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Biology 202
2006 Second Web Paper
On Serendip
It all began with a deviated septum...or so we thought. Two years ago, my brother Steven began making strange honking noises in the back of his nose/throat. The sound was a minor annoyance at first, but it became a persistent one over the course of several days. After seeing a doctor and determining that his septum was not deviated, my mother began to consider other possibilities. To her, the noise he made seemed like a tic. No matter how many times we asked him to stop, he could not. When our family physician disagreed and the honking sound continued, she took him to see a specialist at A.I. DuPont Children's Hospital. After examining my brother and discussing everything from his diet to his behavior patterns, the doctor suggested the possibility of Tourette's Syndrome, if the condition continued for the next year.
Tourette's Syndrome is named for the 19th century French doctor who discovered it, Georges Gilles de la Tourette. It is a neurological disorder comprised of tics: involuntary, purposeless, repetitive movements or sounds. Like schizophrenia, it is believed to be the result of hypersensitive dopamine, norepinephrine, and serotonin receptors. (For more information on the function of these neurotransmitters, see http://serendipstudio.org/bb/neuro/neuro06/web1/cmulligan.html). There are both motor and vocal tics. These include but are not limited to blinking, nose twitching, shoulder shrugging, grimacing, kicking, stamping, grunting, shouting, sniffing, barking, and corpropraxia, the making of obscene gestures. Complex vocal tics also often include corprolalia, the involuntary repetition of obscene words, echolalia, the repetition of other's words, or palilalia, the repeating of one's own words (1).
Steven's symptoms, however, were all nonverbal. Although the honking noise itself eventually stopped, as time went on, we would notice him constantly shifting his weight, spastically moving his legs and arms, and repeatedly adjusting various articles of clothing. He began to seem comfortable in his own skin. These difficulties, unfortunately, were only the beginning.
Tourette's is also associated with a number of other disorders, such as ADD/ADHD, oppositional defiant disorder, learning disabilities, and OCD. In Steven's case, obsessive-compulsive behaviors began to occur. There are two components to OCD: uncontrolled, recurrent thoughts (obsessions) and uncontrolled recurrent behaviors (compulsions). Typical obsessions include fear of germs, excessive doubting (eg: worry one has left the stove on even though he just checked to see it was turned off), need for specific order or symmetry, need for perfection, visions of violence, visions of sexual imagery, or visions of religious imagery. Typical compulsions include washing, excessive checking and re-checking, saving or hoarding, counting, arranging or ordering objects, hair pulling, nail-biting, excessive seeking of reassurance, vocal repetition, compulsive praying, and repeatedly performing an action until it is "perfect"(2)..
Steven repeatedly described the need for perfection. While doing his homework, he would erase his name or his answers and write them over and over again because they had to be "perfect". He would arrange objects on his desk in a specific order. While eating, he would set down his glass or his utensils several times until they were "right". If he did not perform these ritual behaviors, he felt uneasy or "wrong".
So how does one deal with Tourette's Syndrome? Tourette's is traditionally treated with neuroleptics like haloperidol and pimozide, which inhibit the various neurotransmitters mentioned above. Side effects include excessive weight gain, dysphoria, Parkinsonian symptoms, memory problems, intellectual dulling, and personality changes (3). Imagine this rage of side effects in conjunction with the hormones that are already raging in an adolescent boy. Consider the social stigma attached to not being able to perform well in school or to drastic changes in appearance and personality. My mother and Steven's doctors thought long and hard about these issues. Rather than deal with these myriad of side effects, they opted to treat him with clonidine (cataprese), which is also used in the treatment of hypertension. It is administered via a transdermal patch. Unlike the other neuroleptics, cataprese's primary side effect is sedation. Steven says it produces a calming effect. The tension he feels prior to a tic is alleviated. There is less of a compulsion to perform certain behaviors. As a result of this medication, his bouts of tics are fewer and far between. He is able to function in a normal classroom environment, interact with his peers, and most importantly for him, continue to play baseball.
WWW Sources
1)What Is TS?, a rich resource on Tourette's Syndrome
2)OCD, a rich resource on obsessive-compulsive disorder
3)Guide to the Diagnosis and Treatment of Tourette's Syndrome, a rich resource
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