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Tourette Syndrome

Introduction

Tourette syndrome (TS) is a neuropsychiatric disorder that appears in childhood, wherein a person experiences involuntary movements (motor tics) and vocalisations (phonictics) that last for at least one year. It occurs more in males as compared to females with the ration of 4:1. Also, it tends to be more severe in males than females.[1] 0·3–0·9% of school-aged children (4–18 years) and 0·002–0·08% of adults have a diagnosis of Tourette syndrome, although there are a few adult epidemiological studies done worldwide[1].

Patients diagnosed with TS are usually accompanied by other comorbidities such as obsessive compulsive disorder, or OCD, and attention-deficit hypersensitivity disorder, or ADHD.[2] It presents one of the highest familial recurrence rates among neuropsychiatric disorders with complex patterns of inheritance. [3] Traditionally, it has been thought of as a serious illness; however, evidence from epidemiologic and genetic family studies suggests that severity is variable. [4]

Tourette Syndrome and OCD

A recent study addresses significant differences within and between diagnostic groups diagnosed as presenting only with OCD compared to those diagnosed with comorbid TS and OCD. Patients who had only been diagnosed with OCD listed a variety of traditional obsessions and compulsions. In comparison, those patients diagnosed comorbidly with TS and OCD had some particular propensities: for example, nonviolent visual obsessions, anxiety over appearance (eg Body Dysmorphic Disorder), and the acute need for symmetry. This group also reported a higher rate of compulsions, which involved compulsive touching, blinking, or staring, and counting behaviors. Of interest from the study's findings was that while eight of the patients who provided childhood history of ADHD were among those with both OCD and TS, none were found in those with only OCD. These results thus emphasize the unique and often complex symptom profiles typically seen in individuals carrying this comorbidity of TS and OCD. [5]

Etiology

Etiology typically includes

Genetics

There is a strong genetic link for Tourette syndrome[6][7]. First-degree relatives of individuals with tic disorders had significantly higher risk of having TS[8]

Environmental risk factors:

  • Smoking: Prenatal maternal smoking[9] was associated with increased risk.
  • Streptococcus infection: Some research indicates there may be a connection between the onset or aggravation of Tourette’s syndrome (TS) and previous Group A beta-hemolytic streptococcus infection[10][11]. However, other studies do not support the idea that exposure to Group A streptococcus is a contributing factor for the worsening of tics in children [12][13]

[14]

Clinical Presentation and Assessment

The clinical assessment of a child with a presumed tic disorder begins with clarification of the presenting symptoms. When tics are central to the complaints, identification of their onset and progression is often straightforward, since early manifestations typically involve eye blinking or facial movements. Parents often comment on fluctuations in the types and frequency of tics, reporting that one tic may abate while another emerges. [15]

It is critical to consider factors influencing tics to guide diagnosis and treatment. For instance, some medications, such as sympathomimetic decongestants like ephedrine, can exacerbate tics, as can environmental factors like temperature, fatigue, and stress. Recent findings also suggest that streptococcal infections may trigger or worsen tics and obsessive-compulsive symptoms in some cases. [15]

The expression of tics is very individualistic-there is a wide range from almost unnoticeable mild tics to severe and disruptive tics. Tics range in frequency and intensity, with some individuals having fewer symptoms on some days or, in some cases, actually experiencing periods of tic suppression.

What are Tics?

Typically, tics are characterized by brief, rapid repetitive movements or phonetic events that are nonrhythmic and involuntary (motor tics) such as blinking, coughing, sniffing, shoulder shrugging, neck stretching and throat clearing, facial grimacing or vocalizations such as throat clearing and grunting[16]. They can also include more complex movements and vocalizations. Tics typically begin in childhood and peak in severity in early adolescence before often declining in young adulthood. [17]

[18]


Classification of Tics in Tourette Syndrome
Type of Tics Description
Motor Tics Involuntary movements can be simple or complex.
Simple Motor Tics Quick, brief movements such as eye blinking, facial grimacing, head jerking, shoulder shrugging.
Complex Motor Tics More coordinated movements that may involve multiple muscle groups, such as touching objects or people, repetitive jumping or twirling, imitating others' movements (echopraxia).
Vocal Tics Involuntary sounds can also be classified as simple or complex.
Simple Vocal Tics Brief sounds like throat clearing, grunting, sniffing.
Complex Vocal Tics More elaborate sounds or phrases, such as repeating words or phrases (echolalia) or using inappropriate or offensive language (coprolalia).

[19]

Assessment of TS

A comprehensive history and direct clinical observation serve as the basis for diagnosing a tic disorder. The clinical interview should evaluate the following:

  • Onset and Progression of Symptoms
  • Current severity of motor and vocal tics
  • Some may provide premonitory sensations or enable them to suppress tics
  • Overall burden imposed by the tics

Distinguishing tics from other behavioral symptoms can be a challenge in diagnosis. There are no laboratory tests that can be used to diagnose tic disorders. Diagnosis is based upon a persistent history of motor and vocal tics and exclusion of other possible causes. Ordinarily, the standard neurological exam is normal except for the presence of tics. The gold standard for diagnosis and measurement of the severity of tics is thought of in terms of ratings based upon direct interviews and observations by an experienced clinician. [20]

Diagnostic Procedures

Diagnostic procedures may include the following:[6]

  1. Electroencephalography (EEG)
  2. Neuroimaging studies
  3. NeuropsychologyAssessment
  4. Tic Assessment

Differential Diagnosis

Tics can manifest in disorders other than Tourette syndrome, necessitating specific diagnostic tests to confirm these conditions. [20]. Such as:

Outcome Measures

1. The clinician-rated Yale Global Tic Severity Scale, widely regarded as the gold standard for rating tics in Tourette syndrome and other tic disorders. [21]

2. Change is often measured with the CGI-I Scale, reporting general change relative to baseline as rated by the clinician. [22]

3. Diagnostic Interview Schedule for Children (DISC): The sensitivity of the DISC in detecting Tourette syndrome is notably low, indicating a need for modifications and the development of more sensitive screening measures for TS. [23]

Management / Interventions

Treatment usually consists of educating the child, family, and school personnel regarding the natural course of the disorder and its behavioral features. Other therapeutic interventions are generally directed at treating the primary areas of impairment unique to each individual. [15]

Treatment of TS should be multidisciplinary: a pharmacological approach may involve medication, education of the family, school support, and psychotherapy with self-help groups as supplementary treatment. The major goal consists of reduction in the impact of TS on the child and the family and the promotion of optimal development. Interventions target specific symptoms, such as tics and obsessive-compulsive behaviors. [15]

Pharmacotherapy

The most effective treatments for reducing tic severity include antipsychotic medications such as haloperidol, pimozide, and risperidone. However, these medications rarely eliminate tics completely and are often associated with side effects, including sedation, weight gain, fatigue, nausea, dystonia, dysregulation of body temperature, headaches, hallucinations, and cognitive difficulties, and motor disturbances.[24] [17].

Conservative Management

To avoid the side-effects, more conservative approaches are often considered first‐line of approach[24]:

  • psychoeducation,
  • behavioral interventions,
  • biofeedback, and
  • intraoral devices

Surgical Approach

Other methods of treatment includes[6]

The Movement Disorders Society Tourette Syndrome Study Group published a survey indicating that tic disorders are often understudied outside specialized expertise, highlighting the need for improved diagnosis and treatment globally.[25]

Role of Physiotherapy

Recent research suggests that engaging in physical exercise may help reduce the severity of tics in the short term. Additionally, exercise appears to provide benefits for co-occurring symptoms often associated with Tourette Syndrome, such as anxiety. [26]

Chronic physical activity has been shown to significantly reduce the severity of tics, even at higher intensities. This suggests that the benefits of regular exercise extend beyond short-term relief. Several physiological mechanisms may explain the varying effects of acute versus chronic physical activity in individuals with Tourette syndrome. [27] For instance, consistent exercise may lead to long-term changes in brain function, hormone levels, and neurotransmitter balance, all of which can contribute to improved tic management and overall emotional well-being. However, current research on the impact of physical exercise on tic expression and associated mental health conditions in children with Tourette syndrome lacks sufficient quality evidence to draw definitive conclusions.[26]

Aerobic exercise may be a useful intervention for improving self-regulation of tics in young patients. A study[28] examining the effects of nonaerobic movement training (Tai Chi) and aerobic movement training (Kick Boxing) on behavioural measures of cognitive control and tic severity in a group of young people with Tourette Syndrome (TS) demonstrated a significant enhancement in cognitive control task performance, reduction in tic frequency (tics per minute) during both types of exercise, with effect significantly greater and sustained for longer time following Kick Boxing. This research suggests embracing a more active lifestyle and physical activity/ exercises could be integrated into clinical practices alongside existing treatments.

[29]

References

  1. ↑ 1.0 1.1 Johnson KA, Worbe Y, Foote KD, Butson CR, Gunduz A, Okun MS. Tourette syndrome: clinical features, pathophysiology, and treatment. The Lancet Neurology. 2023 Feb 1;22(2):147-58.
  2. ↑ Bloch MH, Leckman JF. Clinical course of Tourette syndrome. Journal of psychosomatic research. 2009 Dec 1;67(6):497-501.
  3. ↑ Scharf JM, Yu D, Mathews CA, Neale BM, Stewart SE, Fagerness JA, Evans P, Gamazon E, Edlund CK, Service SK, Tikhomirov A. Genome-wide association study of Tourette's syndrome. Molecular psychiatry. 2013 Jun;18(6):721-8.
  4. ↑ Scahill L, Ort SI, Hardin MT. Tourette's syndrome, Part I: Definition and diagnosis. Archives of psychiatric nursing. 1993 Aug 1;7(4):203-8.
  5. ↑ Petter T, Richter MA, Sandor P. Clinical features distinguishing patients with Tourette's syndrome and obsessive-compulsive disorder from patients with obsessive-compulsive disorder without tics. Journal of Clinical Psychiatry. 1998 Sep 15;59(9):456-9.
  6. ↑ 6.0 6.1 6.2 Hartmann A, Andrén P, Atkinson-Clement C, Czernecki V, Delorme C, Debes NM, Szejko N, Ueda K, Black K. Tourette syndrome research highlights from 2021. F1000Research. 2022;11.
  7. ↑ Deng H, Gao K, Jankovic J. The genetics of Tourette syndrome. Nature Reviews Neurology. 2012 Apr;8(4):203-13.
  8. ↑ Mataix-Cols D, Isomura K, Pérez-Vigil A, Chang Z, Rück C, Larsson KJ, Leckman JF, Serlachius E, Larsson H, Lichtenstein P. Familial risks of Tourette syndrome and chronic tic disorders: a population-based cohort study. JAMA psychiatry. 2015 Aug 1;72(8):787-93.
  9. ↑ Browne HA, Modabbernia A, Buxbaum JD, Hansen SN, Schendel DE, Parner ET, Reichenberg A, Grice DE. Prenatal maternal smoking and increased risk for Tourette syndrome and chronic tic disorders. Journal of the American Academy of Child & Adolescent Psychiatry. 2016 Sep 1;55(9):784-91.
  10. ↑ Swedo SE, Leonard HL, Garvey M, Mittleman B, Allen AJ, Perlmutter S, Dow S, Zamkoff J, Dubbert BK, Lougee L. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. InObsessive-Compulsive Disorder and Tourette's Syndrome 2022 Apr 18 (pp. 184-191). Routledge.
  11. ↑ Leslie DL, Kozma L, Martin A, Landeros A, Katsovich L, King RA, Leckman JF. Neuropsychiatric disorders associated with streptococcal infection: a case-control study among privately insured children. Journal of the American Academy of Child & Adolescent Psychiatry. 2008 Oct 1;47(10):1166-72.
  12. ↑ Martino D, Schrag A, Anastasiou Z, Apter A, Benaroya-Milstein N, Buttiglione M, Cardona F, Creti R, Efstratiou A, Hedderly T, Heyman I. Association of Group A Streptococcus exposure and exacerbations of chronic tic disorders: a multinational prospective cohort study. Neurology. 2021 Mar 23;96(12):e1680-93.
  13. ↑ Schrag A, Gilbert R, Giovannoni G, Robertson MM, Metcalfe C, Ben-Shlomo Y. Streptococcal infection, Tourette syndrome, and OCD: is there a connection?. Neurology. 2009 Oct 20;73(16):1256-63.
  14. ↑ Neuroscientifically Challenged. 2-Minute Neuroscience: Tourette Syndrome. Available from: http://www.youtube.com/watch?v=8brbjfNX8ck [last accessed 1/5/2025]
  15. ↑ 15.0 15.1 15.2 15.3 Scahill L, Ort SI, Hardin MT. Tourette's syndrome, part II: Contemporary approaches to assessment and treatment. Archives of Psychiatric Nursing. 1993 Aug 1;7(4):209-16.
  16. ↑ Sims A, Stack B. Tourette’s syndrome: a pilot study for the discontinuance of a movement disorder. CRANIO®. 2009 Jan 1;27(1):11-8.
  17. ↑ 17.0 17.1 Piacentini J, Woods DW, Scahill L, Wilhelm S, Peterson AL, Chang S, Ginsburg GS, Deckersbach T, Dziura J, Levi-Pearl S, Walkup JT. Behavior therapy for children with Tourette disorder: a randomized controlled trial. Jama. 2010 May 19;303(19):1929-37.
  18. ↑ Tourettes Action. What is Tourette Syndrome?. Available from: http://www.youtube.com/watch?v=6RFMUs0S6YQ[last accessed 1/5/2025]
  19. ↑ Tourette Association of America. Tourette Syndrome is... Available from: http://www.youtube.com/watch?v=M8clZP-PI2Y[last accessed 1/5/2025]
  20. ↑ 20.0 20.1 Scahill L, Erenberg G, Berlin Jr CM, Budman C, Coffey BJ, Jankovic J, Kiessling L, King RA, Kurlan R, Lang A, Mink J. Contemporary assessment and pharmacotherapy of Tourette syndrome. NeuroRx. 2006 Apr 1;3(2):192-206.
  21. ↑ Haas M, Jakubovski E, Fremer C, Dietrich A, Hoekstra PJ, Jäger B, Müller-Vahl KR, EMTICS Collaborative Group. Yale global tic severity scale (YGTSS): Psychometric quality of the gold standard for tic assessment based on the large-scale EMTICS study. Frontiers in psychiatry. 2021 Feb 25;12:626459.
  22. ↑ Jeon S, Walkup JT, Woods DW, Peterson A, Piacentini J, Wilhelm S, Katsovich L, McGuire JF, Dziura J, Scahill L. Detecting a clinically meaningful change in tic severity in Tourette syndrome: a comparison of three methods. Contemporary clinical trials. 2013 Nov 1;36(2):414-20.
  23. ↑ Lewin AB, Mink JW, Bitsko RH, Holbrook JR, Parker-Athill EC, Hanks C, Storch EA, Augustine EF, Adams HR, Vierhile AE, Thatcher AR. Utility of the diagnostic interview schedule for children for assessing Tourette syndrome in children. Journal of child and adolescent psychopharmacology. 2014 Jun 1;24(5):275-84.
  24. ↑ 24.0 24.1 Stahlke AH, Bonotto D, Bonotto DV, Hilgenberg‐Sydney PB. Conservative Management of Tourette Syndrome Tics Using Intraoral Occlusal Devices: Report of Two Cases. Special Care in Dentistry. 2025 Mar;45(2):e70032.
  25. ↑ Ganos C, Sarva H, Kurvits L, Gilbert DL, Hartmann A, Worbe Y, Mir P, Müller-Vahl KR, Münchau A, Shprecher D, Singer HS. Clinical practice patterns in tic disorders among Movement Disorder Society members. Tremor and Other Hyperkinetic Movements. 2021;11.
  26. ↑ 26.0 26.1 Reilly C, Grant M, Bennett S, Murphy T, Heyman I. Physical exercise in Tourette syndrome–a systematic review. Child and Adolescent Mental Health. 2019 Feb;24(1):3-11.
  27. ↑ Kim DD, Warburton DE, Wu N, Barr AM, Honer WG, Procyshyn RM. Effects of physical activity on the symptoms of Tourette syndrome: a systematic review. European Psychiatry. 2018 Feb;48(1):13-9.
  28. ↑ Jackson GM, Nixon E, Jackson SR. Tic frequency and behavioural measures of cognitive control are improved in individuals with Tourette syndrome by aerobic exercise training. Cortex. 2020 Aug 1;129:188-98.
  29. ↑ Child Mind Institute. What Are the Most Effective Treatments for Tourette's?. Available from: http://www.youtube.com/watch?v=4_qquZsLbYY[last accessed 1/5/2025]