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Medical Mystery Solved

By CORY FRANKLIN M.D.
   One of the earliest books published in Europe during the late 15th Century contained the story of a priest believed to be possessed by the devil. The priest’s “possession” manifested itself by frequent unusual movements and spontaneous obscene vocalizations, usually occurring without warning.
   Several hundred years later, neurologists who studied the case theorized this was the first reported patient with Tourette’s syndrome. In retrospect, the great 18th Century English essayist, Samuel Johnson, considered by many to be the second greatest writer in the English language second only to Shakespeare, was described by biographer James Boswell as suffering from symptoms suggestive of Tourette’s.
   France led the world in medical breakthroughs for much of the early 19th Century and French physician Jean-Marc Gaspard Itard published a description of several patients with prominent motor and vocal tics. One of Itard’s patients, a 26-year old woman, was a prominent member of the French aristocracy. Despite her manners and intellect, she frequently suffered uncontrollable movements associated with terrible outbursts of vocal obscenities. Her behavior was in marked contrast to her elegant background and otherwise refined character.
By chance, nearly a half-century later, the same aristocratic lady, now an elderly woman, happened to be at a social event attended by another prominent French physician, Jean Marie Charcot. Charcot is often considered the greatest neurologist of the 19th Century. During the gathering, Charcot was witness to one of her outbursts (giving her the distinction of being seen almost fifty years apart by two of France’s greatest physicians.) Although he did not formally examine the lady, his training as a neurologist led him to make a mental note about her unusual condition.
   Soon after that, Charcot assigned one of the top students at the hospital where he taught to classify unusual movement disorders. He made sure to tell the student to include patients with symptoms similar to those of the aristocratic woman he had seen at the party. That student was Gilles de la Tourette and within two years Tourette had produced several papers about uncontrollable movement disorders.
    One of those papers, published in 1885, turned out to be a landmark in neurology. It described a syndrome of involuntary movements (motor tics) and uncontrollable utterances (vocal tics) which were often obscene or repetititions of other peoples’ words.
   Professor Charcot was so impressed with his student’s work that he named the disease Gilles de la Tourette’s syndrome, or as we know it today, Tourette Syndrome. For the next eighty years, the cause of the disease was hotly debated. Some believed it to be psychogenic, perhaps the result of oppressed sexual conflicts, oppressive mothers or hysteria. Not until the1960’s when a new class of drugs, neuroleptic agents such as Haldol, showed the ability to reduce symptoms of Tourette’s, did researchers consider the neurobiology of the condition. Even then, it was considered a rare disorder. As recently as 1972, the National Institutes of Health refused a grant to the Tourette Syndrome Association for research on the disease because reviewers believed there were probably no more than 100 cases in the United States.

Modern Research

   Today, Tourette’s Disease is characterized by motor and vocal tics that come and go over time and vary in frequency in different patients. While much is still unknown about Tourette’s, researchers in the past several decades have begun to identify parts of the brain involved producing symptoms as well as studying the genetic and environmental basis of the disease.
   In contrast to the beliefs of the NIH grant reviewers in the 1970’s, Tourette’s is recognized today as a fairly common disorder with biologic, genetic and possibly environmental components. Estimates vary widely from 1 in 1000 children to 1 in 100 children and young adults in the U.S. might meet the diagnostic criteria.

Diagnosis

    There are many common misconceptions about Tourette’s. “One frequent misconception is that Tourette’s is a “swearing disorder.” Actually swearing is a relatively uncommon symptom; it happens in no more than 10-15% of the patients. Another misconception is that people with Tourette’s are intellectually disabled. That is generally not the case.”
   “By definition, a patient has Tourette Syndrome if they have at least two motor tics and vocal tics present on a regular basis for at least a year,” according to Dr. Douglas Woods, head of one of the top research centers for Tourette’s, the University of Wisconsin-Milwaukee Tic Disorders and Trichotillomania Specialty Clinic (trichotillomania is the medical term for chronic hair-pulling resulting in hair loss).
      
Non-Drug Behavioral Therapy

   A large study of patients from the Milwaukee center and several other national university centers was the subject of a recent article in the Journal of the American Medical Association. Woods says, “The study showed that a non-drug behavioral strategy for tics called Comprehensive Behavioral Intervention for Tics (CBIT therapy) was more effective in reducing symptoms in patients compared to control patients who weren’t treated. In addition, symptom control for Tourette’s with CBIT was comparable to that achieved with some drugs, with far fewer attendant side effects.”
   The Milwaukee center headed by Woods specializes in the CBIT therapy. “Essentially we attempt to restructure the patient’s behavior and environment. With this approach, patients learn to manage their tics so the tics won’t happen as frequently or are less intense. For example, if a child is in an anxiety-engendering situation that precipitates a tic, they are taught strategies to minimize the anxiety: relax, and cope with the situation. Likewise, if the child feels a tic is about to happen, he or she is taught to do an exercise to “ride out” that feeling,” says Woods.
   Above all, Tourette’s is a manageable disorder. 
 
Disclosure: The author has a close relative working on the center’s research team.




Published: October 10, 2010
Issue: November 2010 Arts and Politics Issue