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Tourette syndrome

Reviewed May 2008

What is Tourette syndrome?

Tourette syndrome is a complex disorder characterized by repetitive, sudden, and involuntary movements or noises called tics. Tics usually appear in childhood, and their severity varies over time. In most cases, tics become milder and less frequent in late adolescence and adulthood.

Tourette syndrome involves both motor tics, which are uncontrolled body movements, and vocal or phonic tics, which are outbursts of sound. Some motor tics are simple and involve only one muscle group. Simple motor tics, such as rapid eye blinking, shoulder shrugging, or nose twitching, are usually the first signs of Tourette syndrome. Motor tics also can be complex (involving multiple muscle groups), such as jumping, kicking, hopping, or spinning.

Vocal tics, which generally appear later than motor tics, also can be simple or complex. Simple vocal tics include grunting, sniffing, and throat-clearing. More complex vocalizations include repeating the words of others (echolalia) or repeating one's own words (palilalia). The involuntary use of inappropriate or obscene language (coprolalia) is possible, but uncommon, among people with Tourette syndrome.

In addition to frequent tics, people with Tourette syndrome are at risk for associated problems including attention deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), anxiety, depression, and problems with sleep.

How common is Tourette syndrome?

Tourette syndrome is a relatively common disorder. Although its exact incidence is uncertain, it is estimated to affect 1 to 10 in 1,000 children. This disorder occurs in populations and ethnic groups worldwide, and it is more common in males than in females.

What genes are related to Tourette syndrome?

A variety of genetic and environmental factors likely play a role in causing Tourette syndrome. Most of these factors are unknown, and researchers are studying risk factors before and after birth that may contribute to this complex disorder. Scientists believe that tics may result from changes in brain chemicals (neurotransmitters) that are responsible for producing and controlling voluntary movements.

Mutations involving the SLITRK1 gene have been identified in a small number of people with Tourette syndrome. This gene provides instructions for making a protein that is active in the brain. The SLITRK1 protein probably plays a role in the development of nerve cells, including the growth of specialized extensions (axons and dendrites) that allow each nerve cell to communicate with nearby cells. It is unclear how mutations in the SLITRK1 gene can lead to this disorder.

Most people with Tourette syndrome do not have a mutation in the SLITRK1 gene. Because mutations have been reported in so few people with this condition, the association of the SLITRK1 gene with this disorder has not been confirmed. Researchers suspect that changes in other genes, which have not been identified, are also associated with Tourette syndrome.

How do people inherit Tourette syndrome?

The inheritance pattern of Tourette syndrome is unclear. Although the features of this condition can cluster in families, many genetic and environmental factors are likely to be involved. Among family members of an affected person, it is difficult to predict who else may be at risk of developing the condition.

Tourette syndrome was previously thought to have an autosomal dominant pattern of inheritance, which suggests that one mutated copy of a gene in each cell would be sufficient to cause the condition. Several decades of research have shown that this is not the case. Almost all cases of Tourette syndrome probably result from a variety of genetic and environmental factors, not changes in a single gene.

Where can I find information about treatment for Tourette syndrome?

You may find information on treatment or management of Tourette syndrome or some of its symptoms in the links below, particularly the links for MedlinePlus Encyclopedia, Educational resources, and Patient support.

Where can I find additional information about Tourette syndrome?

You may find the following resources about Tourette syndrome helpful. These materials are written for the general public.

  • MedlinePlus - Health information
    • Encyclopedia: Gilles de la Tourette syndrome (http://www.nlm.nih.gov/medlineplus/ency/article/000733.htm)
    • Health Topic: Tourette Syndrome (http://www.nlm.nih.gov/medlineplus/tourettesyndrome.html)
  • Genetic and Rare Diseases Information Center - Information about genetic conditions and rare diseases (http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&DiseaseID=7783)
  • Additional NIH Resources - National Institutes of Health
    • National Institute for Neurological Disorders and Stroke: Scientists Discover First Gene for Tourette Syndrome (December 15, 2005) (http://www.ninds.nih.gov/news_and_events/news_articles/news_article_Tourette_gene_121505.htm)
    • National Institute of Neurological Disorders and Stroke: Tourette Syndrome Fact Sheet (http://www.ninds.nih.gov/disorders/tourette/detail_tourette.htm)
  • Educational resources - Information pages
    • Ask the Geneticist: Inheritance of Tourette syndrome (http://www.askthegen.org/question.php?question_id=153)
    • Ask the Geneticist: Management of Tourette syndrome (http://www.askthegen.org/question.php?question_id=232)
    • Cedars-Sinai Medical Center (http://www.cedars-sinai.edu/3011.html)
    • Children's Hospital Boston (http://www.childrenshospital.org/az/Site1761/mainpageS1761P0.html)
    • Cincinnati Children's Hospital Medical Center (http://www.cincinnatichildrens.org/health/info/neurology/diagnose/tics-tourette.htm)
    • Cleveland Clinic Health Information Center (http://my.clevelandclinic.org/disorders/Tourette_Syndrome/hic_Tourette_Syndrome.aspx)
    • Great Ormond Street Hospital for Children (UK) (http://www.gosh.nhs.uk/gosh_families/information_sheets/tourette_syndrome_introduction/tourette_syndrome_introduction_children.html)
    • KidsHealth from the Nemours Foundation (http://kidshealth.org/parent/medical/brain/tourette.html)
    • Madisons Foundation (http://www.madisonsfoundation.org/index.php/component/option,com_mpower/diseaseID,618/)
    • Mayo Clinic (http://www.mayoclinic.org/tourette-syndrome/)
    • Merck Manual of Medical Information, Home Edition for Patients and Caregivers (http://www.merck.com/mmhe/sec06/ch091/ch091h.html)
    • Netwellness: Tourette syndrome and attention deficit hyperactivity disorder (http://www.netwellness.org/question.cfm/1953.htm)
    • New York Online Access to Health (http://www.noah-health.org/en/bns/disorders/tourette/)
    • Orphanet (http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=856)
    • Penn State Children's Hospital (http://www.hmc.psu.edu/childrens/healthinfo/t/tourette.htm)
    • University of Virginia Health System (http://www.healthsystem.virginia.edu/uvahealth/adult_mentalhealth/tourette.cfm)
  • Patient support - For patients and families
    • Family Village (http://www.familyvillage.wisc.edu/lib_tour.htm)
    • National Alliance on Mental Illness (NAMI) (http://www.nami.org/Template.cfm?Section=By_Illness&Template=/TaggedPage/TaggedPageDisplay.cfm&TPLID=54&ContentID=23053)
    • National Organization for Rare Disorders (http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Tourette+Syndrome)
    • Tourette Syndrome Association (http://tsa-usa.org/)
    • Tourette Syndrome Foundation of Canada (http://www.tourette.ca/)
    • WE MOVE: Worldwide Education and Awareness for Movement Disorders (http://www.wemove.org/ts/)

You may also be interested in these resources, which are designed for healthcare professionals and researchers.

  • Gene Tests - DNA tests ordered by healthcare professionals (http://www.genetests.org/query?testid=293896)
  • ClinicalTrials.gov - Linking patients to medical research (http://clinicaltrials.gov/search/condition=%22Tourette+syndrome%22)
  • PubMed - Recent literature (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&DB=PubMed&term=(Tourette+Syndrome[MAJR])+AND+(Tourette+syndrome[TIAB])+AND+english[la]+AND+human[mh]&orig_db=PubMed&filters=ON&pmfilter_EDatLimit=720+Days)
  • OMIM - Genetic disorder catalog (http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=137580)

What other names do people use for Tourette syndrome?

  • Chronic Motor and Vocal Tic Disorder
  • Gilles de la Tourette's syndrome
  • Gilles de la Tourette Syndrome
  • GTS
  • TD
  • Tourette Disorder
  • Tourette's Disease
  • TS

See How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What if I still have specific questions about Tourette syndrome?

  • See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.
  • Ask the Genetic and Rare Diseases Information Center (http://rarediseases.info.nih.gov/GARD/).
  • Submit your question to Ask the Geneticist (http://www.askthegen.org/).

What glossary definitions help with understanding Tourette syndrome?

ADHD ; anxiety ; attention deficit hyperactivity disorder ; autoimmune ; autosomal ; autosomal dominant ; axons ; cell ; chronic ; coprolalia ; dendrite ; depression ; echolalia ; gene ; hyperactivity ; incidence ; infection ; inheritance ; inheritance pattern ; involuntary ; motor ; mutation ; nerve cell ; neurotransmitters ; obsessive-compulsive disorder ; OCD ; palilalia ; pattern of inheritance ; population ; protein ; risk factors ; sign ; syndrome ; tic

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (http://ghr.nlm.nih.gov/glossary).

References
  • Abelson JF, Kwan KY, O'Roak BJ, Baek DY, Stillman AA, Morgan TM, Mathews CA, Pauls DL, Rasin MR, Gunel M, Davis NR, Ercan-Sencicek AG, Guez DH, Spertus JA, Leckman JF, Dure LS 4th, Kurlan R, Singer HS, Gilbert DL, Farhi A, Louvi A, Lifton RP, Sestan N, State MW. Sequence variants in SLITRK1 are associated with Tourette's syndrome. Science. 2005 Oct 14;310(5746):317-20. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=16224024)
  • Albin RL, Mink JW. Recent advances in Tourette syndrome research. Trends Neurosci. 2006 Mar;29(3):175-82. Epub 2006 Jan 23. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=16430974)
  • Berardelli A, Curra A, Fabbrini G, Gilio F, Manfredi M. Pathophysiology of tics and Tourette syndrome. J Neurol. 2003 Jul;250(7):781-7. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=12883917)
  • Hoekstra PJ, Anderson GM, Limburg PC, Korf J, Kallenberg CG, Minderaa RB. Neurobiology and neuroimmunology of Tourette's syndrome: an update. Cell Mol Life Sci. 2004 Apr;61(7-8):886-98. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=15095010)
  • Jankovic J. Tourette's syndrome. N Engl J Med. 2001 Oct 18;345(16):1184-92. Review. No abstract available. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=11642235)
  • Keen-Kim D, Freimer NB. Genetics and epidemiology of Tourette syndrome. J Child Neurol. 2006 Aug;21(8):665-71. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=16970867)
  • Leckman JF, Bloch MH, Scahill L, King RA. Tourette syndrome: the self under siege. J Child Neurol. 2006 Aug;21(8):642-9. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=16970864)
  • Leckman JF. Tourette's syndrome. Lancet. 2002 Nov 16;360(9345):1577-86. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=12443611)
  • Robertson MM. Tourette syndrome, associated conditions and the complexities of treatment. Brain. 2000 Mar;123 Pt 3:425-62. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=10686169)
  • Singer HS. Tourette's syndrome: from behaviour to biology. Lancet Neurol. 2005 Mar;4(3):149-59. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=15721825)
  • Swain JE, Scahill L, Lombroso PJ, King RA, Leckman JF. Tourette syndrome and tic disorders: a decade of progress. J Am Acad Child Adolesc Psychiatry. 2007 Aug;46(8):947-68. Review. (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&dopt=Abstract&list_uids=17667475)

 

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

 
Reviewed: May 2008
Published: May 4, 2009