Gilles de la Tourette Syndrome

A movement disorder characterized by motor and vocal tics

What's Tourette Syndrome?
Symptoms
Manifestations
Tics, Other Tics
Diagnosis
Neurochemistry
Obsessive Compulsive Behaviours (OCD)
Attention Deficit Hyperactivity Disorder (ADHD)
Treatment
Historical Personalities with TS
Resources

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What's Tourette Syndrome?

Tourette Syndrome (TS) is essentially a movement disorder. It is also a disorder of inhibition. It is classified as a neurological condition. E.M. Bouteille first separated movement disorders in 1810 in his medical literature on the choreas. Although Bouteille did not include symptoms of TS in his journals, in 1825, Dr. J.M.G. Itard described involuntary functions of movement of the locomotor apparatus of grasp and voice, or motor and vocal tics.

In 1884, George Gilles de la Tourette became the intern of the famous neurologist Jean M. Charot. Tourette became interested in movement disorders by reading medical reports of the "jumping Frenchmen of Maine", by George Beard in 1878 and 1880. The "jumping Frenchmen" were lumberjacks of French-Canadian decent living in the Moosehead Lake region of Maine. These men, when startled would jump excessively, and if given a command, would carry it out immediately while repeating the instruction over and over. (As there was little entertainment in a lumber camp, they would often be provoked deliberately by co-workers.)

Gilles de la Tourette made the deduction that if there were jumping Frenchmen in Maine, there ought to be jumping Frenchmen in France. He searched the wards of hospitals but failed to find them. He did however find six patients with tics and abnormal vocalizations. To these six, he added three patients of other doctors, and Tourette Syndrome was born.

Tourette found that the occurrence of tics was familial. He recognized echolalia and coprolalia. He also recognized that the onset of the symptoms was in childhood with a male prepondence. He found that the symptoms were progressive only to a certain point, and that different symptoms would replace each other. Most important, he found that the patients did not deteriorate psychologically or intellectually.

After Tourette died in 1902, very little was done on the subject of TS. Some patients were referred to hypnotists with no avail. Little was learned about TS until the 1960's when advances were made in the understanding of brain chemistry and have put tic disorders in a different perspective. Perhaps, with even more advances in medicine and public awareness, the young TS patients of today will be the last generation to yearn for the light at the end of the tunnel.

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Symptoms of TS

The genetic defect that causes TS has yet to be identified. TS remains in the category of a syndrome and diagnosis can only be made by an analysis of the symptoms. Blood tests, CAT scans, and EEG's can oly help to rule out other medical conditions that may cause similar symptoms.

The official defination of TS in the "Diagnostic and Statistical Manual of Mental Disorders lll" (DSM-lll, 1987, American Psychiatric Association, pg.80) is as follows:

1. Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently.
2. The tics occur many times a day (usually in bouts), nearly every day or intermittently throughout a period of more than one year.
3. The anatomic location, location, number, frequency, complexity, and severity of tics change over time.
4. Onset before the age of 21.

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The Manifestations of TS

Tics are broken down into several categories: voluntary and involuntary, simple and complex, and motor and vocal. Basically, a tic is an involuntary, sudden, rapid, recurrent, non-rhythmic stereotyped motor or vocal movement. It is irresistible, but can be suppressed for varying lengths of time. Eventually it has to be released. Compare it to breathing, an involuntary action. It can be suppressed by holding the breath, but eventually the need to breathe becomes so great, that is is impossible to hold back.

Simple Motor Tics consist of movements such as eye blinking, mouth twitching, eye turning or rolling, head jerking, shoulder shrugging, hand jerking, sudden tightening of stomach muscles, kicking movements and so on. Simple motor tics involve only one group of muscles and produce one basic movement.

Complex Motor Tics are usually a series of simple motor tics performed in a meaningless sequence, such as, touch chin to chest, or any other series of tics that are repeated in the same way each time. Another type of complex motor tic involves a coordinated series of movements that appear to have a purpose. These include squatting, hopping backwards or spinning around while walking or pulling at clothing. These tics appear intentional but they have no particular purpose for the person that performs them.

Copropraxia is another complex motor tic and includes making involuntary obscene gestures such as "giving the finger", and is fairly common. Other copropraxic gestures include the grabbing of one's genitals or reaching out to touch another person's breasts or genitals. These actions can cause considerable embarrassment and are not understood by those not familiar with TS.

Simple Vocal Tics are brief, at times staccato noises. They include grunting, throat clearing, barking and other animal noises, squeaking and so on. These noises are made by a tic movement of the vocal apparatus and serve no purpose or meaning to the person experiencing them.

Complex Vocal Tics are the most puzzling of this disorder. They include the repetition of words or phrases. Coprolalia, the use of obscene or socially unacceptable language, is an example of a complex vocal tic. Also included are ehcolalia -the repeating of words or phrases said by another, TV commercial jingles, or non-existent words. Palilalia is the repeating of words the person themselves had said.

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Tics

Moter Tics
Simple Complex
blinking eyes facial gestures
jerking necks eye rolling
shrugging shoulders smelling things
facial grimacing touching
flipping head jumping
kicking hitting
tensing muscles -
finger movements -
sticking tongue out -
-
Vocal Tics
Simple Complex
throat-clearing animal sounds
sniffing repeating words/phrases
coughing coprolalia
grunting pallilalia
spitting echolalia
yelling -
belching -

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Other Tics

Sensory Tics consist of a localized, uncomfortable sensation occurring in a repetitive fashion, and are relieved by the performance of a motor or vocal tic.

Dystonic Tics also include motor and vocal tics, but consist of slower, more sustained movements. They are repetitive as all tics in TS are.

Mental Play is another phenomenon in TS patients. Mental play is experienced as pleasurable and intentional. It includes visual, auditory, and cognitive word and number games. It includes making up new words by breaking up or changing existing ones and mental mathematics. Visual images can be changed or distorted by squinting the eyes or relaxing them.

Regardless of how perverse the symptoms may be, people with TS are more upset by them then their family or friends. Attempts to "hold back" tics are more or less successful but demand a lot of focus and are distressing and tiring. Typically, TS tics are suppressed while at work or school, and released at home in privacy. Some people disguise their tics so that head tossing can appear as it they were trying to shake their hair out of their eyes, or throat clearing is passed off as an allergy.

The tics of TS fluctuate in severity. New tics replace old ones. During times of stress such as holidays, exams, family problems, colds, or other physical illness, tics increase. Relaxation has a strange effect. It can instigate ticcing behaviour. Tics temporarily stop during periods of intense concentration.

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Diagnosis

TS was once thought to be extremely rare. It is being diagnosed far more frequently today, thereby showing that there have been many cases of the disorder that went overlooked or misdiagnosed. Presently there is about one person in 2,000 suffering with TS. In varying degrees, one in 1,000 people suffer from some type of tic disorder.

TS is known to affect people of all races and in all parts of the world. There is evidence that African-Americans is affected less than others. In a study by the Tourette Syndrome Association's Pennsylvania Chapter in 1993, out of 1504 registered TS cases, only 15 had African-American parentage.

During the last twenty years, the medical community has shown a refreshed interest in TS. Neurologists, psychiatrists, pediatricians, geneticists, and biochemists have seriously started to study this disorder. In spite of this extra attention, most doctors still do not have a clear understanding of TS even if they are presented with all the evidence of the disorder. Diagnosis is often made by lay-people that have seen a television program, or read an article on the subject. Often their medical practitioner will tell a parent that their child cannot have TS because they are "not swearing". If a prescription is given to control tics, the dosage or frequency is often incorrect thus causing behaviour problems that are misdiagnosed and treated as psychiatric conditions. When TS is suspected, it is very important to seek out the care of a physician that is competent in the area of tic disorders.

TS is most often treated by both a neurologist and psychiatrist. This is because until the twentieth century, a distinction between the two fields did not exist. Mental patients were treated by a neurologist or general practitioner, or often not at all. People judged insane were either hidden at home or placed in asylums. The word "bedlam" derived from "St Mary of Bethlehem", a famous lunatic asylum in London, describes what these places were like. "Lunatics" were chained or left to their own devices like animals in a zoo. Nobility visited asylums, as an observance of these people was both frightening and entertaining.

In the late 1800's, Charcot began to study the nature of hysteria. Tourette and Freud were his students. Tourette continued his study of neurology while Freud went on to develop the "talking cure", which eventually evolved into psychoanalysis. Soon a separate medical specialty was born. It was called psychiatry.

When a person is evaluated for TS many problems arise. It is common for them to unintentionally suppress all tics while at a doctor's office. This phenomen can be compared to the toothache that goes away as soon as you step into the dentist's office. Some patients can be observed ticcing in the waiting room when they are not aware that they could be seen or experiencing severe vocal tics while in the washrooms of such places.

TS cannot be diagnosed by any medical test. Diagnosis is made on taking a complete history of the patient, and delving into the nature of their tics and other symptoms. In children, who may also suffer from Attention Deficit Hyperactivity Disorder (ADHD), a common side effect of TS, psycho-educational testing may help identify academic strengths and weaknesses and can be a helpful guide to special educational benefits.

School and job problems need also to be evaluated. For school children it is advisable to have untimed tests or other special considerations. An employee with TS needs to explain his disorder to his supervisor if changes at work are required. Genetic counseling is available to couples to explain the statistical probability of passing the TS gene on to their children.

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Neurochemistry

The basic of the brains' billions of nerve cells are called neurons. When neurons communicate with each other, or transmit signals to other cells, we are able to function correctly. All our movements, sensations, out heart and breathing depend on the proper communication of our neurons.

A neuron has three parts: a body that contains the nucleus, branches called dendrites, and a large branch called an axon. Some axons are covered with myelin, a fatty substance that causes an equivalent to arcing as in an electrical circuit. In order to communicate, an electrical impulse is sent from the neuron cell through the axon to its terminal. When the electrical impulse reaches the axon terminal, it causes the release of chemicals called neurotransmitters. The neurotransmitters spill into the small space or synapse between the cell body and the axon terminal of a neighbour cell. This is the process of cell-to-cell communication. The whole process takes 1/10,000 of a second.

There are many different neurotransmitters in the brain, each having different effects. When the complexity of the mechanics are considered, it seems incredible that things so seldom go wrong. When they do, the result may be short malfunctions such as in epeleptic seizure, or chronic afflictions such as TS. Research into identifying the variety of neurotransmitters and how they work began in the 1930's. Of the 40 types of brain chemicals so far identified, five seem to play an important role in TS. They are: dopamine, norepinephrine, serotonin, acetylcholine, and the opoids.

Dopamine is a major player in the regulation of movement. When dopamine activity is low the result is muscle rigidity and lack of spontaneous movement as seen in Parkinson's Disease.

Norepinephrine is contained in the brain and in the adrenal glands. During stress or excitement, large amounts of this chemical are released enabling one to think and act quickly during an emergency situation, often without being fully aware of their response.

Serotonin is present in many parts of the body as well as in the brain. It plays part in mood, sleep, and eating disorders. It has been implicated in Obsessive-Compulsive Disorders (OCD), migraine headache, anxiety disorder, aggressive and self-abusive behaviour.

Acetylcholine is the first neurotransmitter to have been identified. It facilities many physical movement including tics.

Opoids are neurotransmitters that alleviate pain. They are released at times of great stress or severe injury. An abnormality in the release of opoids plays a part in self-abusive behaviour sometimes associated with TS. Endorphins are the most commonly known of the opoids.

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Obsessive and Compulsive Behaviours (OCD)

In the DSM-lll, obsessions are defined as: "persistent ideas, thoughts, impulses or images that are experienced, at least initially, as intrusive and senseless... The person attempts to ignore or suppress such thoughts or impulses so to neutralize them with some other thought or action. The person recognizes that the obsessions are the product of his or her own mind, and are not imposed from without..." (pg.245)

The same volume defines compulsions as: "a behaviour designed to neutralize or to prevent discomfort or some dreaded event or situation. However, either the activity is not connected in a realistic way with what it is designed to neutralize prevent, or it is clearly excessive. The act is performed with a sense of subjective completion...coupled with a desire to resist the compulsion...The person recognizes that his or her behaviour is excessive or unreasonable...and does not derive pleasure from carrying out the activity, although it provides a release of tension." (pg.245)

Obsessions and compulsions only become a problem when they are severe enough to disrupt one's life. In such cases, they are not only unwanted and disturbing, but also time consuming and interfere in a significant way with the process of daily living.

When attempts are made to resist compulsions, tension will mount till it is unbearable. Giving in to the compulsion relieves the tension, but the relief is only temporary. The cycle in on going, and persons afflicted with compulsions will try to conceal them from others. There are many odd behaviours of compulsion.

Checkers have a need to check on themselves to make sure that they have not made a mistake or forgot to set the alarm clock, or if they had turned the stove off. These people may have to check as many as 50 times before they can continue on with their daily routine.

Hoarders will check the garbage to ensure they did not throw out something that could be valuable. They are afraid of discarding anything. Commonly the live in a clutter of "saved, valuable possessions."

Washers have a fear of dirt and germs. They are known to have raw, chapped hands because of repetitive hand washing. Some may refuse to touch money, books, or other people because of their fear of contamination. In extreme cases, they may refuse to step out of doors.

Counters have a need to count meaningless things. They may count slats on a venetian blind, steps to the corner store, pickets in a fence, or smarties in a box. They may need to play math games in their heads, have lucky and unlucky numbers, and may react by ticcing when they hear a certain number or number sequence.

Other obsessive-compulsive behaviours include: excessive talking, excessive collecting of things, ritual behaviours, and specific ritual religious practices.

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Attention Deficit Hyperactivity Disorder (ADHD)

It is estimated that about 10% of school aged children have problems with paying attention, controlling their impulses, and are sufficiently overactive to be diagnosed with ADHD. This condition, that is often a manifestation of TS, has gone under-diagnosed. Teachers often assume that the child with TS and ADHD is spoiled, and a little discipline would solve the problem. These children can be disruptive and difficult in the classroom.

The child with ADHD seldom finishes assignments, is disorganized, and appears not to be paying attention to his surroundings. When completed, assignments are usually late. The child has a tendency to blurt out answers rather than wait to be asked. Often questions are answered before the question is completed. This restless child will find it impossible to stay seated for a long period to time and will interrupt his teacher or fellow students. At home, they will follow their parents or older siblings around demanding to be entertained. When they begin an activity, they soon drop it for another, leaving a string of unfinished projects. Their impulsive actions, social immaturity, and aggression make them unpopular with peers.

Geneticists have not been able as yet to establish a link between TS and ADHD, but the two are quite often found together. Some scientists have postulated that ADHD is a by-product of TS, explaining that the energy required in focusing on suppressing the tics of TS is so great that it is difficult, or at times impossible, to focus clearly on anything else. TS children with ADHD show no symptoms of either disorder when they are focused on an activity that is stimulating and challenging.

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Treatment

In early medical records and literature, it was found that rare cases of TS responded to psychotherapeutic treatment. These patients were documented as nervous. Some were treated with shock therapy, lobotomy, and even exorcism.

Until 1961, with the discovery of Haloperidol (Haldol), there was no effective treatment or medication for TS. Haldol is an anti-psychotic drug and it is not fully understood how it helps to reduce tics in TS. Haldol is a dopamine-blocking agent. It is theory that in cases where the over-production of dopamine is at fault Haldol is the medication of choice. This drug has side effects that range from excessive thirst to mental dulling and some patients choose not to use it for those reasons.

Clonodine (Catapres) is a totally different type of drug. It has also been around since the 1960's and used in the treatment of hypertension. It is not fully understood how it helps to control tics associated with TS. It also helps with attention problems and helps to modify some unruly behaviours in TS children. The major side effect of Catapres is that it could lower the blood pressure to a dangerous point. Patients on this medication need to have their blood pressure monitored closely.

There are other drugs used to help with the symptoms of TS, but Haldol and Catapres are the most popular as their side effects are mild compared to other medications.

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Historical Personalities With TS

When TS became an interest to medical science, attempts were made to identify personalities in history who suffered from this curious affliction. It is believed that Emperor Claudius of Rome (10BC-54AD) had TS. Claudius was considered as maligned, regarded as stupid, ill mannered, and an embarrassment to his family. He became emperor in 41AD and was astute enough to hold that position for thirteen years. His death is attributed to his fourth wife, Agrippina.

Claudius' behaviour was puzzling. When speaking, his head would jerk and his words alternated with gibberish. He slobbered when he spoke, spit, and was prone to fits of rage. He would blurt out indecent words and repeat things over and over. Claudius also had odd jerky body movements.

It has been suggested that Wolfgang Amadeus Mozart had TS. In his letters, Mozart used a profusion of profanity. He also played nonsensical word games, would mirror image his sentences, and repeat word sounds. With regard to motor tics, he was described as restless, constantly moving, and exhibiting facial grimaces. He was well known for making animal sounds. He attempted to cover his tics with silliness as many children with TS do.

Lexicographer and author, Samuel Johnson (1709-1784) reached historical fame for his "Dictionary of the English Language", published in 1755. It was by far, the most complete English dictionary of its time. Through his biography, "The Life of Samuel Johnson", James Boswell, shows a clear case of TS.

Johnson was a great wit and conversationalist. It is said of him that he was prone to almost constant and quite violent tics and odd gesticulations. He was described as having perpetual convulsive movements of the hands, lips, feet, and knees. In public, people would often gather around him and laugh at him. He was rejected from a jog as headmaster, because it was thought that his facial gestures and motor actions would have an adverse effect on the boys. His was not the last recorded job discrimination case on the grounds of TS. This man with untreated and uncontrollable TS became one of the most celebrated intellects of London.

A clear-cut case of TS is documented in 1810 by E.M.Bouteille, and the same one further studied and documented by De. J.M.G. Itard, in 1825. This was the case of the Marquise de Dampierre. This noblewoman started showing symptoms of TS at age seven. She had involuntary tics of the face, neck and arms. She made strange noises and uttered obscenities and nonsensical words and rhymes. She chose to live in solitude because of the outward signs of her afflictions. Her tics waxed and waned until her death at age 89.

Andre Malraux (1901-1976) became a leading figure in the literary and political life in France from the 1930's to 60's. During World War ll he was an influential figure in the French Resistance and the first unofficial press agent for Charles de Gaulle. He later became a member of de Gaulle's cabinet.

Malraux suffered from facial tics since he was a child. He took extreme efforts such as a "deep sleep cure" in Switzerland in the 60's. His vocal tics were described like the sound of the cry of a wounded jungle beast. His medical documents and course of treatment have been retained by his family and are not available.

More recent cases of TS include Mahmoud Abdul Rauf (formerly Chris Jackson) of basketball fame who was diagnosed with TS while still in high-school. He was the winner of several NCAA awards and voted All-American Player in 1988. He has great difficulty preparing for a game with things such as tying his shoes, but TS has in no way hampered his performance in the game.

Baseball star Jim Eisenreich was not correctly diagnosed until he was 23 years old. His symptoms have given him some difficulty on the playing field, but with medication to control his tics, he has acheived an impressive though belated career in baseball. There are two other major league baseball players who have TS but have chosen to keep their diagnosis private.

In the March 16, 1992 issue of New Yorker magazine, neurologist, Dr. Oliver Sacks writes the story of Dr. Walter Bennett, a Canadian surgeon with TS. This story is also available in an antology by Dr. Sacks titled, "An Anthropologist on Mars".

It is known that many people with TS perform the most extraordinary feats of precision, artistry and courage on a daily basis. It is no longer necessary for them to retreat, as did the Marquis de Dampeirre or be refused their choice of employment as in Samuel Johnson's case. Awareness opens the door to greater understanding and improved treatment. Let us all help open that door.

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Resources

Documentaries

Twitch and Shout
- Aired on TVO's "Vital Signs" January 28, 1997
- All Tourette cast, including cameraman discuss how TS affects their lives
- Shows many of the manifestations of TS

Magazine Articals

A Surgeon's Life
- Published in the "New Yorker" March 16, 1992
- Dr.Oliver Sacks describes the live of a Canadian surgeon with TS
- Very informative and humorous

Books

Children With Tourette Syndrome - A Parent's Guide
- Hearle, Tracy -Woodbine House Press, Rockville MD -1992
- An excellent guide
Tourette Syndrome and Human Behaviour
- Comings, David -E Hope Press Duarte CA 1990
- a very complete textbook

Newsletters

The Green Leaflet (summer 1995)
- Tourette Syndrome Foundation of Canada
- 203 - 3675 Keele Street, Downsview, Ontario M3J 1M6

Phamplets

Questions and Answers on Tourette Syndrome
- Tourette Syndrome Foundation of Canada
- 203 - 3675 Keele Street, Downsview, Ontario M3J 1M6
Facts you should know about the Genetics of Tourette Syndrome
- Tourette Syndrome Association of America
- 42 - 40 Bell Boulevard, Bayside NY, 11361

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