Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Review Article
  • Published:

Advances in understanding and treatment of Tourette syndrome

Abstract

Tourette syndrome is a hereditary, childhood-onset neurodevelopmental disorder that was first clearly described in France in 1885. This disorder is characterized by sudden, rapid, recurrent, nonrhythmic movements (motor tics) or sounds (vocal or phonic tics), often preceded by premonitory sensations or urges. Some individuals also have psychiatric comorbidities, notably attention-deficit hyperactivity disorder or obsessive–compulsive disorder. Tourette syndrome occurs worldwide, in all races and ethnicities, in both sexes and in children as well as in adults. Estimates of its prevalence in children vary, with rates of up to 1% being reported, but rates of 0.3–0.8% are thought to accurately reflect the occurrence of the disorder. Research has led to progress in many aspects of Tourette syndrome, although many questions and unmet needs remain. For example, except for rare cases, the genetic basis remains elusive. The anatomical and neuronal changes in the brain that underlie Tourette syndrome are also unclear, although the evidence increasingly implicates alterations in basal ganglia function. Treatment is often unnecessary for individuals with mild tics, but for those with moderate to severe forms of the syndrome, some drugs are available, albeit frequently ineffective. Behavioral and surgical therapies, in particular deep brain stimulation, are currently undergoing development and show promising results. This Review examines the history of Tourette syndrome and describes its clinical presentation. The article also provides an overview of the epidemiology and pathophysiology of this disorder. Current treatment strategies and potential future therapies are also discussed.

Key Points

  • Tourette syndrome is a hereditary, childhood-onset neurodevelopmental disorder that was first clearly described in 1885

  • Tourette syndrome is clinically characterized by sudden, rapid, recurrent, nonrhythmic movements (motor tics) or sounds (vocal or phonic tics), which are often accompanied by psychiatric comorbidities

  • The genetic basis of Tourette syndrome is not well-elucidated and, except for rare cases, the underlying mutations remain elusive

  • The pathophysiology of Tourette syndrome remains unclear, although increasing evidence suggests a role for alterations in basal ganglia function

  • Pharmacological treatments are available, but are often ineffective; behavioral and surgical therapies are undergoing development and have shown promising results

  • Future research into Tourette syndrome should focus on investigating the pathophysiology of disease, development of suitable animal models and discovery of safe and effective therapies

This is a preview of subscription content, access via your institution

Access options

Rent or buy this article

Prices vary by article type

from$1.95

to$39.95

Prices may be subject to local taxes which are calculated during checkout

Figure 1
Figure 2: The consistently increasing level of research into Tourette syndrome from the 1960s to 2008.
Figure 3: Timeline showing key discoveries and advances in Tourette syndrome.

Similar content being viewed by others

References

  1. Gilles de la Tourette, G. Étude sur une affection nerveuse caractérisée par de l'incoordination motrice accompagnée d'écholalie et de coprolalie [French]. Arch. Neurol. 9, 158–200 (1885).

    Google Scholar 

  2. Walusinski, O. Jean-Martin Charcot's house officers at La Salpêtrière Hospital. Front. Neurol. Neurosci. 29, 9–35 (2011).

    Article  Google Scholar 

  3. Trivet, H. A., Chien, H. F., Munhoz, R. P. & Barbosa, E. R. Charcot's contribution to the study of Tourette syndrome. Arq. Neuropsiquiatr. 66, 918–921 (2008).

    Article  Google Scholar 

  4. Rickards, H. & Cavanna, A. E. Gilles de la Tourette: the man behind the syndrome. J. Psychosom. Res. 67, 469–474 (2009).

    Article  Google Scholar 

  5. Goetz, C. G., Chmura, T. A. & Lanska, D. J. History of tic disorders and Gilles de la Tourette syndrome: part 5 of the MDS-sponsored history of movement disorders exhibit, Barcelona, June 2000. Mov. Disord. 16, 346–349 (2001).

    Article  CAS  Google Scholar 

  6. Finger, S. Origins of Neuroscience: the History of Explorations into Brain Function 220–239 (Oxford University Press, New York, 1994).

    Google Scholar 

  7. Itard, J. M. Mémoire sur quelques functions involontaires des appareils de la locomotion, de la préhension et de la voix [French]. Arch. Gen. Med. 8, 385–407 (1825).

    Google Scholar 

  8. Trousseau, A. Clinique Médicale de l'Hôtel Dieu de Paris 264–271 (J.-B. Bailliere, Paris, 1868).

    Google Scholar 

  9. Rickards, H., Woolf, I. & Canvanna, A. E. “Trousseau's disease:” a description of the Gilles de la Tourette syndrome 12 years before 1885. Mov. Disord. 25, 2285–2289 (2010).

    Article  Google Scholar 

  10. Hughlings Jackson, J. Clinical Lectures and Reports to the London Hospital 1, 452 (1884).

    Google Scholar 

  11. Freeman, R. D. et al. Coprophenomena in Tourette syndrome. Dev. Med. Child. Neurol. 51, 218–227 (2009).

    Article  Google Scholar 

  12. Steinberg, T. et al. Tic disorders and the premonitory urge. J. Neurol. Transm. 117, 277–284 (2010).

    Article  Google Scholar 

  13. Kwak, C., Dat Vuong, K. & Jankovic, J. Premonitory sensory phenomenon in Tourette's syndrome. Mov. Disord. 18, 1530–1533 (2003).

    Article  Google Scholar 

  14. Eddy, C. M. et al. Clinical correlates of quality of life in Tourette syndrome. Mov. Disord. 26, 735–738 (2011).

    Article  Google Scholar 

  15. Lewin, A. B. et al. A phenomenological investigation of women with Tourette or other chronic tic disorders. Compr. Psychiatry http://dx.doi.org/10.1016/j.comppsych.2011.07.004.

  16. Kurlan, R. et al. The behavioral spectrum of tic disorders: a community-based study. Neurology 59, 414–420 (2002).

    Article  CAS  Google Scholar 

  17. Freeman, R. D. & Tourette Syndrome International Database Consortium. Tic disorders and ADHD: answers from a world-wide clinical dataset on Tourette syndrome. Eur. Child. Adolesc. Psychiatry 16, 15–23 (2007).

    Article  Google Scholar 

  18. Zinner, S. H. & Coffey, B. J. Developmental and behavioral disorders grown up: Tourette's disorder. J. Dev. Behav. Pediatr. 30, 560–573 (2009).

    Article  Google Scholar 

  19. O'Rourke, J. A. et al. The familial association of tourette's disorder and ADHD: The impact of OCD symptoms. Am. J. Med. Genet. B Neuropsychiatr. Genet. 156, 553–560 (2011).

    Article  Google Scholar 

  20. Bloch, M. H. & Leckman, J. F. Clinical course of Tourette syndrome. J. Psychosom. Res. 67, 497–501 (2009).

    Article  Google Scholar 

  21. Jankovic, J., Gelineau-Kattner, R. & Davidson, A. Tourette's syndrome in adults. Mov. Disord. 25, 2171–2175 (2010).

    Article  Google Scholar 

  22. Trajanovic, N. N., Voloh, I., Shapiro, C. M. & Sandor, P. REM sleep behaviour disorder in a child with Tourette's syndrome. Can. J. Neurol. Sci. 31, 572–575 (2004).

    Article  Google Scholar 

  23. Lombroso, P. J., Mack, G., Scahill, L., King, R. A. & Leckman, J. F. Exacerbation of Gilles de la Tourette's syndrome associated with thermal stress: a family study. Neurology 41, 1984–1987 (1991).

    Article  CAS  Google Scholar 

  24. Leckman, J. F., Bloch, M. H., Scahill, L. & King, R. A. Tourette syndrome: the self under siege. J. Child. Neurol. 21, 642–649 (2006).

    Article  Google Scholar 

  25. Conelea, C. A. et al. Exploring the impact of chronic tic disorders on youth: results from the Tourette Syndrome Impact Survey. Child. Psychiatry Hum. Dev. 42, 219–242 (2011).

    Article  Google Scholar 

  26. Freeman, R. D. et al. An international perspective on Tourette syndrome: selected findings from 3,500 individuals in 22 countries. Dev. Med. Child Neurol. 42, 436–447 (2000).

    Article  CAS  Google Scholar 

  27. Robertson, M. M., Eapen, V. & Cavanna, A. E. The international prevalence, epidemiology, and clinical phenomenology of Tourette syndrome: a cross-cultural perspective. J. Psychosom. Res. 67, 475–483 (2009).

    Article  Google Scholar 

  28. Centers for Disease Control and Prevention (CDC). Prevalence of diagnosed Tourette syndrome in persons aged 6–17 years—United States, 2007. MMWR Morb. Mortal. Wkly Rep. 58, 581–585 (2009).

  29. Kurlan, R. et al. Prevalence of tics in schoolchildren and association with placement in special education. Neurology 57, 1383–1388 (2001).

    Article  CAS  Google Scholar 

  30. Scahill, L., Sukhodolsky, D. G., Williams, S. K. & Leckman, J. F. Public health significance of tic disorders in children and adolescents. Adv. Neurol. 96, 240–248 (2005).

    PubMed  Google Scholar 

  31. Motlagh, M. G. et al. Severe psychosocial stress and heavy cigarette smoking during pregnancy: an examination of the pre- and perinatal risk factors associated with ADHD and Tourette syndrome. Eur. Child Adolesc. Psychiatry 19, 755–764 (2010).

    Article  Google Scholar 

  32. Murphy, T. K., Kurlan, R. & Leckman, J. The immunobiology of Tourette's disorder, pediatric autoimmune neuropsychiatric disorders associated with Streptococcus, and related disorders: a way forward. J. Child Adolesc. Psychopharm. 20, 317–331 (2010).

    Article  Google Scholar 

  33. O'Rourke, J. A., Scharf, J. M., Yu, D. & Pauls, D. L. The genetics of Tourette syndrome: a review. J. Psychosom. Res. 67, 533–545 (2009).

    Article  Google Scholar 

  34. State, M. W. The genetics of child psychiatric disorders: focus on autism and Tourette syndrome. Neuron 68, 254–269 (2010).

    Article  CAS  Google Scholar 

  35. Pauls, D. L., Cohen, D. J., Heimbuch, R., Detlor, J. & Kidd, K. K. Familial pattern and transmission of Gilles de la Tourette syndrome and multiple tics. Arch. Gen. Psychiatry 38, 1091–1093 (1981).

    Article  CAS  Google Scholar 

  36. Kidd, K. K., Prusoff, B. A. & Cohen, D. J. Familial pattern of Gilles de la Tourette syndrome. Arch. Gen. Psychiatry 37, 1336–1339 (1980).

    Article  CAS  Google Scholar 

  37. Price, R. A., Kidd, K. K., Cohen, D. J., Pauls, D. & Leckman, J. F. A twin study of Tourette syndrome. Arch. Gen. Psychiatry 42, 815–820 (1985).

    Article  CAS  Google Scholar 

  38. Hyde, T. M., Aaronson, B. A., Randolph, C., Rickler, K. C. & Weinberger, D. R. Relationship of birth weight to the phenotypic expression of Gilles de la Tourette's syndrome in monozygotic twins. Neurology 42, 652–658 (1992).

    Article  CAS  Google Scholar 

  39. Bolton, D., Rijsdijk, F., O'Connor, T. G., Perrin, S. & Eley, T. C. Obsessive–compulsive disorder, tics and anxiety in 6-year-old twins. Psychol. Med. 37, 39–48 (2007).

    Article  Google Scholar 

  40. Kurlan, R., Eapen, V., Stern, J., McDermott, M. P. & Robertson, M. M. Bilineal transmission in Tourette's syndrome families. Neurology 44, 2336–2342 (1994).

    Article  CAS  Google Scholar 

  41. Tourette Syndrome Association International Consortium for Genetics. Genome scan for Tourette disorder in affected-sibling-pair and multigenerational families. Am. J. Human. Genet. 80, 265–272 (2007).

  42. Abelson, J. F. et al. Sequence variants in SLITRK1 are associated with Tourette's syndrome. Science 310, 317–320 (2005).

    Article  CAS  Google Scholar 

  43. Ercan-Sencicek, A. G. et al. L-histidine decarboxylase and Tourette's syndrome. N. Engl. J. Med. 362, 1901–1908 (2010).

    Article  CAS  Google Scholar 

  44. Sundaram, S. K., Huq, A. M., Wilson, B. J. & Chugani, H. T. Tourette syndrome is associated with recurrent exonic copy number variants. Neurology 74, 1583–1590 (2010).

    Article  Google Scholar 

  45. Leckman, J. F., Bloch, M. H., Smith, M. E., Larabi, D. & Hampson, M. Neurobiological substrates of Tourette's disorder. J. Child Adolesc. Psychopharmacol. 20, 237–247 (2010).

    Article  Google Scholar 

  46. Kataoka, Y. et al. Decreased number of parvalbumin and cholinergic interneurons in the striatum of individuals with Tourette syndrome. J. Comp. Neurol. 518, 277–291 (2010).

    Article  Google Scholar 

  47. Kalanithi, P. S. et al. Altered parvalbumin-positive neuron distribution in basal ganglia of individuals with Tourette syndrome. Proc. Natl Acad. Sci. USA 102, 13307–13312 (2005).

    Article  CAS  Google Scholar 

  48. Rickards, H. Functional neuroimaging in Tourette syndrome. J. Psychosom. Res. 67, 575–584 (2009).

    Article  Google Scholar 

  49. Hyde, T. M. et al. Cerebral morphometric abnormalities in Tourette's syndrome: a quantitative MRI study of monozygotic twins. Neurology 45, 1176–1182 (1995).

    Article  CAS  Google Scholar 

  50. Peterson, B. S. et al. Basal ganglia volumes in patients with Gilles de la Tourette syndrome. Arch. Gen. Psychiatry 60, 415–424 (2003).

    Article  Google Scholar 

  51. Lee, J. S. Abnormal thalamic volume in treatment-naïve boys with Tourette syndrome. Acta Psychiatr. Scand. 113, 64–67 (2006).

    Article  Google Scholar 

  52. Sowell, E. R. et al. Thinning of sensorimotor cortices in children with Tourette syndrome. Nat. Neurosci. 11, 637–639 (2008).

    Article  CAS  Google Scholar 

  53. Peterson, B. S. et al. A functional magnetic resonance imaging study of tic suppression in Tourette syndrome. Arch. Gen. Psychiatry 55, 326–333 (1998).

    Article  CAS  Google Scholar 

  54. Bohlhalter, S. et al. Neural correlates of tic generation in Tourette syndrome: an event-related functional MRI study. Brain 129, 2029–2037 (2006).

    Article  CAS  Google Scholar 

  55. Krack, P., Hariz, M. I., Baunez, C., Guridi, J. & Obeso, J. A. Deep brain stimulation: from neurology to psychiatry. Trends Neurosci. 33, 474–484 (2010).

    Article  CAS  Google Scholar 

  56. Hassler, R. & Dieckmann, G. Traitement stereotaxique des tics et cris inarticulés ou coprolaliques considérés comme phénomène d'obsession motrice au cours de la maladie de Gilles de la Tourette [French]. Rev. Neurol. (Paris) 123, 89–100 (1970).

    CAS  Google Scholar 

  57. Temel, Y. & Visser-Vandewalle, V. Surgery in Tourette syndrome. Mov. Disord. 19, 3–14 (2004).

    Article  Google Scholar 

  58. Servello, D., Porta, M., Sassi, M., Brambilla, A. & Robertson, M. M. Deep brain stimulation in 18 patients with severe Gilles de la Tourette syndrome refractory to treatment: the surgery and stimulation. J. Neurol. Neurosurg. Psychiatry 79, 136–142 (2008).

    Article  CAS  Google Scholar 

  59. Ackermans, L. et al. Deep brain stimulation in Tourette's syndrome: two targets? Mov. Disord. 21, 709–713 (2006).

    Article  Google Scholar 

  60. Shale, H., Fahn, S. & Mayeux, R. Tics in a patient with Parkinson's disease. Mov. Disord. 1, 79–83 (1986).

    Article  CAS  Google Scholar 

  61. Parraga, H. C., Harris, K. M., Parraga, K. L., Balen, G. M. & Cruz, C. An overview of the treatment of Tourette's disorder and tics. J. Child Adolesc. Psychopharmacol. 20, 249–262 (2010).

    Article  Google Scholar 

  62. Singer, H. S. Treatment of tics and Tourette syndrome. Curr. Treat. Options Neurol. 12, 539–561 (2010).

    Article  Google Scholar 

  63. Zinner, S. H. & Mink, J. W. Movement disorders I: tics and stereotypies. Pediatr. Rev. 31, 223–233 (2010).

    Article  Google Scholar 

  64. Conelea, C. A. et al. Exploring the impact of chronic tic disorders on youth: results from the Tourette Syndrome Impact Survey. Child Psychiatry Hum. Dev. 42, 219–242 (2011).

    Article  Google Scholar 

  65. Piacentini, J. et al. Behavior therapy for children with Tourette disorder: a randomized controlled trial. JAMA. 303, 1929–1937 (2010).

    Article  CAS  Google Scholar 

  66. Ward, H. E., Hwynn, N. & Okun, M. S. Update on deep brain stimulation for neuropsychiatric disorders. Neurobiol. Dis. 38, 346–353 (2010).

    Article  Google Scholar 

  67. Shapiro, A. K. & Shapiro, E. Treatment of Gilles de la Tourette's syndrome with haloperidol. Br. J. Psychiatry 114, 345–350 (1968).

    Article  CAS  Google Scholar 

  68. Kwak, C. H., Hanna, P. A. & Jankovic, J. Botulinum toxin in the treatment of tics. Arch. Neurol. 57, 1190–1193 (2000).

    Article  CAS  Google Scholar 

  69. Ondo, W. G., Jong, D. & Davis, A. Comparison of weight gain in treatments for Tourette syndrome: tetrabenazine versus neuroleptic drugs. J. Child Neurol. 23, 435–437 (2008).

    Article  Google Scholar 

  70. Erenberg, G., Cruse, R. P. & Rothner, A. D. Gilles de la Tourette's syndrome: effects of stimulant drugs. Neurology 35, 1346–1348 (1985).

    Article  CAS  Google Scholar 

  71. Tourette's Syndrome Study Group. Treatment of ADHD in children with tics: a randomized controlled trial. Neurology 58, 527–536 (2002).

  72. Bloch, M. H., Panza, K. E., Landeros-Weisenberger, A. & Leckman, J. F. Meta-analysis: treatment of attention-deficit/hyperactivity disorder in children with comorbid tic disorders. J. Am. Acad. Child Adolesc. Psychiatry 48, 884–893 (2009).

    Article  Google Scholar 

  73. Miguel, E. C., Shavitt, R. G., Ferrão, Y. A., Brotto, S. A. & Diniz, J. B. How to treat OCD in patients with Tourette syndrome. J. Psychosom. Res. 55, 49–57 (2003).

    Article  Google Scholar 

  74. Azrin, N. H. & Nunn, R. G. Habit-reversal. a method of eliminating nervous habits and tics. Behav. Res. Ther. 11, 619–628 (1973).

    Article  CAS  Google Scholar 

  75. Verdellen, C. W., Keijsers, G. P., Cath, D. C. & Hoogduin, C. A. Exposure with response prevention versus habit reversal in Tourette's syndrome: a controlled study. Behav. Res. Ther. 24, 501–511 (2004).

    Article  Google Scholar 

  76. Burdick, A. et al. Lack of benefit of accumbens/capsular deep brain stimulation in a patient with both tics and obsessive–compulsive disorder. Neurocase 16, 321–330 (2010).

    Article  Google Scholar 

  77. Dueck, A. et al. Deep brain stimulation of globus pallidus internus in a 16-year-old boy with severe Tourette syndrome and mental retardation. Neuropediatrics 40, 239–242 (2009).

    Article  CAS  Google Scholar 

  78. Mink, J. W. et al. Patient selection and assessment recommendations for deep brain stimulation in Tourette syndrome. Mov. Disord. 21, 1831–1838 (2006).

    Article  Google Scholar 

  79. Kompoliti, K., Fan, W. & Leurgans, S. Complementary and alternative medicine use in Gilles de la Tourette syndrome. Mov. Disord. 24, 2015–2019 (2009).

    Article  Google Scholar 

  80. O'Connor, K. P. et al. Cognitive behavioral management of Tourette's syndrome and chronic tic disorder in medicated and unmedicated samples. Behav. Res. Ther. 47, 1090–1095 (2009).

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Contributions

Both authors contributed equally to all aspects of the article, including researching data, discussion of content, and writing, reviewing and editing the manuscript before submission.

Corresponding author

Correspondence to Kevin St. P. McNaught.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

McNaught, K., Mink, J. Advances in understanding and treatment of Tourette syndrome. Nat Rev Neurol 7, 667–676 (2011). https://doi.org/10.1038/nrneurol.2011.167

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/nrneurol.2011.167

This article is cited by

Search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing