Tourette’s Syndrome: Difference or Disease?
HISTORY OF TOURETTE’S SYNDROME
Tourette’s syndrome has been around for far longer than its original documentation in 1825. Although it was then that Jean Marc Gaspard Itard wrote the first case study of what would later be called Tourette’s, (1) evidence of people who twitched and shouted uncontrollably has been found in all cultures, socioeconomic statuses and age groups in every era in which it has been looked for. (2) Itard wrote about the Marquise de Dampierre, a noblewoman who had frequent muscle spasms, repetitive actions and coprolalia—sudden outbursts of inappropriate and often incredibly insulting language.
In 1885, Georges Albert Édouard Brutus Gilles de la Tourette’s, a French neurologist, wrote the first comprehensive paper about a “maladie des tics,” (1) In his paper, Gilles de la Tourette’s discussed nine cases who were afflicted with various motor and vocal tics. His original intent was to study patients at the Pitié-Salpêtrière Hospital, as a student of Jean-Martin Charcot, and test Charcot’s new hypothesis about the existence of a disease distinct from both hysteria, characterized by extreme emotional outbursts, and chorea, patients with which exhibit quick, involuntary dance-like motions that have no inherent rhythm, but which seem to flow from one to another. He soon found several patients that did not seem to fall under either category and had soon described this new rare condition. (1) His mentor, Jean-Martin Charcot, renamed it to Tourette’s syndrome in Georges’ honor shortly after the paper was published. (1)
Today, Tourette’s syndrome is not considered as rare as it once was, although exact numbers of patients vary depending on which source is being cited and what their criteria are. The Tourette’s Syndrome Association states that the generally accepted number of affected individuals in the United States is between 100,000 and 200,000. (3) Most of these people are likely undiagnosed as most cases are mild and because many medical professionals are not aware of the nuances of the syndrome—many still think that copralalia (outbursts of culturally unacceptable language) is necessary for a diagnosis to be valid, and many patients are not properly diagnosed until they go to their doctor armed with their own information about Tourette’s. (2)
CHARACTERIZATION OF TOURETTE’S SYNDROME
The DSM IV diagnoses people with Tourette’s only if they have
both multiple motor tics and one or more vocal tics, [which] must be present at the same time, although not necessarily concurrently; the tics must occur many times a day (usually in bouts) nearly every day or intermittently over more than 1 year, during which time there must not have been a tic-free period of more than 3 consecutive months; the age at onset must be less than 18 years; [and] the disturbance must not be due to the direct physiological effects of a substance (e.g. stimulants) or a general medical condition (e.g. Huntington's disease or postviral encephalitis). (4)
Tics can include everything from blinking or grimacing to more complex motions like jumping and kicking to copropraxia (inappropriate gestures, i.e. raising the middle finger involuntarily). Vocal tics range from coughing or clearing one’s throat to actual words and phrases to coprolalia. It is important to note that both copropraxia and coprolalia occur in only 10% of all people with Tourette’s, even though the media tend to focus on this symptom because of its shock value. (3) Tic may wax and wane depending on the individual’s stress level and life circumstances, and may change over time. Often, people start ticcing with a simple facial movement, which may become a vocal tic or a more complex movement. Tourette’s is not a degenerative disease, however, and most people’s tics are so mild that they are left either undiagnosed, or untreated because many people find the side effects of the medications given to alleviate symptoms worse than the symptoms themselves. (3)
The diagnosis of individuals with Tourette’s syndrome is not always easy to accomplish, since many other similar disorders must be ruled out (i.e. chorea, drug induced imbalances, immunological reactions to some bacterial infections and even asthma can all cause Tourette’s-like symptoms). Additionally, there are several conditions that often arise in conjunction with Tourette’s (including Attention Deficit (Hyperactive) Disorder and Obsessive Compulsive Disorder) that can delay a proper identification.
People who have Tourette’s have normal intelligence and lifespan. There are some learning differences associated with the syndrome, and sometimes tics can interfere with reading and writing (if you have to move your head or hand in a quick, jerky fashion it’s difficult to read or write in a fluent manner), (5) (6) but none of this is required of the syndrome and there are Tourettic people in all walks of life.
CAUSES OF TOURETTE’S SYNDROME
The cause of Tourette’s syndrome is unknown. It seems to have a strong genetic aspect, following an autosomal dominant pattern where parents with Tourette’s are 50% likely to pass on the predisposition onto a child. (4) Having this predisposition to tourettic behavior does not imply that these traits will manifest into the disorder itself and symptoms tend to vary from generation to generation. For unknown reasons, males are about twice as likely to show tourettic traits as females. (4)
Although it is generally accepted that Tourette’s is based in biology (i.e. the brain tissue itself is malfunctioning in some manner) how people tend to deal with having the disease seems to dictate more about their quality of life than the severity of their tics. Oliver Sacks has written about a couple of severely Tourettic people who have succeeded very well in their lives including a highly skilled surgeon/pilot who is married, has two children and a rewarding career. (7)
Typically, the disorder is more difficult to deal with and understand for children than for adults. This could be true for any number of reasons: 1) tics are typically more severe for children, especially during early adolescence, because stress can exacerbate the severity of tics; 2) children usually do not have the same coping mechanisms available to them for dealing with the unique stressor that having this syndrome is; 3) because of the visual nature of the condition, school-age children tend to get teased often, making it difficult for them to “fit in.”
There is much more to it that just this simple description, however. The HBO family documentary, “I Have Tourette’s, but Tourette’s Doesn’t Have Me,” interviews several children with Tourette’s and lets them explain their world to a wider audience. (6) Throughout the show their various tics are shown simply and without judgment—they are seen to be teaching us about their lives. One child describes his various and complex tics like a cough—you can hold it in, but not for any real length of time. One boy is affected by a tic involving shaking his head. While he’s talking to the camera, it seems like he is consistently trying to say “No,” but does not seem any more pertinent or debilitating than that, however, he complains of having trouble reading because he has a hard time focusing on a book while moving his head from side to side. Another child shows his current tic to be a high jumping motion, about it he comments “I might do that 100 or 200 times a day—it’s exhausting.”
It is encouraging that when asked, many people would choose not to take a pill, if one existed, to rid them of their Tourette’s. They feel it is a part of who they are—their quirks, and what makes them themselves—and that they would lose much more than just the disorder. While their behavior sometimes wildly differs from that of “normal” people, there is nothing inherently different about people with Tourette’s syndrome. This disorder highlights one of those grey areas that Sophie warns us about—where new research and information might have a tendency to shrink “normal” to include so little that conformity is the only option. (8) Who is to say that this “disorder” is actually anything so extraordinarily different than “normal?” While most people who are diagnosed tend to find it a relief that their actions have a name, and that other people are going through the same issues, it is unnecessary and unhelpful that they be regarded as less than “whole.” Obviously, something interesting is going on in these people’s brains that does not go on in most people’s brains, and that difference can and should be used to learn more about all people’s brains. Current research seems to be focusing on two main questions about Tourette’s: 1) what are the genetic components that lead to a predisposition to Tourette’s and 2) are Tourettic people either impaired in some way by their syndrome or are they enhanced in their abilities. The fact that the second is on the table is exciting, and seems to me that society is still open to looking at this collection of behaviors as merely a difference.
(1) "Tourette's Syndrome." Wikipedia. 2 Nov. 2008. Wikipedia Foundation, Inc. 3 Nov. 2008 <http://en.wikipedia.org/wiki/tourette_syndrome>.
(2) Robertson, Mary M., and Simon Baron-Cohen. Tourette's Syndrome. New York: Oxford UP, 1998.
(3) "Tourette Syndrome." Tourette Syndrome Association. 3 Nov. 2008 <http://tsa-usa.org/>.
(4) "Tourette's Disorder." Tourette's Disorder Information. 18 Dec. 2004. 3 Nov. 2008 <http://www.tourettes-disorder.com/dsm.html#dsm>.
(5) Handler, Lowell. Twitch and Shout : A Touretter's Tale. New York: University of Minnesota P, 2004.
(6) I Have Tourette's, but Tourette's Doesn't Have Me. Dir. Ellen G. Kent. Prod. HBO and the Tourette Syndrome Association. DVD. 2005.
(7) Sacks, Oliver. An Anthropologist on Mars : Seven Paradoxical Tales. New York: Vintage, 1996.
(8) Mental Health and the Brain forum area, on the Serendip web site, http://serendip.brynmawr.edu/exchange/courses/bio245/f08/genesdevel, accessed 3 November 2008.