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Can’t Control – The Anatomy of Tourette Syndrome

We can control movement by our own free will. But not all of us can do that. Some people cannot control their movements – and even speech – voluntarily. This doesn’t necessarily mean they’re crazy. It’s beyond their control. This condition is called Tourette Syndrome.

What is Tourette Syndrome?

Tourette syndrome (TS) is a neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics. The disorder is named for Dr. Georges Gilles de la Tourette, the pioneering French neurologist who in 1885 first described the condition in an 86-year-old French noblewoman.[1] TS does characteristically wax and wane, can be suppressed temporarily, and are preceded by a premonitory urge. Tourette’s is defined as part of a spectrum of tic disorders, which includes provisional, transient and persistent (chronic) tics.[2] Usually, the tics can manifest right away then slowly disappears and then appears again. Sometimes, Tourette Syndrome is called the swearing disease[3] because some people with TS involuntarily blurts out inappropriate comments and/or curse words. TS does not affect intelligence or life expectancy.

How common is Tourette Syndrome?

Tourette Syndrome is not as rare as it presents, though uncommon, with mild symptoms to some people. Males are about three to four times more likely than females to develop Tourette syndrome.[4] Between 0.4% and 3.8% of children ages 5 to 18 may have Tourette’s[2][5];the prevalence of other tic disorders in school-age children is higher, with the more common tics of eye blinking, coughing, throat clearing, sniffing, and facial movements.[2]

What causes Tourette Syndrome?

Genetics and environment may play a role in the development of TS, but the exact cause is unknown.[2] Experts don’t know the exact cause of TS, but some research points to changes in the brain and problems with how nerve cells communicate. A disturbance in the balance of neurotransmitters — chemicals in the brain that carry nerve signals from cell to cell — might play a role.[6] Tics are believed to result from dysfunction in cortical and subcortical regions, the thalamus, basal ganglia and frontal cortex. Neuroanatomic models implicate failures in circuits connecting the brain’s cortex and subcortex, and imaging techniques implicate the basal ganglia and frontal cortex.[7]

What are the symptoms of Tourette Syndrome?

The main symptoms of TS are motor tics (sudden, apparently uncontrollable movements like exaggerated blinking of the eyes) or vocal tics (apparently uncontrollable uttered sounds such as throat clearing, grunting, or sniffing).[6] When under stress, the tics usually exacerbate. It can be prolonged or changed.

Tics are classified as either simple or complex. Simple motor tics usually involve just one group of muscles. Some examples are eye blinking and grimacing. In contrast, complex motor tics usually involve more muscle groups and might look like a series of movements.[6] Simple vocal tics can be throat clearing, sniffing, or humming, whereas complex vocal tics can involve repeating other people’s words (a condition called echolalia) or involuntary swearing (called coprolalia).[6]

Motor tics involve movement. They include[8]:

Arm or head jerking
Blinking
Making a face
Mouth twitching
Shoulder shrugging

Vocal tics include[8]:

Barking or yelping
Clearing your throat
Coughing
Grunting
Repeating what someone else says
Shouting
Sniffing
Swearing

Before a motor tic happens, a person with TS may get a sensation that can feel like a tingle or tension. The movement makes the sensation go away.[8]

Symptoms usually subside as the child grows up and tics can become minimal when they become adolescents and adults.

Some people can control tic symptoms. But tension builds, and it eventually has to be released as a tic.[6] Since controlling a tic requires much effort, they usually cannot concentrate in other activities (i.e. classroom or work).

image

This is an illustration of how Tourette Syndrome is presented.[7]

How is Tourette Syndrome diagnosed?

For Tourette Syndrome to be diagnosed, the patient is referred to a neurologist. There he or shecwill be tested and asked by the neurologist about how TS presents. The following are the usual questions asked[8]:

-What did you notice that brought you here today?
-Do you often move your body in a way you can’t control? How long has that been happening?
-Do you ever say things or make sounds without meaning to? When did it start?
-Does anything make your symptoms better? What makes them worse?
-Do you feel anxious or have trouble focusing?
-Does anyone else in your family have these kinds of symptoms?

Testing includes[8]:

MRI. It uses powerful magnets and radio waves to make pictures of organs and structures inside your body.
CT scan. It’s a powerful X-ray that makes detailed images of your insides.

How is Tourette Syndrome treated?

There is no cure for TS. But there are strategies used to manage and/or control its symptoms[9]:

Habit reversal therapy – involves monitoring the pattern and frequency of the tics and identifying any sensations that trigger them. The next stage is to find an alternative, less noticeable method of relieving the sensations that cause a tic (known as premonitory sensations). This is known as a competing response.[9]

Exposure with response prevention (ERP) –involves increasing exposure to the urge to tic in order to suppress the tic response for longer. This works on the theory that you get used to the feeling of needing to tic until the urge, and any related anxiety, decreases in strength.[9]

Medications can also help if the symptoms are more frequent and severe like alpha2-adrenergic agonists, muscle relaxants and dopamine antagonists[9] to relax muscles or control nerve impulses in tics.

Antidepressants and anxiolytics can help when the patient has depression and/or anxiety.

What is the prognosis for Tourette Syndrome?

The symptoms usually improve in two-thirds of TS cases after 10 years. The tics may diminish a lot and may even disappear. Medications and therapy are not necessarily needed. However, the symptoms continue in the remaining one-third of cases and medications will be continued. Nevertheless, TS symptoms get milder as the patient ages.

image
The cycle of Tourette Syndrome.[10]

What happens if Tourette Syndrome is not diagnosed?

If not identified right away, complications may arise. The patient may lose concentration in classes or work. They may even become subjects of ridicule and discrimination and he or she will have low self-esteem, anxiety, and depression.

Can someone with Tourette Syndrome be successful?

Yes. A person with TS can become successful in many areas of life given he or she has awareness, acceptance, and/or therapy or medicine. The most important factor is, that he or she will be given support and understanding instead of discrimination.

Below are some famous people with diagnosed or suspected TS[11]:

Jim Eisenreich, professional baseball player
Mahmoud Abdul-Rauf, professional basketball player
Samuel Johnson, British writer who penned the Dictionary of the English Language and the Lives of Poets
Wolfgang Amadeus Mozart (huh? Mozart again? He also had suspected ADHD and/or autism)
Tim Howard, soccer player[12]
Howard Hughes, one of the richest men in history[12]
Dan Ackroyd, actor[12] (he also had Aspergers)
David Beckham[12]
Michael Wolff, jazz musician and actor[12]
Dash Mihok, actor[12]

image
Image courtesy of njcts.org

So, people with TS should be treated equally with us instead of laughing at them. Who knows, the one with involuntary tics may become more successful than you!

image
Image courtesy of njcts.org

This concludes the description of the conditions nder the umbrella of neurodiversity. There are more conditions aside from dyslexia, ADHD, autism, dyspraxia, dyscalculia, and Tourette Syndrome, but I will discuss them in my following blog posts.

References:
1. http://www.ninds.nih.gov/disorders/tourette/detail_tourette.htm
2. http://en.m.wikipedia.org/wiki/Tourette_syndrome
3. http://www.reviewjournal.com/life/health/tourette-syndrome-challenges-patients-doctors-alike
4. http://www.mayoclinic.org/diseases-conditions/tourette-syndrome/basics/definition/con-20043570
5. Robertson MM. “Gilles de la Tourette syndrome: the complexities of phenotype and treatment”. Br J Hosp Med (Lond). 2011 Feb;72(2):100–7. PMID 21378617 
6. http://kidshealth.org/parent/medical/brain/tourette.html
7. http://www.medz.website/2014/12/tourette-syndrome-tourette-s-disorder.html
8. http://www.webmd.com/brain/tourettes-syndrome
9. http://www.nhs.uk/conditions/Tourette-syndrome/Pages/Introduction.aspx
10. http://www.netterimages.com/images/vpv/000/000/006/6082-0550×0475.jpg
11. https://faculty.washington.edu/chudler/ts.html#fp
12. http://www.disabled-world.com/artman/publish/tourettes-famous.shtml

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Wired Differently

I’m back. Last time, I introduced you what will I blog in this page. Now, I will define my topic. We sometimes encounter people who are weird in some ways and think and behave in a manner which deviates from normal. We usually refer to them as ‘wired differently’ or simply abnormal or ‘special.’ In the medical community, they are diagnosed with ‘learning disabilities.’ But not all of them agree with this concept. Instead, they advocate neurodiversity.

What? What’s that word again?

Neurodiversity. According to the definition in Wikipedia, neurodiversity is an approach to learning and disability that suggests that diverse neurological conditions appear as a result of normal variations in the human genome.[1] This term was coined in the late 1990s as a challenge to prevailing views of neurological diversity as inherently pathological, and it asserts that neurological differences should be recognized and respected as a social category on a par with gender, ethnicity, sexual orientation, or disability status.[2] To make this definition a little less complicated, let’s just compare this to biodiversity. Like in ecosystems where there are diverse species of life forms from plants to animals, the same goes for humans who have brains wired differntly resulting in multiple intelligences and differing in the way of thinking.

But how is that? Of course people think differently. Each person is unique.

Err, what shall I say? Yes, each individual thinks differently, but what I mean is the way the brain develops from childhood to adulthood. Let me explain further.

Neurodiversity encompasses all people whose brains develop differently from the normal people. The development can be either delayed or advanced or deficient. People under neurodiversity are called neurodovergent. Neurodiversity include dyslexia (difficulty in reading letters), attention deficit hyperactivity disorder (ADHD) (short attention span, hyperactivity, and impulsiveness), autism spectrum disorder (ASD) (hyperfocus to detail, deficient in social skills), Tourette syndrome (involuntary body movement), developmental coordination disorder (DCD) or dyspraxia (difficulty in planning and coordinating movement), and dyscalculia (difficulty in reading numbers, or ‘dyslexia of numbers.’) These conditions are also collectively known as learning disabilities. On the other hand, people whose brains develop without these conditions stated above are called neurotypical.[3]

This concept has attracted controversy because it attacks the traditional notion that ADHD, ASD, and the like are disabilities that are needed to be fixed or cured, but rather, respect the differences in thinking as part of the normal human genome variation, just like the variations in human sexual orientation or variations in human physical appearances.

Neurodiversity is a concept akin to biodiversity or cultural diversity that recognizes neurological disorders as a natural human variation. Rather than looking for cures, neurodiversity advocates work to promote social support systems and spotlight the value of neurological differences, in the same vein as variations in learning styles or social tendencies like introversion and extroversion.[4]

In short, people under neurodiversity are just normal variations of the human specie, not an abnormality of some sort.

To illustrate this, the diagram of neurodiversity[7] by the late Mary Colley, author of Living With Dypraxia, is shown below:
image

That’s the presentation with the difficulties associated with those conditions. The following diagram[8] below shows the strength with each condition:
image

They’re really overlapping. Okay, I think you are somewhat getting the point, but who started and how did neurodiversity begin?

An autism advocate and an autist herself, Judy Singer, coined the term in 1990s as part of the autism advocacy campaign.[2] Another autism advocate, Jim Sinclair, wrote in his 1993 article “Don’t Mourn For Us” told parents that the autism itself cannot be separated from the person who is born with it, but rather part of the person itself.[5] The term neurodiversity appeared on Harvey Blume’s 1998 The Atlantic article where he said, “Neurodiversity may be every bit as crucial for the human race as biodiversity is for life in general. Who can say what form of wiring will prove best at any given moment? Cybernetics and computer culture, for example, may favor a somewhat autistic cast of mind.”[6]

Since then, hundreds of people with otherwise neurotypical development have advocated neurodiversity as the way of being the way sub-Saharan Africans in the United States and LGBT communities have advocated their rights before. A lot of neurodiverse people have contributed to society whether be in art, science, politics, and so forth. However, people with neurodiverse conditions are still continued to be bullied, ridiculed, and abused in all walks of life from infancy to old age. That’s why they are prone to suffer from anxiety and depression. Nevertheless, neurodiversity campaign remains strong, and more neurotypical people are beginning to accept neurodiverse people as who they are, particularly in the Western World.

It’s a long way to go. They’re really wired differently, but the same members of the modern human specie Homo sapiens like us.

Next time, I will post about the different conditions under neurodiversity one by one, their presentation, diagnosis, treatment, prognosis, advocacy, and some samples of people who have these conditions.

References:

[1] Jaarsma P, Welin S (February 2011). “Autism as a Natural Human Variation: Reflections on the Claims of the Neurodiversity Movement” (PDF). Health Care Anal 20 (1): 20–30.
[2] http://en.m.wikipedia.org/wiki/Neurodiversity.
[3] Sinclair, Jim (1998). “A note about language and abbreviations”. Archived from the original on 2008-06-06.
[4] http://www.pbs.org/pov/neurotypical.
[5] Autism Network International newsletter, Our Voice, Volume 1, Number 3, 1993.
[6] Blume, Harvey (September 30, 1998). “Neurodiversity”. The Atlantic. Retrieved November 7, 2007.
[7] http://joelgethinlewis.com/2013/05/23/self-storm-troopers-strongbox-neurodiversity-and-snowfall/
[8] http://www.geniuswithin.co.uk/infographics/neuro-diversity-venn-diagram/