bridge Denckla, MD.  "Attention-Deficit Hyperactivity Disorder (ADHD) Comorbidity: A Case of "Pure" Tourette Syndrome?"  J Child Neurol. 2006 Aug;21(8):701-3.
 

Table of Contents
Terms relevant to Tourette Syndrome                                    
Spectrum disorder/syndrome
Comorbid
Ascertainment or referral bias
Clinical sample/population
Epidemiological study/broad-based population sample
DSM-III-R, DSM-IV, and DSM-IV-TR diagnosis of Tourette's disorder
Tourette Syndrome Plus

Tourette's Syndrome Concepts
Comorbid Conditions
Learning Disabilities in Tourette's
Classroom Accommodations for Tourette's
The "2/3 rule"
Other Conditions and Tourette's
Medicatio for Tourette Syndrome
" Rage " attacks, anger, temper and Tourette Syndrome

If you’re new to Tourette Syndrome, please read this section carefully; these terms and concepts will help you better interpret the rest of what you will read as you learn about Tourette's.

Tourette Syndrome is a neurobiological condition resulting in motor and vocal tics.  Some common tics include coughing, throat clearing, sniffing, blinking, shoulder shrugging, arm thrusts, or neck stretching.   Some researchers believe that a subtype of OCD (obsessive-compulsive disorder) may be genetically linked to Tourette's.  There’s not too much else that has been conclusively shown to be "part of" Tourette Syndrome.  A lot of literature about Tourette's is misleading, and sorting it all out can be very difficult.  The following topics, concepts, clarifications and definitions may be useful as you read those sites about Tourette Syndrome. 

Terms

A spectrum disorder occurs along a continuum, from severe to mild and a syndrome is a medical condition that is diagnosed based on a history of a collection of symptoms rather than via a specific medical test.  There is currently no clinical means of medically testing for Tourette's:  it is diagnosed based on a history of tics which wax and wane and had a childhood onset, and after ruling out other conditions which might cause tourettism or other secondary causes of tics.  Tourette's syndrome is a spectrum disorder, with VERY few people falling in the severe range.  Claims that Tourette's is on the "same spectrum" as autism (e.g.; Pervasive Developmental Disorders), ADHD (attention deficit/hyperactivity disorder), bipolar (manic-depression), etc. are controversial and not accepted by all researchers.  Milder expressions of Tourette Syndrome are probably less likely to be recognized and/or diagnosed, and a recent research suggests that the vast majority of Tourette's goes undetected.   Back to Top

Comorbid refers to a medical condition that is present along with another medical condition, and doesn’t necessarily mean their causes are related or one leads to the other, etc.  Some people mistakenly interpret the term comorbid to mean separate medical conditions that are related to the same cause or genetically linked.    Back to Top

Ascertainment is the way persons with a trait are selected or found for genetic or medical studies and bias is a difference between the estimated and true value in a statistical sample.  Ascertainment bias and referral bias refer to inaccurate estimates because the study was biased, often by the presence of another condition.  If you surveyed all persons referred to a psychiatric clinic for Tourette Syndrome, you would find a high rate of psychiatric disorders in Tourette's patients  (the fact that the survey was done in a psychiatric clinic rather than in the general population would bias the results.)  Since other conditions are likely to be what brought the person with Tourette's to the clinic to begin with, ascertainment bias caused distorted representations of those conditions in patients with Tourette syndrome.  Comorbidity rates may be overstated in clinical samples of Tourette syndrome patients because other conditions may be what caused the difficulty that brought the person with tics to the clinic to begin with.  In conclusion, don't let reports of high comorbidity and/or psychopathology in clinical samples of persons with Tourette's overly influence you, and learn to read the studies carefully and understand the study population.     Back to Top

A clinical sample refers to a study done on patients from a clinical population — patients from a clinic or hospital.  This can be a good way to conduct research, but since persons who are in worse shape or have multiple diagnoses are more likely to come to a specialty clinic, these samples may reflect only the worst-case scenario.  You must carefully interpret EVERY statement that includes wording such as who seek medical attention or in clinical samples.  NOTICE the population sample that the study is based upon.  Some statements may not reflect the type of Tourette Syndrome more likely to be diagnosed today and may be affected by ascertainment bias.  Be even more wary of statements like "50% of persons with Tourette's have ADHD."  The more correct statement would begin with, "In clinical populations, 50% ..."    Back to Top

Epidemiological study or a broad-based population sample refers to a study done on patients obtained from an entire population, for example, all of the third grade children in a certain school district.  There have been very few studies of this type on Tourette's syndrome subjects.  This type of research has some drawbacks, but as long as Tourette's remains a highly underdetected and misdiagnosed condition, clinical samples may not reflect the same trends that one would find in a broad-based population study.  Those persons who are coping well or who don’t have comorbid conditions or complicating environmental factors may be more likely to be identified in broader epidemiological studies.  For instance, Robertson's study in the U.K. looked at all children in an entire school district of a certain age, and found that Tourette syndrome was vastly under-detected.     Back to Top

DSM-III-R or DSM-IV or DSM-IV-TR is the Diagnostic and Statistical Manual of Mental Disorders, the reference still used to diagnose Tourette's Disorder in the U.S.  DSM-IV added the "significant distress" criterion, meaning that the fourth revision considered that you don’t have Tourette's disorder, even if you meet all the other criteria, unless you have "significant distress."  This means that any study using DSM-IV diagnostic criteria may only reflect more severe Tourette's.  The "significant distress" criterion was dropped from the most current revision,  DSM-IV-TR.   Furthermore, chronic tics are thought by many Tourette syndrome researchers to be due to the same underlying, genetic mechanism as Tourette's, so distinguishing between Tourette's and chronic tic disorders may be useful only for research purposes.  (See Classifications of Tic Disorders on Tourette Syndrome Links page.)      Back to Top

Tourette Syndrome Plus is a term coined by Leslie Packer, Ph.D., referring to patients who have Tourette Syndrome plus features of one or more other disorders.  The term was intended to help people remember that not everything may be a tic of Tourette's.  There may be other comorbid problems that are actually impacting more than the Tourette Syndrome.  It was intended as shorthand for communications purposes, but newcomers may not be aware that many statements about TS+ may not apply to most persons with Tourette's, or that some of these other disorders are not always or often present in persons with Tourette syndrome.  Care should be taken to use and interpret the term as Leslie intended.  Many of the difficulties associated with TS+ probably arise not because of the Tourette's, but because of the other conditions which may also be present in some cases.  For instance, when considering the specific impairment or issues of any particular individual, it may be more useful to think in terms of ADHD plus tics, or bipolar plus tics, rather than "Tourette syndrome plus" -- terminology which rolls all behaviors under the Tourette's umbrella may be misleading in terms of treatment and attention to the issues at hand, and may lead to further misunderstanding about the nature of the core condition of Tourette syndrome.   It is unfortunate that the terminology "TS +" may lead some to believe that the other issues are secondary to Tourette's, rather than considering the terminology, for example, "ADHD+" or "bipolar+" when tics are also present with these conditions.  The other conditions are often far more impairing than the tics, and should take treatment priority; hence, rolling symptoms into a Tourette's spectrum umbrella is misleading.    Back to Top

Concepts

The terms provided above will help you use your own knowledge to better interpret the Tourette Syndrome literature.  Some of the more common concepts, misconceptions and inaccurate generalizations are discussed here:

Comorbid Conditions Those with more comorbidities (ADHD, OCD and others) are more likely to come to clinical attention and receive a Tourette's diagnosis.  As awareness of Tourette Syndrome improves and Tourette's becomes increasingly more detected, our views on comorbidity may change.  Until there are epidemiological studies on the rates of comorbidity in TS, view all numbers with skepticism, since rates quoted are generally based on clinical samples only.  The comorbid presence of ADHD makes it far more likely that Tourette's will be diagnosed, so rates of ADHD comorbidity may be overstated.  Don’t assume ADHD (or any other condition) will present in your child or your case and don’t fail to carefully consider other sources of what appear to be attentional issues, such as boredom, tic suppression, depression, bipolar disorder, etc.  Also, keep in mind that comorbid conditions don't mean life is going to be awful !  The distinction is made between "TS only" and "TS+" so that you can be sure to get to the source of any difficulties which may arise and get the right diagnosis and, hopefully, the right treatment. 

"The increased prevalence of these disorders in TS clinic populations (and perhaps that of ADHD as well) therefore most likely reflects a clinic ascertainment bias in which children who have multiple disorders are more likely to present to clinic than are children who have just a single illness.   Children who happen by chance to have both tics and disruptive behaviors, for example, may come to clinical attention primarily because of their behavioral disruption.  At the time of clinic evaluation, tics are noticed, TS is diagnosed, and the behavioral disruption is erroneously attributed to TS.  The treatment implication here is that the child's comorbid illnesses, not the tics, are often what require treatment."   Peterson and Cohen, The Treatment of Tourette's Syndrome:  Multimodal, Developmental Intervention.  Presented at a closed symposium held in New Orleans, Louisiana on April 12, 1996.  No longer online at psychiatrist.com/psychosis/worldwide/current/tourettes.htm

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Learning Disabilities in Tourette's   Many recent studies have shown that the presence of learning disabilities in clinical populations of persons with Tourette's seems to be largely explained by the presence of comorbid ADHD.  

"A new study suggests that AD/HD may, in fact, account for much of the impairment seen in patients with tic disorder ... children in both groups (tics plus AD/HD and AD/HD alone) had an almost identical patterns of cognitive impairment, lower academic achievement, arithmetic learning disabilities and impairment in global assessment of functioning ratings ... Dr. Spencer emphasized that treatment of AD/HD may be sufficient in those patients with tics plus AD/HD and there may be no need to treat specifically for tics.  Only rarely do tics cause impairments and they tend to improve with time even in the absence of pharmacotherapy, he said."   From "DG DISPATCH - AACAP: AD/HD A Major Factor In Tic Disorder," Lara Pullen, Chicago, IL, October 27, 1999 (see TS Links):  

Comorbid ADHD makes it more likely that a person with tics will come to clinical attention, so the rate of learning disabilities in all persons with Tourette Syndrome is probably overstated in the literature and may be no higher than in the general population.  However, visuomotor integration deficits resulting in poor handwriting may be associated with Tourette's and should be tested for in all children with Tourette Syndrome.  

Furthermore, OCD can also cause academic difficulties, but in a different way.  Some examples are perseveration, perfectionism, relentless editing and erasing/correcting of work, and the inability to prioritize, as in cleaning and organizing one's desk rather than actually doing the work.   But, as Tom Benedict points out, "These same things, turned around, can be seen as strengths if not taken to extreme.  Perseveration becomes, 'Gets the job done, no matter what.'  Perfectionism becomes, 'First drafts look like final drafts.'  And so long as it doesn't interfere with other work, being organized isn't a bad trait."   For help with academic issues, see Leslie Packer's TS "Plus" website.      Back to Top

Classroom Accommodations There has been very little research in this area, yet one encounters broad statements and recommendations, sometimes based only on personal experience with small or biased samples.  These recommendations may be based only on samples of children with multiple diagnoses or may have more to do with ADHD than with TS/OCD.  Accommodations based on clinical observations may not benefit all children with Tourette's: I have encountered many often-repeated suggestions that would be highly counterproductive for my sons.  Do not assume that suggested accommodations are ALL in your child’s best interest, or that you should attempt to apply them all.  You know your child best: does the suggestion really make sense for him/her?  Don’t assume, rather give careful thought to suggested accommodations given your child’s diagnosis.  Unnecessarily sending any child the message the he or she is not capable may do more long term harm than good, and some children may come up with surprisingly good coping mechanisms when left to their own devices.    Back to Top

The "2/3 rule,"  put forth by the book, Teaching the Tiger, and at Tourette Syndrome Association conferences, is an example of the kinds of issues that affect much of the Tourette's literature and have extended beyond the literature into popular use.   From p. 39 of Teaching the Tiger by Marilyn Dornbush and Sherry Pruitt, Hope Press, 1995, 

"When designing modifications, clinical experience suggests that the student's cognitive, behavioral, social and emotional age equivalents are approximately 2/3 the student's chronological age.* For example, a neurologically-impaired 12-year old may have a functional age of 8." * Barkley, R. A. New ways of looking at ADHD. (Lecture, 1991). Third Annual CH.A.D.D. Conference on Attention Deficit Disorders, Washington, D.C.

There is no print or peer-reviewed, medical journal reference for verification of the content of this information;  there’s no clear indication of what population Barkley, an ADHD expert, was referring to;  and there are no indications that he was referring to children with Tourette's at all.  But this "rule," implying a global level of developmental delay in a well-known book ostensibly about Tourette Syndrome, has been put forward at Tourette Syndrome Association conferences and has become generalized to ALL children with Tourette's, something that is not backed up by any research!  The statement should be referenced, qualified and used more carefully.  This "rule" in fact, was explained by Barkley in a later paper published at SchwabLearning.com, where it was clear that he was speaking about delays found in AD/HD.  Simply stated, there is no evidence that the majority of children with Tourette Syndrome have severe hyperactivity or global delays in multiple domains of functioning, as was implied by Dornbush and Pruitt.  Authors fail to consider ALL children with Tourette Syndrome when they make these types of statements in print or at conferences.  Statements that apply to some children with multiple diagnoses may not be applicable to the majority of children with Tourette's, given what we now know about ascertainment bias and the probable rates of undiagnosed Tourette Syndrome.  Be aware of such frequent, sweeping generalizations often heard at TS conferences, and insist that speakers clarify their populations and discuss ALL persons with Tourette's rather than only those with TS/ADHD.    Back to Top

Other Conditions The presence of comorbid mood or anxiety disorders (such as bipolar disorder) or developmental disorders (autims, PDD, Asperger’s) can alter the course and severity of Tourette Syndrome, making for a rougher ride with Tourette's.  Also, it can be very difficult to distinguish between ADHD and childhood bipolar, and bipolar may often be misdiagnosed as ADHD (see links at the end of the TS Links page).  With the possible exception of OCD, these other conditions have not been associated (genetically) with Tourette's; yet you will hear more from persons with comorbid conditions because they may be having a much harder time with Tourette Syndrome than others.  Tourette Syndrome plus comorbid diagnoses appears to be very different from Tourette's uncomplicated by other diagnoses.       Back to Top

Medication  Tourette syndrome experts advocate for the "judicious" use of medication, only when tics or other behaviors significantly interfere with functioning, and using the lowest dosage possible, not attempting to completely eliminate tics.  MOST people with Tourette's do not need medication or choose not to use it because the side effects outweigh the benefits, and today there are better pharmacological options than the older neuroleptics.   But this is often glossed over and we routinely hear of doctors who prescribe Haldol, Orap or Risperdal as the first medication for someone reporting adequate social and academic functioning and little interference from tics !!   The overall effect of these medications — both intended effects and side effects — can be worse than tics, and if a doctor hands them out first, you may have the wrong doctor.  Often doctors seem to miss the point that knowledge and understanding is all that is needed to help many people with Tourette Syndrome.  If there’s not an immediate crisis, a Tourette's diagnosis doesn’t mean you need to start medication, so take time to learn as much as you can about your choices.  Changing the environment so that the tics are understood, are not a big deal and the child is encouraged not to view himself or herself as "defective" can make a difference.  Also, children should have a say in whether or not they want or feel the need for medication.  At times, the parent may be wanting to medicate a child who isn't worried as much about the tics as the parent is !    

"Because of the understanding and hope that it provides, education is also the single most important treatment modality that we have in TS."  Peterson and Cohen, The Treatment of Tourette's Syndrome:  Multimodal, Developmental Intervention.  Presented at a closed symposium held in New Orleans, Louisiana on April 12, 1996.  No longer online at psychiatrist.com/psychosis/worldwide/current/tourettes.htm

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"Rage" attacks have never been shown to be a part of Tourette Syndrome by any research.  However, in one preliminary study, "rage" has been correlated with the number of diagnoses along with tic disorders (including mood and anxiety disorders, learning disabilities, developmental delays, or processing deficits) that one has.  In other words, the more other diagnoses one has, beyond tic disorders, the more likely is the behavior that has come to be referred to as "rage."  Furthermore, "rage" is often seen with bipolar disorder (manic-depressive), depression, ADHD, and autism.  Yet, even though it is known that "rage" is not a symptom of Tourette's, we often hear the misnomer "Tourette's rage attacks."  Most persons with Tourette Syndrome do not have "rage."  The Tourette International Consortium (TIC) database shows a relative absence of anger-control issues (in fact, the absence of most issues) in persons with Tourette's only.  Additionally, there is no such diagnostic entity as "rage."  What has come to be called "rage" may be a different entity for each person, some "rage" may be due to other disorders or seizure activity, and it’s not even clear that everyone who uses the term "rage attacks" is referring to the same phenomenon.  Ascertainment bias may explain why we hear so often about "rage" and Tourette's — persons who experience "rage" are more likely to have more diagnoses and more likely to come to clinical attention and more likely to show up in support groups with anger-related issues.

Here are similar conclusions from a 2003 study:

RESULTS:  Children with TS-only did not differ from unaffected controls on the parent ratings of aggression and delinquent behavior or on the teacher ratings of conduct problems.  CONCLUSIONS:   Comorbid ADHD is highly associated with disruptive behavior and functional impairment in children with TS.   Disruptive behavior in children with Tourette's syndrome: association with ADHD comorbidity, tic severity, and functional impairment.  Sukhodolsky DG, Scahill L, Zhang H, Peterson BS, King RA, Lombroso PJ, Katsovich L. Findley D, Leckman JF.  J Am Acad Child Adolesc Psychiatry. 2003 Jan;42(1):98-105.

And, from another 1999 study:

CONCLUSIONS: These findings provide additional evidence that aggressive behaviour observed in children with TS may be associated with comorbid ADHD or OCD (6), independent of tic severity or age. This is consistent with the clinical observation that most TS patients have only minimal symptoms, which do not interfere with their daily functioning.   Aggressive behaviour in children with Tourette syndrome and comorbid attention-deficit hyperactivity disorder and obsessive-compulsive disorder.  Can J Psychiatry. 1999 Dec;44(10):1036-42.  Stephens RJ, Sandor P.

Persons with "rage" are advised to look beyond the tics of Tourette Syndrome for causes and treatment, avoiding heartbreaking medication trial and error from the incorrect assumption that these behaviors are coming from the Tourette's.  Quicker assessment of the comorbid conditions (such as mood disorders, autism, or other comorbid conditions) that may be leading to "rage" will be helpful.  For example, the early detection and treatment of bipolar disorder can help avoid a lot of medication trial and error, pain and heartache.  

If you're new to Tourette Syndrome, you may be reading and/or hearing a lot about "rage," and wondering how it applies to your child.  (I remember treading carefully around my son when his Tourette's first surfaced, incorrectly assuming that all sorts of behaviors would be forthcoming!  Now I treat him like the regular guy that he is.)  Those parents whose children have "rage" say you can’t miss it, and literature is available online and in print to understand how to help with "rage." (Ross Greene’s book, "The Explosive Child," is a very helpful primer on effective, flexible parenting for chronically explosive-inflexible children.  He explains the issues which may contribute to explosive and inflexible behavior and how good parenting techniques can help improve the situation.  "The Bipolar Child" by the Papolos explores the often-misdiagnosed condition of early childhood bipolar.)

Readers whose children with Tourette's syndrome do NOT seem to have "rage" shouldn’t worry about it any more than any other issue.   Parental anticipation or fear of uncontrollable rage reactions can certainly lead bright children to learn to manipulate their parents and may encourage behaviors that should not be excused as neurological in origin.  Those who question whether certain behaviors are TS-related may be looking at learned or manipulative or bad behavior.  Assuming that uncontrollable anger or other behaviors are part of Tourette Syndrome and can’t be helped is not going to help that child learn to get on in life — ultimately, our most important goal in raising any child.