Tourette Syndrome

Overview

Tourette Syndrome (TS) is a type of tic disorder that affects the development of the nervous system.[1] Individuals with TS experience sudden, uncontrollable movements or sounds known as tics.[2] These tics can be repetitive and unwanted. The worldwide prevalence of Tourette Syndrome is estimated to be between 0.3% and 1%, although a milder or unidentified form may affect 1% to 3% of children and adolescents.[3]

Symptoms

According to the Tic Association, tics typically emerge between the ages of 5 and 7 years as a motor tic in the head or neck region.[4] They then tend to increase in frequency and severity between the ages of 8 and 12 years, ranging from mild to severe, until late adolescence, when most people see improvements, even becoming tic-free in some cases.[5] Tics regularly change in type, frequency, and severity for unknown reasons or due to specific internal/external factors. (e.g., stress, anxiety, excitement, fatigue, illness).[6] A study by Prado et al. (2008) found that participants felt a premonitory urge with more than 80% of the tics.[7] These premonitory urges involved distinct cognition, emotion, or localized sensation/discomfort (e.g., itching, tingling, tension in the neck, nasal stuffiness, sore/dry throat).[8]

The National Institute of Neurological Disorders and Stroke (2023) and the Tic Association (2023) present the following distinctions in tics:[9,10]

Simple motor: sudden, brief, repetitive motor movements that involve few muscle groups and often come before complex tics.
Eye blinking and other eye movements
Facial grimacing
Shoulder shrugging
Head or shoulder jerking.
Simple vocal: sudden, brief, repetitive sounds.
Repetitive throat clearing
Sniffing
Barking
Grunting
Complex motor: involves multiple muscle groups or combinations of movements and tends to be slower and more purposeful in appearance.
Sniffing or touching an object
Hopping
Jumping
Bending
Twisting
Complex vocal: words or phrases that may or may not be recognizable but consistently occur out of context.
Repeating one's own words or phrases
Repeating others' words or phrases (echolalia)
Using vulgar, obscene, or swear words (coprolalia)

Coprolalia is often portrayed, and even mocked, in the media as a common symptom of Tourette disorder; thus, it is essential to note that coprolalia accounts for only 10-15% of cases and that (as with other tics) it is uncontrollable.[11]

Causes

While the cause of Tourette Ssyndrome is unknown, the National Institute of Neurological Disorders and Stroke spotlights the following current research that points to abnormalities in:[12]

Specific brain regions, including the basal ganglia, frontal lobes, and cortex
Circuits that connect these regions
Neurotransmitters (dopamine, serotonin, and norepinephrine) responsible for communication between nerve cells (neurons)
Changes (mutations) in one or more genes have been shown to be involved with TS. A small number of people with TS have mutations involving the SLITRK1 gene, which affects how neurons grow and connect. Abnormalities in the NRXN1 and CNTN6 genes, which also regulate the normal formation of these nerve connections, also may play a role in TS. Scientists continue to look for other genes involved with TS. Although there may be a few genes with substantial effects, many genes with more negligible effects and environmental factors may play a role in the development of TS. It is essential to understand that genetic tendency may not necessarily result in TS; instead, it may express itself as a milder tic disorder or as obsessive-compulsive behavior. It is also possible that children who inherit the gene abnormality will not develop any TS symptoms.

Risk Factors

Particular family histories (e.g., tics, Tourette Syndrome, ADHD, and OCD) can increase the likelihood of an individual developing Tourette Syndrome.[13] While ethnicity does not influence the risk, gender plays a significant role, with males being affected three to four times more frequently than females.[14] Additionally, external factors can trigger or exacerbate the severity of tics in Tourette Syndrome. Tics may worsen due to excitement or anxiety but improve during periods of calm, focused activity, or sleep.[15] Physical experiences (e.g., tight collars triggering neck tics or hearing someone sniff or clear their throat causing similar sounds) can also trigger tics.[16]

Diagnosis

There are no tests to confirm the diagnosis of Tic Disorders; however, other tests may be done to rule out other conditions. A licensed healthcare professional will use the following diagnostic criteria:[17]

At least 2 motor tics and at least 1 vocal (phonic) tic have been present, not necessarily simultaneously.
Tics may wax and wane in frequency but have occurred for more than 1 year.
Tics started to appear before the age of 18.
The use of a substance or other medical condition does not cause tics.

Specific Tic Disorders also have unique diagnostic criteria:

Persistent (Chronic) Motor or Vocal Tic Disorder: Either motor tics OR vocal tics have been present for more than 1 year; cannot be both motor and vocal tics.
Provisional Tic Disorder: Motor and/or vocal tics have been present for less than 1 year and have not met the criteria for TS, persistent (chronic) motor or vocal tic disorder.

Conditions that can occur along with Tourette Syndrome include ADHD/ADD, impulsivity, and obsessive-compulsive disorder (OCD).[18] Additional neurobehavioral co-occurring conditions may include anxiety, learning disabilities, behavior or conduct issues (e.g., aggression, anger, problems adjusting emotionally and socially), problems falling or staying asleep, and deficits in social skills.[19]

Complications

Motor tics can sometimes inadvertently lead to self-harm due to the type and severity of the movement. Some examples of motor tics include punching oneself in the face or "whiplash tics," which can lead to cervical artery dissection and non-compressive or compressive cervical myelopathy.[20,21] While some repetitive motor tics may not be severely impairing, they can still be painful. The pain associated with these less aggressive tics can be managed with heat or ice, massage, stretching, environmental alterations, and relaxation techniques.[22]

Tourette Syndrome can also create challenges for children in school. According to a study by Claussen et al. (2018), children with TS were more likely to have an individualized education program, have a parent contacted about school problems, and not complete their homework.[23] Another study by Charania et al. (2021) found that children with Tourette Syndrome were more likely to be involved in bullying, both as victims and as perpetrators.[24]

Despite these challenges, most people with Tourette Syndrome are able to lead productive lives with no barriers to achievement in their personal and professional lives.[25]

Treatment

There is currently no "cure" for Tourette Syndrome or evidence that indicates a relation between better outcomes and early intervention.[26] Thus, if individuals with TS report no decreased quality of life, no treatment may be appropriate since treatment is geared at minimizing tic intensity and relieving as much associated psychological and physical distress as possible.[27] Regarding medications, dopamine blockers are the most consistently useful in suppressing tics.[28] To better quality of life, stimulant medication can lessen ADHD symptoms in people with TS without increasing tic severity; furthermore, serotonin reuptake inhibitors can help some people control symptoms of depression, OCD, and anxiety.[29] A recent National Institutes of Health-funded, multi-center randomized control trial named Cognitive Behavioral Intervention for Tics (CBIT) found that training to voluntarily move in response to a premonitory urge can reduce tic symptoms.[30] CBIT is a non-medicated treatment consisting of three crucial components: training to become more aware of the tic and the premonitory urge, training patients to do competing behavior when they feel the urge to tic, and making routine changes to help reduce tics.[31]

Dealing with tics in certain public venues and situations can be tricky, but Waltz (2023) suggests some strategies that may help:[32]

Firstly, do not isolate yourself. Even though you may receive unpleasant comments and stares, you have the right to enjoy public spaces like everyone else. In most public places (e.g., libraries, swimming pools, shopping malls, and sidewalks), there is no need to worry about your tics. However, some places like upscale restaurants or the opera may require special consideration where you may wish to reduce your tics - for such rare exceptions, see below.

Another effective strategy is to bring something attention-absorbing. When your mind is occupied, your tics may decrease. So, carry a book of challenging crosswords, number puzzles, a hand-held computer game, needlework, a sketchbook, or an iPod with your favorite music playlist. These activities can help you get absorbed and reduce your tic frequency.

If you feel nervous, bringing a friend can help put strangers around you at ease. Planning and preparing for your tics can also minimize discomfort and potential reactions. For instance, if you have a spitting tic, carry a handkerchief to be discreet. If you sniff loudly, try using Vicks VapoRub or a similar strong-smelling ointment under your nose. This may change the sensation, interrupting the tic for a while. If you have complex tics that slow you down, leave some extra time to get into and out of events. Moreover, if you find that you are more relaxed after a workout or yoga class, plan your schedule accordingly.

Finally, prepare yourself to respond to rude comments or behavior. If someone is rude, you can explain your situation, complain to the venue's management, or take your business elsewhere. Do not take it personally - you cannot control your tics, but you can educate others and raise awareness. Furthermore, do not forget to seek opportunities to release energy and enjoy life. Activities such as running, skiing, swing dancing, swimming, or bicycling can be great outlets for your energy and help you relax. This is especially useful for those suffering from tics which can cause physical discomfort and harm to oneself.

Contributed by: Maria Karla Bermudez

Editor: Jennifer (Ghahari) Smith, Ph.D.

References

1 U.S. Department of Health and Human Services. (2023, February 1). Tourette syndrome. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/health-information/disorders/tourette-syndrome

2 Ibid.

3 Kallol K. Set, Jacqueline N. Warner,Tourette syndrome in children: An update, Current Problems in Pediatric and Adolescent Health Care,Volume 51, Issue 7,2021,101032,ISSN 1538-5442,https://doi.org/10.1016/j.cppeds.2021.101032.

4 What is Tourette. Tourette Association of America. (2023, August 14). https://tourette.org/about-tourette/overview/what-is-tourette/

5 Ibid.

6 Ibid.

7 H.S. Prado, M.C. Rosario, J. Lee, et al. Sensory phenomena in obsessive-compulsive disorder and tic disorders: a review of the literature CNS Spectr, 13 (2008), pp. 425-432

8 Ibid.

9 National Institute of Neurological Disorders and Stroke (2023)

10 Tourette Association of America (2023)

11 Ibid.

12 National Institute of Neurological Disorders and Stroke (2023)

13 Tourette Association of America (2023)

14 Ibid.

15 National Institute of Neurological Disorders and Stroke (2023)

16 Ibid.

17 Diagnosis. Tourette Association of America. (2023a, August 11). https://tourette.org/about-tourette/overview/diagnosis/

18 Ibid.

19 National Institute of Neurological Disorders and Stroke (2023)

20 Ibid.

21 Kallol et al. (2021)

22 Ibid.

23 Claussen AH, Bitsko RH, Holbrook JR, Bloomfield J, Giordano K. Impact of Tourette syndrome on school measures in a nationally representative sample. J Dev Behav Pediatr. 2018;39(4):335–342

24 Charania SN, Danielson ML, Claussen AH, Lebrun-Harris LA, Kaminski JW, Bitsko RH. Bullying victimization and perpetration among US children with and without Tourette syndrome. J Dev Behav Pediatr. Published online May 26, 2021

25 FAQs. Tourette Association of America. (2023b, August 11). https://tourette.org/about-tourette/overview/faqs/

26 Kallol et al. (2021)

27 Ibid.

28 National Institute of Neurological Disorders and Stroke (2023)

29 Ibid.

30 Ibid.

31 CBIT, HRT, habit reversal therapy, behavior therapy. Tourette Association of America. (2023a, August 11). https://tourette.org/research-medical/cbit-overview/

32 Waltz, M. (2023, August 11). Ticcing in public. Tourette Association of America. https://tourette.org/about-tourette/adults-ticcing-public/