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
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Tourette syndrome is a hereditary, childhood-onset neurodevelopmental disorder that was first clearly described in 1885
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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
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The genetic basis of Tourette syndrome is not well-elucidated and, except for rare cases, the underlying mutations remain elusive
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The pathophysiology of Tourette syndrome remains unclear, although increasing evidence suggests a role for alterations in basal ganglia function
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Pharmacological treatments are available, but are often ineffective; behavioral and surgical therapies are undergoing development and have shown promising results
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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
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References
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).
Walusinski, O. Jean-Martin Charcot's house officers at La Salpêtrière Hospital. Front. Neurol. Neurosci. 29, 9–35 (2011).
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).
Rickards, H. & Cavanna, A. E. Gilles de la Tourette: the man behind the syndrome. J. Psychosom. Res. 67, 469–474 (2009).
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).
Finger, S. Origins of Neuroscience: the History of Explorations into Brain Function 220–239 (Oxford University Press, New York, 1994).
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).
Trousseau, A. Clinique Médicale de l'Hôtel Dieu de Paris 264–271 (J.-B. Bailliere, Paris, 1868).
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).
Hughlings Jackson, J. Clinical Lectures and Reports to the London Hospital 1, 452 (1884).
Freeman, R. D. et al. Coprophenomena in Tourette syndrome. Dev. Med. Child. Neurol. 51, 218–227 (2009).
Steinberg, T. et al. Tic disorders and the premonitory urge. J. Neurol. Transm. 117, 277–284 (2010).
Kwak, C., Dat Vuong, K. & Jankovic, J. Premonitory sensory phenomenon in Tourette's syndrome. Mov. Disord. 18, 1530–1533 (2003).
Eddy, C. M. et al. Clinical correlates of quality of life in Tourette syndrome. Mov. Disord. 26, 735–738 (2011).
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.
Kurlan, R. et al. The behavioral spectrum of tic disorders: a community-based study. Neurology 59, 414–420 (2002).
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).
Zinner, S. H. & Coffey, B. J. Developmental and behavioral disorders grown up: Tourette's disorder. J. Dev. Behav. Pediatr. 30, 560–573 (2009).
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).
Bloch, M. H. & Leckman, J. F. Clinical course of Tourette syndrome. J. Psychosom. Res. 67, 497–501 (2009).
Jankovic, J., Gelineau-Kattner, R. & Davidson, A. Tourette's syndrome in adults. Mov. Disord. 25, 2171–2175 (2010).
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).
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).
Leckman, J. F., Bloch, M. H., Scahill, L. & King, R. A. Tourette syndrome: the self under siege. J. Child. Neurol. 21, 642–649 (2006).
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).
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).
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).
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).
Kurlan, R. et al. Prevalence of tics in schoolchildren and association with placement in special education. Neurology 57, 1383–1388 (2001).
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).
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).
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).
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).
State, M. W. The genetics of child psychiatric disorders: focus on autism and Tourette syndrome. Neuron 68, 254–269 (2010).
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).
Kidd, K. K., Prusoff, B. A. & Cohen, D. J. Familial pattern of Gilles de la Tourette syndrome. Arch. Gen. Psychiatry 37, 1336–1339 (1980).
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).
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).
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).
Kurlan, R., Eapen, V., Stern, J., McDermott, M. P. & Robertson, M. M. Bilineal transmission in Tourette's syndrome families. Neurology 44, 2336–2342 (1994).
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).
Abelson, J. F. et al. Sequence variants in SLITRK1 are associated with Tourette's syndrome. Science 310, 317–320 (2005).
Ercan-Sencicek, A. G. et al. L-histidine decarboxylase and Tourette's syndrome. N. Engl. J. Med. 362, 1901–1908 (2010).
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).
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).
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).
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).
Rickards, H. Functional neuroimaging in Tourette syndrome. J. Psychosom. Res. 67, 575–584 (2009).
Hyde, T. M. et al. Cerebral morphometric abnormalities in Tourette's syndrome: a quantitative MRI study of monozygotic twins. Neurology 45, 1176–1182 (1995).
Peterson, B. S. et al. Basal ganglia volumes in patients with Gilles de la Tourette syndrome. Arch. Gen. Psychiatry 60, 415–424 (2003).
Lee, J. S. Abnormal thalamic volume in treatment-naïve boys with Tourette syndrome. Acta Psychiatr. Scand. 113, 64–67 (2006).
Sowell, E. R. et al. Thinning of sensorimotor cortices in children with Tourette syndrome. Nat. Neurosci. 11, 637–639 (2008).
Peterson, B. S. et al. A functional magnetic resonance imaging study of tic suppression in Tourette syndrome. Arch. Gen. Psychiatry 55, 326–333 (1998).
Bohlhalter, S. et al. Neural correlates of tic generation in Tourette syndrome: an event-related functional MRI study. Brain 129, 2029–2037 (2006).
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).
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).
Temel, Y. & Visser-Vandewalle, V. Surgery in Tourette syndrome. Mov. Disord. 19, 3–14 (2004).
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).
Ackermans, L. et al. Deep brain stimulation in Tourette's syndrome: two targets? Mov. Disord. 21, 709–713 (2006).
Shale, H., Fahn, S. & Mayeux, R. Tics in a patient with Parkinson's disease. Mov. Disord. 1, 79–83 (1986).
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).
Singer, H. S. Treatment of tics and Tourette syndrome. Curr. Treat. Options Neurol. 12, 539–561 (2010).
Zinner, S. H. & Mink, J. W. Movement disorders I: tics and stereotypies. Pediatr. Rev. 31, 223–233 (2010).
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).
Piacentini, J. et al. Behavior therapy for children with Tourette disorder: a randomized controlled trial. JAMA. 303, 1929–1937 (2010).
Ward, H. E., Hwynn, N. & Okun, M. S. Update on deep brain stimulation for neuropsychiatric disorders. Neurobiol. Dis. 38, 346–353 (2010).
Shapiro, A. K. & Shapiro, E. Treatment of Gilles de la Tourette's syndrome with haloperidol. Br. J. Psychiatry 114, 345–350 (1968).
Kwak, C. H., Hanna, P. A. & Jankovic, J. Botulinum toxin in the treatment of tics. Arch. Neurol. 57, 1190–1193 (2000).
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).
Erenberg, G., Cruse, R. P. & Rothner, A. D. Gilles de la Tourette's syndrome: effects of stimulant drugs. Neurology 35, 1346–1348 (1985).
Tourette's Syndrome Study Group. Treatment of ADHD in children with tics: a randomized controlled trial. Neurology 58, 527–536 (2002).
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).
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).
Azrin, N. H. & Nunn, R. G. Habit-reversal. a method of eliminating nervous habits and tics. Behav. Res. Ther. 11, 619–628 (1973).
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).
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).
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).
Mink, J. W. et al. Patient selection and assessment recommendations for deep brain stimulation in Tourette syndrome. Mov. Disord. 21, 1831–1838 (2006).
Kompoliti, K., Fan, W. & Leurgans, S. Complementary and alternative medicine use in Gilles de la Tourette syndrome. Mov. Disord. 24, 2015–2019 (2009).
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).
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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
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DOI: https://doi.org/10.1038/nrneurol.2011.167
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