Nov 23 2012
I can think of few conditions with clinical features more ideal for establishing a pattern of abuse at the hands of practitioners of so-called alternative medicine than Tourette syndrome. Tourette syndrome (TS), which first manifests itself in early childhood in the overwhelming majority of patients, is a neurological disorder with infamous motor and vocal manifestations and a troubled past. Historically the condition was blamed on everything from emotional disturbances to outright faking to demon possession. But over the past few decades it has increasingly been recognized as a primarily organic disorder caused by negative genetic and environmental influences on areas in the brain which control movement and behavior.
I have a particular interest in Tourette syndrome, not just because I am a pediatrician but because I was diagnosed with the disorder at the age of seven. I have been lucky in that my symptoms, after a few rocky years in middle school and early high school, have been fairly mild. It is obvious to most people that I have a movement disorder, but it has never impacted my ability to function in society and succeed in my chosen profession of pediatric medicine. In fact, I often think of my Tourette’s as a positive aspect of my life, believing that it helped shape who I am as a person. I feel that it has helped instill in me positive personality attributes that are beneficial in the practice of medicine, such as empathy and compassion.
Patients that would have been institutionalized a hundred years ago, or worse as you go farther back in time, are now treated based on scientific advances in neuroscience and pharmacology, typically very successfully — that is if they manage to avoid involvement with quacks and charlatans. A 2009 survey of TS patients, or parents of patients with TS, revealed that nearly two thirds partake in alternative therapies with no proven benefit.
Until fairly recently, TS was considered by experts to be inherited in an autosomal dominant pattern. That is, there would be a 50% chance that the child of an individual with TS would inherit the gene or genes involved. The present and more nuanced consensus is that the condition is significantly more complex, with variable expression and incomplete penetrance. In other words, far less than 50% of children at risk of inheriting the condition show any symptoms at all, and the symptom severity in those that do does not correlate well with symptom severity of the parent. A father with very mild TS may have a child with symptoms severe enough to impact function and quality of life dramatically, or one that is similarly affected. While candidates do exist (SLITRK1 gene on chromosome 13q31.1), the genetic basis has not been hammered out by researchers in any clinically meaningful way.
The Tourette syndrome tic
The most recognizable feature of TS is the tic. These are sudden, short-lived and seemingly random movements or vocalizations which exist in the grey area between voluntary and involuntary. By that I mean that although they are generally considered involuntary, and they certainly can take patients by surprise, tics can typically be suppressed voluntarily and patients can learn to use purposeful and less socially awkward movements to dissipate the premonitory urge universally described by patients with the condition. Think of it as a sort of internal itch, maddening at times, that only certain movements or vocalizations can scratch. It is a near constant source of frustration and annoyance in many patients with TS.
Tics can be simple movements, such as eye blinking, grimacing, or jerking of the head and neck, or surprisingly complex behaviors, even ritualistic. They can be violent or subtle, easy to conceal or extremely disruptive. Vocalizations follow a similar pattern of simple and more complex noises. Grunting, throat clearing, and sniffing are extremely common but the most widely known form, thanks to a number of misleading characterizations of TS in movies and on television for comic effect, is that of coprolalia. This symptom of TS, which occurs in ten to forty percent of cases, involves the involuntary utterance of obscene words. Of note copropraxia, when complex motor tics take the form of obscene gestures, is also not uncommon.
Motor tics and vocalizations (which are really just motor tics as well if you think about it) are unfortunately not the only manifestations of TS. ADHD and OCD are quite common comorbid conditions, along with anxiety and mood disorders, migraines, sleep disorders, and even cervical artery dissection from forceful jerking of the neck. Botulinum toxin (Botox) is actually a science based treatment successfully used in a number of TS patients with severe motor tics. It can even help with vocal tics when lower decibel vocalizations are desired. How’s that for an all-natural remedy? In some patients, the comorbid conditions are considered worse than the tics, which can help guide treatment. A patient with severe ADHD, for instance, may benefit from stimulant medication, which may indirectly improve tic severity.
Once felt to be quite rare, we now know that TS is a common cause of tics, although most children with tics don’t have it. Transient tic disorders that last less than one year and do not meet diagnostic criteria for TS are far more common. TS is believed to occur in about 8 children out of every 1000, and is considerably more common in boys. Diagnosis typically occurs around age 7 years but tics can appear up to age 18 (some say 21). A small percentage of those diagnosed actually require any form of treatment, although some are debilitated by the tics and comorbidities.
Alternative medicine and Tourette syndrome
Many patients with TS, even milder forms, seek guidance, reassurance and treatment from medical professionals. Unfortunately, as noted above, some seek out care from practitioners of what I like to call irregular medicine, but is more commonly known as complementary and alternative (CAM) or integrative (IM) medicine. Why they do this is actually easy to understand considering how pervasive CAM has become, even infiltrating our top-tier academic medical centers and boasting well-funded government agencies like the National Center for Complementary and Alternative Medicine (NCCAM). The media credulously reports findings published in questionable journals or simply regurgitates press releases. And skeptics are often portrayed as foolish, or worse, closed-minded.
Alternative medicine, however, is championed by well-known celebrity practitioners and supporters able to reach millions with a seemingly ubiquitous presence on television and in print media. Silver-tongued gurus and their snake-oil spouting minions take full advantage of the large percentage of “shruggies” in the practice of medicine. Reality takes a backseat to belief and patients suffer the consequences. But despite the success they have had without it, proponents of alternative medicine still crave the legitimacy of science and publication in peer reviewed journals.
The most common incursion of alternative medicine into published journals, most of which have impact factors only appreciable with the aid of a high-powered magnification device, is in the form of the case study. A rigorously documented case study can be a valuable thing, even if its place in the hierarchy of evidence is above only the undocumented anecdote or testimonial. They can be particularly useful in determining that an all-inclusive assumption is in error, the classic example being that the existence of even one black swan disproves the statement that all swans are white. Case reports can also be helpful, especially in series, in guiding future research. And in instances of rare conditions, case reports may be all that is available to guide therapy. But a case report, regardless of how well it is documented or of how good a journal it is published in, is incapable of answering the question of cause and effect. They can lead a researcher to come up with a dandy hypothesis or, as occurs with much higher frequency, to waste time and money on a wild goose chase. A physician simply can’t say that a treatment works with anything resembling confidence based on case reports. Prior plausibility as well as the totality of the medical literature must be taken into account.
I have never needed treatment of any kind for my Tourette’s, but I have tried to keep up with the literature on various therapies, pharmaceutical or otherwise, for the condition. I usually stick to reputable sources but every now and then I like to go “slumming” in the world of chiropractic research. During my last trip across the border between science and pseudoscience, I discovered a gem which perfectly illustrates the trouble with case reports and with chiropractic (well, alternative medicine in general). I stumbled on a press release for a case report published in late 2009 in the Journal of Pediatric, Maternal & Family Health — Chiropractic involving the year-long treatment of TS in a 20-year-old female. (Barbara Loe Fisher, founder of the rabidly antivaccine National Vaccine Information Center sits on the editorial board of this journal.) Not unexpectedly, the results were reported as positive.
A Google search using “chiropractic for Tourette’s” reveals over 300,000 results, with many (I stopped counting at 100) individual chiropractors referencing this particular case report when calling for patients with TS to come in for treatment. They were not equivocal on the subject either but then again practitioners of alternative medicine don’t tend to care much for nuance and subtlety. Warning bells should go off whenever a proposed abnormality, be it the chiropractic subluxation, stagnant chi, or liver fluke infection, causes every problem and has a quick and easy, though often pricey, solution. So chiropractors are claiming to be able to cure or significantly impact the symptoms of TS, and this is the proof. Or is it?
The answer is of course no, but I’ll explain.
Tourette syndrome: perfect for fooling patients and practitioners
Once again, TS is a condition perfectly suited for fooling patients and practitioners into thinking their intervention helped, primarily via a number of placebo effects. It waxes and wanes in severity, with many sufferers experiencing periods of time during which their tics aren’t as forceful or frequent. TS is generally at its peak in early adolescence, or about ten years after diagnosis for most patients. But, and I hope this clearly shows why using this case report as anything other than lining your child’s hamster cage overestimates its worth, two-thirds of TS patients experience remission or significant amelioration of their tics in late adolescence or early adulthood. The patient described in this case report was 20-years-old and the treatment took place over an entire year. It is very possible, if not highly likely, that the patient’s symptoms would have improved regardless of the therapy she sought out. Not taking into account the natural course of a condition tends to occur when you lack experience in diagnosing and caring for patients with that condition, and when your background in pediatric pathophysiology involves a weekend course held at the airport Howard Johnson. I’d say it’s a rookie mistake, but chiropractic was invented in 1895.
The tics, whether motor or vocal, simple or complex, experienced by TS patients are unique. They can be suppressed, for a time, only to come back with a vengeance eventually (car rides home after job interviews for instance). They are suggestible. They are affected by stress and fatigue. And, like pain, there is a considerable subjective component to each individual patient’s ability to cope with these movements and outbursts. One patient’s suffering with the condition may be much greater than another’s despite having the same tics at the same frequency in the same location.
The potential factors at play are too numerous to list but to give one example, my most obvious tic, where I forcefully jerk my left arm outward and vigorously flex and extend my arm several times until it “feels right” doesn’t bother me when I’m sitting in bed reading a good book, my bedside lamp safely out of reach and loving wife on my “good side”. But ask me how I feel when sitting in a crowded movie theater with a stranger on my left who I really don’t want to elbow in the face. Now what if I had a stressful job that required long shifts in close quarters? Life could be pretty miserable. Life might suddenly become much less miserable if I was moved to a different location that allowed for more breathing room and less chance of injuring a co-worker or losing an arm. I might report a definite improvement in my quality of life, decreased stress, and subsequent decrease in severity of tics simply because my boss took my condition into account.
We don’t know what else was going on in the life of the patient in this case report. We don’t know about changes in her life that might have decreased (or even increased) her anxiety. Perhaps simply receiving hands on treatment from a perceived authority figure adamantly claiming a high rate of success resulted in less stress and fewer tics. We don’t know if her improvement was simply a regression to the mean. All we know is that she reported an improvement after a year of chiropractic care. Correlation does not necessarily equal causation as the old saying goes.
Another aspect of placebo effects involves reporting bias. Patients who are emotionally invested in a therapy are more likely to report positive results. Patients who like their physician or other practitioner, such as a chiropractor for instance, are more likely to report positive results. Patients in a study, or who are going to be written about in a case report, are more likely to report positive results. This may stem from a Hawthorne effect or from simply not wanting to feel like they let somebody down who stands to gain from positive results. There are other well established psychological entities, such as cognitive dissonance theory, that motivate positive reporting. Nobody wants to feel foolish, for instance, wasting a year of their life and the money required to do it on a failed therapy. None of these effects can be accounted for in case reports such as this.
But is this case report even useful as a hypothesis generator? Does it support the authors’ calls for more research into the chiropractic subluxation as the etiology of TS and chiropractic spinal manipulation as treatment? Once again, the answer is no. While the exact cause of TS is not established, there is expert consensus that it has a genetic origin and involves connections between the cortex and subcortex leading to abnormal synaptic neurotransmission. Some experts have raised the possibility of an autoimmune etiology, particularly related to infection with Group A Strep, but this is highly controversial. There is absolutely no legitimate concern that impairment of spinal nerves, as is claimed to occur in the presence of chiropractic subluxation, is a factor. Sure there have been instances where experts have been wrong, and as a skeptic I always leave room for surprising discoveries, but the evidence required to overturn the current consensus would need to be extraordinary.
Testimonials, anecdotes and case reports are frequently used as evidence to support a variety of alternative therapies, not just chiropractic. Acupuncture, for example, has also achieved wide acceptance by the public and many in the medical field despite negative outcomes in large and well-designed prospective studies. Anecdotes and a number of logical fallacies such as arguments from antiquity and popularity, and successful appeals to magical thinking are more powerful than any placebo-controlled double blinded machination of “western” science, it seems. The conditions necessary to achieve a positive result, or at least positive reporting, in patients with self-limited and subjective complaints are all too easily set up. And negative case reports don’t tend to be accepted for publication. With a foot in the door, a host of even less plausible therapies, like homeopathy and energy healing, continue to slip through the ever widening crack.
It seems at times that we are fighting a losing battle. I’m not sure what it will take to wake up the sleeping giant — the legion of mistakenly disinterested shruggies whose silence plays such a large role in fostering the current CAM friendly environment. Perhaps economics will force their hand, perhaps patient suffering. I hope that won’t be the case. Perhaps reason will one day prevail.
There is cause for hope. The Tourette Syndrome Association, a highly respected organization which has done a great deal for patients with the condition, addresses the issue of unproven alternative therapies for TS in a policy statement. It is well reasoned and calls for proponents of unproven therapies to apply for research money and prove it. They clearly get it. For more in depth analysis of the use of CAM modalities in TS, check out the discussion by Dr. Katie Kompoliti, an Associate Professor of Neurological Sciences at Rush University Medical Center, provided by the TSA.
About the Author
Clay Jones, M.D. is a pediatric hospitalist practicing at Our Lady of the Lake Children’s Hospital in Baton Rouge, LA. He is the section chief of pediatric inpatient medicine, a title which sounds significantly more fancy than is deserved, and devotes his full time to educating pediatric residents and medical students. Dr. Jones first became aware of and interested in the incursion of pseudoscience into his chosen profession while completing his pediatric residency at Vanderbilt Children’s Hospital. He has since focused his efforts on teaching the application of critical thinking skills and scientific skepticism to the practice of pediatric medicine.
Dr. Jones has no conflicts of interest to disclose and no ties to the pharmaceutical industry.
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