Diseases and Disorders

Tourette Syndrome - Life with Tics

Jasmin Alami


Abstract

The first recorded case of Tourette syndrome was in the year 1885. The French neurologist Georges Gilles de la Tourette described symptoms of involuntary, uncontrolled movements and sounds in nine of his patients [1][2]. Named after this doctor, today, the disorder is called Tourette syndrome and its main characteristic is tics, which are exactly what Gilles de la Tourette described many years ago. In addition to these tics, there are other disorders that often accompany Tourette’s. This article will discuss in detail what tics are, how the brain is altered in affected people and which methods are used in the treatment of this disorder.

 

Symptoms and Diagnosis

Tourette Syndrome (TS) is characterized by tics, which are involuntary, sudden, and repetitive vocalizations or movements [3]. They first appear during childhood, usually at the age of four to six, and are about four times more common among boys than girls [1][3]. They continue to become worse until their severity peaks in the early teen years. The symptoms of most TS patients then improve during adolescence and might even disappear completely [3]. As mentioned before, tics can be movements or vocalizations and they are categorized accordingly. First, there are motor tics. Most commonly present in the head and shoulder area, however they may later become more complex and spread to the extremities. Examples of common motor tics include blinking, twitching, and grimacing of the face [3]. The second type of tics, vocal tics, are sounds the patient produces with their voice, such as humming, clearing the throat or yelling certain words or phrases. Echolalia, repeating someone’s words, and coprolalia, yelling inappropriate words, account for other forms of vocal tics [4]. Another component of a tic, other than the actual execution, is the premonitory urge, which a patient experiences in the moments leading up to the tic. One could compare it to the moment before sneezing when you can already anticipate the sneeze [3]. Completing the tic relieves this premonitory urge [5]. 

In addition to tics, people suffering from TS have a high chance of comorbidity with several other disorders with about 90% of the patients experiencing such. Especially common are attention-deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). Another potential comorbid condition is autism spectrum disorder (ASD) [5].

Diagnosing TS is not as easy and definite as diagnosing many other disorders because no single test exists. This is why a clinical diagnosis is based on the expression of the symptoms. A person has Tourette’s syndrome if they express both motor and vocal tics over the course of at least one year [4].

 

Pathology

The cause of Tourette Syndrome can be attributed to genetic as well as environmental factors and is a very complex subject matter [5]. As to the genetic origin, there are many genes involved in the development of TS. And even though no single gene has been found responsible so far, the presence of a genetic connection is commonly accepted [6]. In many cases, a child with a parent that suffered or still suffers from Tourette syndrome is more likely to develop the disorder themselves [5]. Nevertheless, it was shown that genetic factors do not solely account for the cause of Tourette’s. Environmental factors such as heavy smoking during pregnancy and complications during childbirth can influence the development of the syndrome as well [6].

Regarding the pathophysiology of Tourette syndrome, several scientific studies suggest that the underlying changes are spread over many areas of the brain rather than concentrated in one place. For one, there is a reduced number of GABAergic interneurons, which would normally inhibit the cortico-basal ganglia pathways [5]. The basal ganglia play an important role in the production of motor movements. Thus, there is no proper inhibition of movements in TS patients. Additionally, neural circuits that connect the limbic and sensory systems to other structures are also affected by this disinhibition, which accounts for the sensory aspect of the syndrome. Finally, the neurotransmitters histamine and dopamine are implicated in the genesis of TS because they are present in abnormal levels [5].

Treatment 

Although tics are involuntary, it is possible for some affected patients to temporarily suppress them. However, this suppression has exhausting effects and is not a long-term solution. Another way of ameliorating the symptoms is concentrating on a calm activity that includes fine movements. Focusing on playing an instrument or playing a sport, for example, can make the tics less severe. In contrast to that, stress can worsen them [3]. Still, these methods are only temporary and have no long-term cure. As no such cure has been found yet, treatment is symptomatic [4]. Because many patients’ lives are not drastically affected by the tics, it is not necessary to treat them in this case. If they do interfere with everyday life or are painful, medication can be used to treat Tourette syndrome [4]. Drugs used in treatment include alpha-adrenergic agonists, antipsychotics, botulinum neurotoxin, and sometimes cannabinoids [7]. It is important to note, however, that these drugs cannot be taken mindlessly as they carry a variety of side effects with them [1]. An alternative approach is behavioral therapy, where the patient learns to recognize the premonitory urge and replace the tic with another, more healthy habit [5]. If the tics continue in their severity and significantly disable the patient, deep brain stimulation is an option for adults. This is an invasive procedure that targets structures of the basal ganglia [7]. 

 

Conclusion

As much as we know about Tourette syndrome, there are still many secrets about it to be discovered. Research is currently being done in order to find out more about its cause and the effects on the brain, with the goal of finding better treatment options or even a cure in mind [5]. The future needs to focus not only on the biochemical aspects of the disorder, but in equal intensity on the behavioral and social causes and effects on an individual. Being different and mocked for it can take a big toll on a young child’s psyche and can – regardless of whether their tics improve – have a long-lasting impact on their life. It is therefore necessary for us as a society to not only progress the research on the topic, but also raise public awareness and acceptance of Tourette syndrome.


References


  1. National Institute of Neurological Disorders and Stroke. (01/2012). Tourette Syndrome Fact Sheet. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Tourette-Syndrome-Fact-Sheet#3231_8. Retrieved: 03/09/2019.

  2. Lajonchere, C. (06/1996). Gilles de la Tourette and the Discovery of Tourette Syndrome. Archives of Neurology. 53(6), 567. Retrieved: 03/09/2019.

  3. Bloch, M. H., & Leckman, J. F. (12/2009). Clinical course of Tourette syndrome. Journal of Psychosomatic Research. 67(6), 497–501. Retrieved: 02/09/2019.

  4. Center for Disease Control and Prevention. (18/07/2019). What is Tourette’s Syndrome? https://www.cdc.gov/ncbddd/tourette/facts.html. Retrieved: 02/09/2019.

  5. Dale, R. C. (12/2017). Tics and Tourette: a clinical, pathophysiological and etiological review. Current Opinion in Pediatrics. 29(6), 665–673. Retrieved: 02/09/2019.

  6. Jakubovski, E., & Müller-Vahl, K. (06/2017). Gilles de la Tourette-Syndrom: Klinik, Ursachen, Therapie. Psychotherapie Psychosomatik Medizinische Psychologie. 67(06), 252–268. Retrieved: 03/09/2019.

  7. Hallett, Mark. (08/2015). Tourette Syndrome: Update. Brain & development. 37(7), 651–655. Retrieved: 02/09/2019.

  8. Cognitive Behavior Therapy Center of Sacramento Valley. Tourette Syndrome and Tic Disorders. http://cbtsacramento.com/obsessive-compulsive-ocd-therapy-counseling/tourette-syndrome-tic-disorders/. Retrieved: 04/09/2019.

  9. Rivlin, Michal. (19/01/2015). Introduction to Neuroscience: The Basal Ganglia. https://www.weizmann.ac.il/neurobiology/labs/ulanovsky/sites/neurobiology.labs.ulanovsky/files/uploads/intro_systemsneurosci_lecture12_rivlin_basal_ganglia_19jan2015.pdf. Retrieved: 04/09/2019.

Jasmin Alami

Jasmin Alami


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