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Inaccurate description of Tourette's
Disorder
published by Jankovic in the
New England Journal of Medicine (NEJM)
"The Internet has given voice to the masses in ways that will require greater vigilance by those who presume to speak with both authority and accuracy." Kathryn A. Taubert, former Tourette Syndrome Association national board member, Dr. Laura Does It Again, ABCNews.com, Wired Women, June 13, 2001. http://abcnews.go.com/sections/scitech/WiredWomen/wiredwomen010613.html
January, 2006.
Did your professional caregiver learn about Tourette's syndrome via on online
CME credit course from the UAB Health System (sponsored
by the University of Alabama School of Medicine
Division of Continuing Medical Education, signed by by
Leon S. Dure, MD) ? The online CME course, utilizing the inaccurate
Venn diagram, has now expired, but you might want to review your caregiver's
knowledge of the diagnostic criteria for the condition.
UAB Health System, Understanding Tourette Syndrome, Leon Dure, MD
September, 2004.
An
article in Science Magazine has a correct Venn diagram (noticeably
different from Jankovic's Venn diagram), showing the relationship between Tourette's
Syndrome,
tics and comorbid conditions.
Making Sense of Tourette's (PDF)
May, 2003. Joseph Jankovic has published an "adapted
and modified" version of his NEJM article at www.orpha.net/data/patho/GB/uk-Tourette.html, which no longer contains the
inaccurate definition of Tourette Syndrome, and now more correctly uses the terms
"some" and "often" in describing associated features.
The misleading Venn diagram and definition of Tourette's is interestingly missing from the new
"adapted and modified" article.
Tourette syndrome
Author: Professor Joseph Jankovic, M.D.
Scientific Editor: Professor Alessandro Filla
Adapted and modifed from Jankovic J. Tourette’s syndrome. N Engl J Med 2001; 345:1184-1192.
Creation date: May 2003
On 7/2/2002,
a family member of a patient of Dr. Jankovic has reported on an
internet bulletin board that Dr. Jankovic stated to her
that his article was approved by the Tourette Syndrome Association. This information has
not been confirmed.
If you have any concern for the unnecessary stigmatization and possibility of future misdiagnoses of people with Tourette's syndrome, please write to the addresses listed at the bottom of this page, asking that this misleading information be addressed. A sample letter is included here.
Joseph Jankovic, M.D. is a physician at the Baylor College of Medicine in Texas, specializing in Parkinson's disease and movement disorders, and a member of the Tourette Syndrome Association Scientific Advisory Board.
He wrote the following article, published in the widely-read New England Journal
of Medicine and further endorsed in the American Family Physician, the clinical
journal of the American Academy of Family Physicians:
Jankovic J. Tourette's syndrome.
New England Journal of Medicine, October 18, 2001; 345:1184-92.
psychiatry.wustl.edu/Resources/LiteratureList/2001/November/Jankovic.pdf
Figure 1 shows a Venn diagram with four intersecting circles. Tourette's syndrome is indicated to lie at the intersection of tics, ADHD, OCD, and behavioral problems, poor impulse control, and other behavioral disorders. For those who cannot access the pdf file (above), here is a drawing similar to the Venn diagram shown in the article, made by a child with TS whose mother is a member of an online TS support group (thanks for the drawing, Katie :-)
Under Figure 1 is the caption:
Figure 1. Clinical Hallmarks of Tourette's Syndrome.
The diagnosis is based on the occurrence of tics along with behavioral disorders, including attention - deficit -- hyperactivity disorder (ADHD) and obsessive - compulsive disorder (OCD). Other behavioral disorders include anxiety and mood disorders, learning disorders, sleep disorders, conduct and oppositional behavior, and self-injurious behavior.
The Venn Diagram, showing Tourette's to consist of tics plus ADHD plus OCD plus behavioral disturbances, is front and center on the page, making the greatest visual impact. The Venn diagram is incorrect: only tics are required for a Tourette's diagnosis. The statement under Figure 1, that "the diagnosis is based on the occurrence of tics along with ..." is also factually incorrect. While it would be correct to show a Venn diagram with some degree of overlapping areas of these diagnoses, it is not correct to represent that Tourette's syndrome lies only at the point where tics, ADHD, OCD and behavioral disturbances intersect.
Other excerpts from this article include:
Coprolalia, perhaps the most recognizable and certainly one of the most distressing symptoms of Tourette's syndrome, is actually present in less than half of patients with Tourette's syndrome.
A majority of patients with Tourette's syndrome have symptoms of attention-deficit-hyperactivity disorder, obsessive-compulsive disorder, or both at some time during the course of their illness (Fig. 1).
Other behavioral problems associated with Tourette's syndrome include poor impulse control and an inability to control anger, as a result of which some patients may have outbursts of temper, episodic attacks of rage, emotional storms, inappropriate sexual aggressiveness, antisocial or oppositional behavior, and symptoms of anxiety and depression. One of the most distressing symptoms of Tourette's syndrome is self-injurious behavior.
There are some misleading statements in this work. One wonders why a member of the TSA Scientific Advisory Board
does not subscribe to the basic DSM diagnostic definition of Tourette's Syndrome, and why the TSA has not refuted this article and information. Referring
to Tourette's as an "illness" is troubling to many, as TS is not a
degenerative condition, and not all persons with Tourette's syndrome consider themselves
"ill."
LETTERS TO THE EDITOR OF NEJM
Letters to the Editor, New England Journal of Medicine, February 28, 2002; 346:710. (Scroll down to page 710, 6 of 9.)
Tourette's Syndrome
To the Editor: The review of Tourette's syndrome by Jankovic (Oct. 18 issue) contains a Venn diagram (Fig. 1 of the article) that may be misleading. The figure suggests that Tourette's syndrome lies at the intersection of tics, attention - deficit -- hyperactivity disorder, obsessive - compulsive disorder, and other behavioral disorders. Although these disorders often coexist with Tourette's syndrome and even with each other, they are not necessary for the diagnosis of Tourette's syndrome. The presence of tics alone, not caused by other conditions, is sufficient for the diagnosis.
Joseph DeVeaugh-Geiss, M.D.
Duke University Medical Center
Durham, NC 27705
Jankovic's partial response, printed in the same NEJM edition {emphasis added}:
Dr. Jankovic replies:
To the Editor: I agree with Dr. DeVeaugh-Geiss that motor and phonic tics without coexisting disorders are sufficient for a diagnosis of Tourette's syndrome. On the basis of the personal evaluation of well over 1000 patients with Tourette's syndrome, however, I believe that Tourette's syndrome consisting of tics alone occurs in only a minority of the patients who present to our Movement Disorders Clinic - admittedly a biased sample of patients. According to a survey of 3500 patients with a diagnosis of Tourette's syndrome, only 12 percent had tics alone as the manifestation of the disorder (1). Thus, the Venn diagram was designed to draw attention to the frequent association of other disorders with tics and to highlight the importance of focusing on the whole person, rather than one particular symptom, such as tics.
Dr. DeVeaugh-Geiss refers to the criteria outlined in the Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV) (2), which include a requirement that the tics "cause marked distress of significant impairment in social, occupational, or other important areas of functioning." Because of this vague language, milder cases of Tourette's syndrome would be difficult to classify, and the criteria do not take into account the frequently associated behavioral disorders that may be more distressing than the tics. Because of these and other matters of concern …, the criteria will be modified in subsequent editions. Until then, I and others who engage in research on Tourette's syndrome prefer to use the classification developed by the Tourette Syndrome Classification Study Group (3)....
1. Freeman RD, Fast DK, Burd L, Kerbeshian J, Robertson MM, Sandor P. An international perspective on Tourette syndrome: selected findings from 3,500 individuals in 22 countries. Dev Med Child Neurol 2000; 42: 436-47.
2. DSM-IV.
3. The Tourette Syndrome Classification Study Group. Definitions and classification of tics disorders. Arch Neurol 1993; 50: 1013-6.
ROGER FREEMAN, M.D., TIC DATA, AN INTERNATIONAL PERSPECTIVE ON TS
A troubling aspect of Jankovic's response to Dr. DeVeaugh-Geiss is his
application of the TIC (Tourette syndrome International database Consortium)
data of the much-respected Dr. Roger Freeman. He references
Freeman's journal study in support of his claim that "only 12 percent have tics alone as the manifestation of the disorder."
(Freeman, Fast, Burd, Kerbeshian, Robertson, Sandor.
An international perspective on Tourette
syndrome.)
The following snips from that paper are of note (emphasis added):
There are some obvious designed-in limitations. The simplicity and brevity of the data entry form has succeeded in recruiting a large number of sites and individuals in a relatively short time. This is an advantage in the initial analysis of small subgroups and in generating hypotheses, but cannot fully settle the issue of unknown degrees of ascertainment bias.
The lack of a standardized assessment procedure and of information on interrater reliability represents the major weakness of this study.
Finally, even with the largest representation of sites and countries, generalization of these findings to non-referred cases cannot be supported.
Dr. Freeman's article responsibly and accurately acknowledged the limitations inherent in this data, due to the manner in which the data was collected. He states that the data is an entry point for looking at research hypotheses, that there are interrater issues, and that his data cannot be extended to non-referred populations. Any bias inherent in the TIC data is likely in the direction of underrepresenting "TS only" and overrepresenting TS when comorbid with other conditions. Clinically-referred data is known to overrepresent populations with comorbid conditions, so if anything, the 12% is likely to be an underestimate of "TS only."
It is generally agreed that a large but undetermined proportion of individuals with TS remains undiagnosed in the community (Robertson and Gourdie 1990, Bruun and Budman 1997, Peterson and Cohen 1998 ) .
The small proportion of individuals with TS only reflects a clinical and epidemiological dilemma: most individuals with TS seen and followed in clinics are comorbid and therefore contribute to the idea that TS is necessarily associated with other disorders and behavioral problems, as well as high rates of coprolalia and SIB. However, the prevalence of behavioral problems in the TS only group may not differ from the general population (Caron and Rutter 1991, Carter et al. 1994, Costello et al. 1996, Budman et al. 1998, Ozonoff et al. 1998, Sherman et al. 1998, Spencer et al. 1998, Stephens and Sandor 1999 ). Which is the 'real' TS? What boundaries constitute a useful phenotype? Both social factors and scientific issues are involved (Kushner 1999 ).
For the practicing clinician, it is important to know that evidence is accumulating, from this study and from others, that pure TS is not usually associated with problem behaviors. Their presence should alert the clinician to the likelihood of associated disorders, which may require separate consideration and intervention as part of a comprehensive treatment plan.
Furthermore, this same body of data (the TIC database), along with a substantial body of other published work, does not support the notion of "episodic attacks of rage, outbursts of temper, emotional storms, inappropriate sexual aggressiveness, and self-injurious behaviors" as part of the condition for most people with Tourette's.
Because behavioral problems are associated with comorbidity, their presence should dictate a high index of suspicion of the latter, whose treatment may be at least as important as tic reduction. The established database can be used as the entry point for further research when large samples are studied and generalizability of results is important.
The study has confirmed previous findings (at a single site) that anger control problems are associated with comorbidity (Budman et al. 1998 ) and that there is a very high rate of comorbid ADHD in clinical cases.
As shown in Table VII, anger control problems and SIB were four times more frequent in the comorbid TS group than in the TS only group. Almost twice as many individuals in the comorbid group had severe tics, and coprolalia (involuntary use of obscene or unacceptable words) was three times more likely.
Figure 3 shows the almost linear positive relationship between comorbidity score and anger control and sleep problems. Figure 4 shows the same for coprolalia and SIB.
Anger control problems, sleep problems, and SIB occur at levels that are probably comparable to the general population in persons who present solely with the symptoms of TS (this pattern holds for both males and females).
The TIC data (even allowing for clinical overrepresentation of comorbid features) suggests that many of the issues raised in the Jankovic article are not present in the majority of persons with TS, even when comorbid with other conditions.
Some of the data presented in the Table VII of the Freeman journal report is:
| TS Only | All Comorbid Individuals | |
| Anger Control Problems | 10% | 40% |
| Sleep Problems | 14% | 27% |
| Coprolalia | 6% | 18% |
| Self-Injurious Behaviors | 4% | 16% |
| Social Skills Problems | 3% | 22% |
| Sexually inappropriate behaviors | 1% | 6% |
These low numbers are associated with clinically-referred populations: they cannot be generalized to non-referred
populations. Even if they could, they do not substantiate claims of these issues in TS populations to the extent that
would warrant their inclusion as features of the diagnosis. Furthermore,
association of these problems with Tourette's is not
consistent with the criteria formulated by the Study Group which Jankovic references
and includes in his
article on page 1185, column 2, paragraph 1.
The TIC data -- the very data
Jankovic uses to defend his position -- does not support his claim of behavioral
disorders, even in the majority of comorbid individuals.
OTHER MISLEADING STATEMENTS IN THE ARTICLE
In conclusion, even if we were to incorrectly and strictly interpret
the Freeman TIC data to mean that only 12% of persons with Tourette's have tics only, that still begs the question:
whatever the percentage is, a diagnosis of Tourette's is
still based on tics, not on ADHD, OCD or behavioral disturbances. The
lasting impression that Jankovic's diagram will leave on family physicians is that Tourette's only occurs when ADHD, OCD and behavioral disturbances
are also present.
There are some other troubling statements in the Jankovic article.
Coprolalia, perhaps the most recognizable and certainly one of the most distressing symptoms of Tourette's syndrome, is actually present in less than half of patients with Tourette's syndrome.
Most experts put the number at about 10-20%, including the data from Freeman which Jankovic referenced.
What is the purpose in overstating the prevalence of coprolalia?
Coprolalia not typical of TS
Neal Swerdlow, M.D., Ph.D., responded to a marked rise in media stories spreading the misperception that outbursts of foul language are common manifestations of the neurological disorder. "The fact is that 85 to 90 percent of people with TS do not generally voice obscenities," he states. Especially hurtful to all people with TS is the frequent media labeling of the disorder as 'the cursing disease'."
Dr. Jankovic misses valuable opportunities to spread accurate, optimistic information about Tourette's. He cites the Yale/Leckman study on tic severity and states that "by 18 years of age, half of patients with Tourette's syndrome are free of tics." But he misses the opportunity to mention the full extent of the positive news from that study: regardless of initial or highest-ever tic severity, only 11% of the birth cohort had moderate to marked levels of tic severity at the end of the second decade of life, and tic symptoms for a majority were minimal or absent by the age of eighteen.
Dr. Jankovic's statements referring to the majority of persons with TS fail to point out something that family physicians need to know: the extent of the issue of ascertainment bias in referred populations of persons with Tourette's. Statements like "a majority of patients with Tourette's syndrome have symptoms of ... " followed by various diagnoses, are less than correct: they should state that a majority of patients in clinically-referred populations have these diagnoses, which may not be representative of all persons with Tourette's. Good epidemiological data is just not available. Family physicians will see patients whose symptoms may not warrant referral to a tertiary specialty clinic, and should be aware that Tourette's does not always present with behavioral disturbances.
"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
The statement that TS is associated with the "inability to control anger, outbursts of temper, episodic attacks of rage, and emotional
storms" fails to acknowledge the Bruun/Budman research, parts of the Freeman work, or press releases put out by the TSA which clearly show that "rage" is not part and parcel of TS, and not an issue for the vast majority of persons with TS.
When so many have worked so hard to dispel the notion of "rage" being attached to Tourette's, it is unfortunate and discouraging to find a member of a TSA Advisory Board publishing information which attaches "rage" to the Tourette's diagnosis.
Explosive Anger not Typical of Tourette syndrome:
A BW HealthWire press release from from BAYSIDE, N.Y. of Nov. 25, 1998 titled "Explosive Anger Not Typical of Tourette Syndrome, New Research Reveals," stated that the "connection between TS and explosive anger" is misunderstood. Based on a pilot study of rage attacks, according to Ruth Bruun, M.D., the article stated that, "research so far has demonstrated that ... rages are not specifically associated with TS. However, there are children who have TS along with other disorders such as obsessive - compulsive disorder, attention - deficit/hyperactivity disorder, depression, etc. It appears that behavior problems such as those frequently depicted in the media are more likely to be associated with having one or more of these other disorders than with TS alone." In that article, Cathy Budman, M.D. was quoted as saying, "If you see a range of symptoms in a child with TS, you must look for other causes (i.e. comorbidities)."
Jankovic's response to Dr. DeVeaugh-Geiss with respect to DSM-IV-TR (which he never mentions)
is confusing. He leaves the impression that the Tourette Syndrome Classification Study Group's work agrees with his notion of Tourette's lying at the intersection of tics, ADHD, OCD and behavioral disturbances.
Definitions and Classification of Tic Disorders
http://www.tsa-usa.org/Medical/definitions.html
http://www.tsa-usa.org/Medical/definitionstable.html
The Study Group decided to keep the basic tenets of the DSM-IIIR definition for Tourette syndrome, chronic tic disorder, transient tic disorder, and non-specified tic disorder, since these terms have become widely used, and there is no solid reason to abandon them. However, for research purposes we divided each of these DSM-IIIR definitions into two categories: "definite," in which the tics have been witnessed by a reliable observer, and "historical," in which the putative tics have not been reliably witnessed. These definitions are reported here in greater detail, along with the definitions of other categories of tic syndromes that were developed at the workshop and are not covered by existing DSM-IIIR criteria. These additional tic categories are chronic single tic disorder; definite tic disorder - diagnosis deferred; and probable Tourette syndrome.
The diagnostic categories depend on the presence of tics alone, although patients with Tourette syndrome may have behavioral problems or mental disorders that may overshadow the tic disorder in severity, impairment, or impact on normal functioning.
His statement that "the criteria do not take into account the frequently associated behavioral disorders that may be more distressing than the tics" appears to suggest that either the Study Group classifications or the most recent DSM revisions account for comorbid issues in the diagnostic definition of Tourette's. They do not.
It is understandable that he appears to want to correctly call attention to the fact that, when comorbid conditions are present, they often cause more difficulty than tics. However, in doing so, he has redefined Tourette's Syndrome in a manner not consistent with the TSA Study Group, the DSM, or the symptoms of a large number of persons with tics.
Inaccurate
statements about Tourette's lead to the underdiagnosis of the "milder cases of Tourette's syndrome (which) would be difficult to classify."
DSM-IV-TR has dropped the "significant impairment" language (Jankovic
never mentions this) and refers only to tics, leading in the direction of increased recognition that TS exists in forms that encompass only tics.
DSM-IV-TR Tourette's Disorder
http://www.behavenet.com/capsules/disorders/touretteTR.htm
AMERICAN ACADEMY OF FAMILY PHYSICIANS
This misleading information about
Tourette's has been picked up and published even more widely: to the front lines of family physicians who are in the best position to recognize Tourette's in their practices.
American Family Physician
March 15, 2002
Recognition and Treatment of Tourette's Syndrome.
Author/s: Bill Zepf
http://articles.findarticles.com/p/articles/mi_m3225/is_6_65/ai_84072795
Zepf states that "Jankovic ... addresses the recognition of this disorder in clinical practice."
How are family physicians to recognize Tourette's syndrome if it is assumed to be present only when tics, ADHD, OCD and various behavioral disturbances all must be present?
Jankovic's article does just the opposite: it increases the likelihood that many children with TS will continue to go
undiagnosed. That inaccurate Venn diagram will be a visual image hard to remove from
physician's minds.
This article may increase the stigma already unnecessarily attached to
Tourette's syndrome, and make physicians less likely to confer the diagnosis.
JANKOVIC ON THE 2/3 RULE
Dr. Jankovic was reported by an attendee of the Houston TS Conference several years ago to have put up a slide which
listed the infamous "2/3 rule" as a "TS comorbid."
The 2/3 rule:
https://tourettenowwhat.tripod.com/about_ts.htm
The "2/3 rule," put forth by the book, Teaching the Tiger, and at TS conferences, is an example of the kinds of issues that affect much of the TS 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 TS at all. But this "rule,"
implying a global level of developmental delay across all realms of
functioning in a well-known book ostensibly about TS, has been put forward at TSA conferences and has become generalized to ALL children with
TS. This is not backed up by any research! The statement should be referenced, qualified and used more carefully. Authors fail to consider ALL children with TS when they make these types of statements in print or at conferences.
Kudos to Dr. Joseph DeVeaugh-Geiss, for speaking
up with correct information about Tourette's syndrome. Unfortunately, not
everyone who sees the original article will see his correction.
Jankovic Contact Data and Bio
http://www.bcm.edu/neurol/jankovic/biography_jankovic.htm
Other
"Hall of Fame/Hall of Shame" items here
as well as a feedback forum for your comments, and a sample letter to
register your dissatisfaction with the publication of this misleading definition
of Tourette's.
(Just a note: this website was
designed for newcomers to Tourette's syndrome, to be read through in page order.
Growing up with Tourette's
Syndrome: Information for Kids
HBO Documentary on Tourette's Syndrome Tourette
Syndrome Research Article Summary
Tourette's Syndrome - Now What?
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