Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Tourette syndrome

Joseph Jankovic, MD
Section Editor
Douglas R Nordli, Jr, MD
Deputy Editor
John F Dashe, MD, PhD


Tourette syndrome (TS) is a neurological disorder manifested by motor and phonic tics with onset during childhood [1,2]. This topic will review Tourette syndrome. Other hyperkinetic movement disorders are discussed separately. (See "Hyperkinetic movement disorders in children".)


TS is thought to result from a complex interaction between social and environmental factors and multiple genetic abnormalities. In most cases a bilineal transmission (inheritance from both parents) is clearly evident [3]. Despite a large number of genetic studies, no single or even multiple causative genes for TS have been identified [4]. The disorder likely results from a disturbance in the striatal-thalamic-cortical (mesolimbic) spinal system, which leads to disinhibition of the motor and limbic system [5].

Although the genetic basis remains elusive, several loci have been identified as candidate susceptibility regions [6]. The discovery of a mutation in the SLITRK1 gene on chromosome 13q31.1 was a major advance in the search for the elusive TS gene or genes [7]. The SLITRK1 gene is expressed in brain regions previously implicated in TS (cortex, hippocampus, thalamic, subthalamic and globus pallidus nuclei, striatum, and cerebellum) and it appears to play a role in dendritic growth. However, it is not clear how the altered gene product leads to the complex neurobehavioral disorder. This mutation appears to be a rare cause of TS as it has not been found in hundreds of TS patients tested.

Another possible rare genetic cause of TS is a mutation in the HDC gene on chromosome 15q21-q22, as detected in two generations of a family with apparent autosomal dominant inheritance of TS [8]. The HDC gene encodes for L-histidine decarboxylase, which is the rate-limiting enzyme that catalyzes the biosynthesis of histamine from histidine. In the central nervous system, histaminergic neurons are located in the posterior hypothalamus but have widespread axonal connections to other brain regions [9]. These findings suggest the possibility of using pharmacologic manipulation of histaminergic neurotransmission to treat TS [8]. However, it is unknown how histamine abnormalities might cause or contribute to TS symptoms.

Neuropathologic examinations have detected no consistent brain abnormalities in patients with TS, but a number of neuroimaging studies have found evidence of structural or metabolic changes in the brain [10]:

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Oct 2017. | This topic last updated: Aug 22, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Kurlan R. Clinical practice. Tourette's Syndrome. N Engl J Med 2010; 363:2332.
  2. Jankovic J, Kurlan R. Tourette syndrome: evolving concepts. Mov Disord 2011; 26:1149.
  3. Cuker A, State MW, King RA, et al. Candidate locus for Gilles de la Tourette syndrome/obsessive compulsive disorder/chronic tic disorder at 18q22. Am J Med Genet A 2004; 130A:37.
  4. Pauls DL, Fernandez TV, Mathews CA, et al. The Inheritance of Tourette Disorder: A review. J Obsessive Compuls Relat Disord 2014; 3:380.
  5. Leckman JF, Cohen DJ, Goetz CG, Jankovic J. Tourette syndrome: pieces of the puzzle. Adv Neurol 2001; 85:369.
  6. Deng H, Gao K, Jankovic J. The genetics of Tourette syndrome. Nat Rev Neurol 2012; 8:203.
  7. Abelson JF, Kwan KY, O'Roak BJ, et al. Sequence variants in SLITRK1 are associated with Tourette's syndrome. Science 2005; 310:317.
  8. Ercan-Sencicek AG, Stillman AA, Ghosh AK, et al. L-histidine decarboxylase and Tourette's syndrome. N Engl J Med 2010; 362:1901.
  9. Haas HL, Sergeeva OA, Selbach O. Histamine in the nervous system. Physiol Rev 2008; 88:1183.
  10. Robertson MM. The Gilles de la Tourette syndrome: the current status. Arch Dis Child Educ Pract Ed 2012; 97:166.
  11. Fredericksen KA, Cutting LE, Kates WR, et al. Disproportionate increases of white matter in right frontal lobe in Tourette syndrome. Neurology 2002; 58:85.
  12. Muellner J, Delmaire C, Valabrégue R, et al. Altered structure of cortical sulci in gilles de la Tourette syndrome: Further support for abnormal brain development. Mov Disord 2015; 30:655.
  13. Plessen KJ, Bansal R, Peterson BS. Imaging evidence for anatomical disturbances and neuroplastic compensation in persons with Tourette syndrome. J Psychosom Res 2009; 67:559.
  14. Pourfar M, Feigin A, Tang CC, et al. Abnormal metabolic brain networks in Tourette syndrome. Neurology 2011; 76:944.
  15. Scharf JM, Miller LL, Gauvin CA, et al. Population prevalence of Tourette syndrome: a systematic review and meta-analysis. Mov Disord 2015; 30:221.
  16. Freeman RD, Fast DK, Burd L, et al. An international perspective on Tourette syndrome: selected findings from 3,500 individuals in 22 countries. Dev Med Child Neurol 2000; 42:436.
  17. 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 2009; 58:581.
  18. Knight T, Steeves T, Day L, et al. Prevalence of tic disorders: a systematic review and meta-analysis. Pediatr Neurol 2012; 47:77.
  19. Stillman A, Ercan-Sencicek AG, State MW. Tourette disorder overview. GeneReviews. www.ncbi.nlm.nih.gov/books/NBK21138/ (Accessed on June 10, 2011).
  20. Robertson MM. The prevalence and epidemiology of Gilles de la Tourette syndrome. Part 1: the epidemiological and prevalence studies. J Psychosom Res 2008; 65:461.
  21. Browne HA, Modabbernia A, Buxbaum JD, et al. Prenatal Maternal Smoking and Increased Risk for Tourette Syndrome and Chronic Tic Disorders. J Am Acad Child Adolesc Psychiatry 2016; 55:784.
  22. Leckman JF, Zhang H, Vitale A, et al. Course of tic severity in Tourette syndrome: the first two decades. Pediatrics 1998; 102:14.
  23. Robertson MM. The Gilles de la Tourette syndrome: the current status. Br J Psychiatry 1989; 154:147.
  24. Jankovic J, Gelineau-Kattner R, Davidson A. Tourette's syndrome in adults. Mov Disord 2010; 25:2171.
  25. Jankovic J, Mejia NI. Tics associated with other disorders. Adv Neurol 2006; 99:61.
  26. Demirkiran M, Jankovic J. Paroxysmal dyskinesias: clinical features and classification. Ann Neurol 1995; 38:571.
  27. Kwak C, Dat Vuong K, Jankovic J. Premonitory sensory phenomenon in Tourette's syndrome. Mov Disord 2003; 18:1530.
  28. Lin JJ, Wang HS, Wong MC, et al. Tourette's syndrome with cervical disc herniation. Brain Dev 2007; 29:61.
  29. Krauss JK, Jankovic J. Severe motor tics causing cervical myelopathy in Tourette's syndrome. Mov Disord 1996; 11:563.
  30. Dobbs M, Berger JR. Cervical myelopathy secondary to violent tics of Tourette's syndrome. Neurology 2003; 60:1862.
  31. Isaacs JD, Adams M, Lees AJ. Noncompressive myelopathy associated with violent axial tics of tourette syndrome. Neurology 2010; 74:697.
  32. van Meerbeeck P, Behar C, Czernecki V, et al. Motor tic of the neck: a probable cause of stroke in a child with Gilles de la Tourette syndrome. Mov Disord 2011; 26:928.
  33. Lehman LL, Gilbert DL, Leach JL, et al. Vertebral artery dissection leading to stroke caused by violent neck tics of Tourette syndrome. Neurology 2011; 77:1706.
  34. Hirschtritt ME, Lee PC, Pauls DL, et al. Lifetime prevalence, age of risk, and genetic relationships of comorbid psychiatric disorders in Tourette syndrome. JAMA Psychiatry 2015; 72:325.
  35. Kurlan R, Como PG, Miller B, et al. The behavioral spectrum of tic disorders: a community-based study. Neurology 2002; 59:414.
  36. Ghosh D, Rajan PV, Das D, et al. Sleep disorders in children with Tourette syndrome. Pediatr Neurol 2014; 51:31.
  37. Lee WT, Huang HL, Wong LC, et al. Tourette Syndrome as an Independent Risk Factor for Subsequent Sleep Disorders in Children: A Nationwide Population-Based Case-Control Study. Sleep 2017; 40.
  38. Kwak C, Vuong KD, Jankovic J. Migraine headache in patients with Tourette syndrome. Arch Neurol 2003; 60:1595.
  39. Ghosh D, Rajan PV, Das D, et al. Headache in children with Tourette syndrome. J Pediatr 2012; 161:303.
  40. Barabas G, Matthews WS, Ferrari M. Tourette's syndrome and migraine. Arch Neurol 1984; 41:871.
  41. Tulen JH, Azzolini M, de Vries JA, et al. Quantitative study of spontaneous eye blinks and eye tics in Gilles de la Tourette's syndrome. J Neurol Neurosurg Psychiatry 1999; 67:800.
  42. Farber RH, Swerdlow NR, Clementz BA. Saccadic performance characteristics and the behavioural neurology of Tourette's syndrome. J Neurol Neurosurg Psychiatry 1999; 66:305.
  43. Peterson BS, Thomas P, Kane MJ, et al. Basal Ganglia volumes in patients with Gilles de la Tourette syndrome. Arch Gen Psychiatry 2003; 60:415.
  44. Bloch MH, Leckman JF, Zhu H, Peterson BS. Caudate volumes in childhood predict symptom severity in adults with Tourette syndrome. Neurology 2005; 65:1253.
  45. Definitions and classification of tic disorders. The Tourette Syndrome Classification Study Group. Arch Neurol 1993; 50:1013.
  46. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), American Psychiatric Association, Arlington, VA 2013.
  47. Robertson MM, Banerjee S, Kurlan R, et al. The Tourette syndrome diagnostic confidence index: development and clinical associations. Neurology 1999; 53:2108.
  48. Jankovic J. Differential diagnosis and etiology of tics. Adv Neurol 2001; 85:15.
  49. Prado HS, Rosário MC, Lee J, et al. Sensory phenomena in obsessive-compulsive disorder and tic disorders: a review of the literature. CNS Spectr 2008; 13:425.
  50. Sanger TD, Chen D, Fehlings DL, et al. Definition and classification of hyperkinetic movements in childhood. Mov Disord 2010; 25:1538.
  51. Serajee FJ, Mahbubul Huq AH. Advances in Tourette syndrome: diagnoses and treatment. Pediatr Clin North Am 2015; 62:687.
  52. Kurlan RM. Treatment of Tourette syndrome. Neurotherapeutics 2014; 11:161.
  53. Pringsheim T, Marras C. Pimozide for tics in Tourette's syndrome. Cochrane Database Syst Rev 2009; :CD006996.
  54. Kenney C, Jankovic J. Tetrabenazine in the treatment of hyperkinetic movement disorders. Expert Rev Neurother 2006; 6:7.
  55. Wijemanne S, Wu LJ, Jankovic J. Long-term efficacy and safety of fluphenazine in patients with Tourette syndrome. Mov Disord 2014; 29:126.
  56. Jankovic J. Therapeutic developments for tics and myoclonus. Mov Disord 2015.
  57. Tourette's Syndrome Study Group. Treatment of ADHD in children with tics: a randomized controlled trial. Neurology 2002; 58:527.
  58. Goetz CG, Tanner CM, Wilson RS, et al. Clonidine and Gilles de la Tourette's syndrome: double-blind study using objective rating methods. Ann Neurol 1987; 21:307.
  59. Gaffney GR, Perry PJ, Lund BC, et al. Risperidone versus clonidine in the treatment of children and adolescents with Tourette's syndrome. J Am Acad Child Adolesc Psychiatry 2002; 41:330.
  60. Du YS, Li HF, Vance A, et al. Randomized double-blind multicentre placebo-controlled clinical trial of the clonidine adhesive patch for the treatment of tic disorders. Aust N Z J Psychiatry 2008; 42:807.
  61. Leckman JF, Hardin MT, Riddle MA, et al. Clonidine treatment of Gilles de la Tourette's syndrome. Arch Gen Psychiatry 1991; 48:324.
  62. Scahill L, Chappell PB, Kim YS, et al. A placebo-controlled study of guanfacine in the treatment of children with tic disorders and attention deficit hyperactivity disorder. Am J Psychiatry 2001; 158:1067.
  63. Cummings DD, Singer HS, Krieger M, et al. Neuropsychiatric effects of guanfacine in children with mild tourette syndrome: a pilot study. Clin Neuropharmacol 2002; 25:325.
  64. Murphy TK, Fernandez TV, Coffey BJ, et al. Extended-Release Guanfacine Does Not Show a Large Effect on Tic Severity in Children with Chronic Tic Disorders. J Child Adolesc Psychopharmacol 2017; 27:762.
  65. Weisman H, Qureshi IA, Leckman JF, et al. Systematic review: pharmacological treatment of tic disorders--efficacy of antipsychotic and alpha-2 adrenergic agonist agents. Neurosci Biobehav Rev 2013; 37:1162.
  66. Jankovic J, Jimenez-Shahed J, Brown LW. A randomised, double-blind, placebo-controlled study of topiramate in the treatment of Tourette syndrome. J Neurol Neurosurg Psychiatry 2010; 81:70.
  67. Yang CS, Zhang LL, Zeng LN, et al. Topiramate for Tourette's syndrome in children: a meta-analysis. Pediatr Neurol 2013; 49:344.
  68. Scott BL, Jankovic J, Donovan DT. Botulinum toxin injection into vocal cord in the treatment of malignant coprolalia associated with Tourette's syndrome. Mov Disord 1996; 11:431.
  69. Marras C, Andrews D, Sime E, Lang AE. Botulinum toxin for simple motor tics: a randomized, double-blind, controlled clinical trial. Neurology 2001; 56:605.
  70. Kwak CH, Hanna PA, Jankovic J. Botulinum toxin in the treatment of tics. Arch Neurol 2000; 57:1190.
  71. Porta M, Maggioni G, Ottaviani F, Schindler A. Treatment of phonic tics in patients with Tourette's syndrome using botulinum toxin type A. Neurol Sci 2004; 24:420.
  72. Simpson DM, Blitzer A, Brashear A, et al. Assessment: Botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2008; 70:1699.
  73. McGuire JF, Piacentini J, Brennan EA, et al. A meta-analysis of behavior therapy for Tourette Syndrome. J Psychiatr Res 2014; 50:106.
  74. Piacentini J, Woods DW, Scahill L, et al. Behavior therapy for children with Tourette disorder: a randomized controlled trial. JAMA 2010; 303:1929.
  75. Azrin NH, Nunn RG. Habit-reversal: a method of eliminating nervous habits and tics. Behav Res Ther 1973; 11:619.
  76. Schrock LE, Mink JW, Woods DW, et al. Tourette syndrome deep brain stimulation: a review and updated recommendations. Mov Disord 2015; 30:448.
  77. Viswanathan A, Jimenez-Shahed J, Baizabal Carvallo JF, Jankovic J. Deep brain stimulation for Tourette syndrome: target selection. Stereotact Funct Neurosurg 2012; 90:213.
  78. Zhang JG, Ge Y, Stead M, et al. Long-term outcome of globus pallidus internus deep brain stimulation in patients with Tourette syndrome. Mayo Clin Proc 2014; 89:1506.
  79. Kefalopoulou Z, Zrinzo L, Jahanshahi M, et al. Bilateral globus pallidus stimulation for severe Tourette's syndrome: a double-blind, randomised crossover trial. Lancet Neurol 2015; 14:595.
  80. Welter ML, Houeto JL, Thobois S, et al. Anterior pallidal deep brain stimulation for Tourette's syndrome: a randomised, double-blind, controlled trial. Lancet Neurol 2017; 16:610.
  81. Piacentini J, Chang S. Behavioral treatments for Tourette syndrome and tic disorders: state of the art. Adv Neurol 2001; 85:319.
  82. Nass R, Bressman S. Attention deficit hyperactivity disorder and Tourette syndrome: what's the best treatment? Neurology 2002; 58:513.
  83. Bloch MH, Panza KE, Landeros-Weisenberger A, Leckman JF. Meta-analysis: treatment of attention-deficit/hyperactivity disorder in children with comorbid tic disorders. J Am Acad Child Adolesc Psychiatry 2009; 48:884.
  84. Cohen SC, Mulqueen JM, Ferracioli-Oda E, et al. Meta-Analysis: Risk of Tics Associated With Psychostimulant Use in Randomized, Placebo-Controlled Trials. J Am Acad Child Adolesc Psychiatry 2015; 54:728.
  85. Mataix-Cols D, Isomura K, Pérez-Vigil A, et al. Familial Risks of Tourette Syndrome and Chronic Tic Disorders. A Population-Based Cohort Study. JAMA Psychiatry 2015; 72:787.
  86. Hanna PA, Janjua FN, Contant CF, Jankovic J. Bilineal transmission in Tourette syndrome. Neurology 1999; 53:813.
  87. Kano Y, Ohta M, Nagai Y, et al. A family study of Tourette syndrome in Japan. Am J Med Genet 2001; 105:414.
  88. Pauls DL, Raymond CL, Stevenson JM, Leckman JF. A family study of Gilles de la Tourette syndrome. Am J Hum Genet 1991; 48:154.
  89. Walkup JT, LaBuda MC, Singer HS, et al. Family study and segregation analysis of Tourette syndrome: evidence for a mixed model of inheritance. Am J Hum Genet 1996; 59:684.
  90. McMahon WM, Carter AS, Fredine N, Pauls DL. Children at familial risk for Tourette's disorder: Child and parent diagnoses. Am J Med Genet B Neuropsychiatr Genet 2003; 121B:105.
  91. Groth C, Mol Debes N, Rask CU, et al. Course of Tourette Syndrome and Comorbidities in a Large Prospective Clinical Study. J Am Acad Child Adolesc Psychiatry 2017; 56:304.
  92. Bagheri MM, Kerbeshian J, Burd L. Recognition and management of Tourette's syndrome and tic disorders. Am Fam Physician 1999; 59:2263.