• Clinical science

Tourette syndrome

Summary

Tourette syndrome is a severe neurological movement disorder characterized by tics, which are involuntary, repeated, intermittent movements, or vocalizations. It is a genetic disorder that commonly presents in boys and is often associated with attention deficit hyperactivity disorder (ADHD) or obsessive-compulsive disorder (OCD). Diagnosis is based upon multiple motor tics and at least one vocal tic, lasting for longer than a year, and the exclusion of other suspected medical conditions. Treatment is symptomatic and includes behavioral therapy and dopamine antagonists. Approximately 50% of cases resolve by adulthood.

Epidemiology

  • Sex: > [1]
  • Age of onset: usually 4–6 years of age (tics are most severe between 10–12 years of age and then decline during adolescence) [2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

Clinical features

  • Tics: sudden and rapid involuntary, intermittent, nonrhythmic movements or vocalizations without any recognizable purpose
    • Temporarily suppressible
    • An urge or sensation preceding the tic is relieved by its onset.
Simple Complex
Vocal tics
  • Throat clearing
  • Grunting
  • Lip smacking
  • Barking
  • Sniffing
  • Coprolalia: uttering obscene or socially inappropriate words or phrases
  • Echolalia: repeating vocalizations of others
Motor tics
  • Facial grimacing
  • Blinking
  • Shoulder shrugging
  • Head jerking
  • Jumping
  • Twisting the body
  • Echopraxia: repeating movements of others

Diagnostics

Tourette syndrome is a clinical diagnosis based on all of the following:

  • Multiple motor tics and at least 1 vocal tic with a variable anatomical location, frequency, number, complexity, type, or severity over time
  • Onset before 18 years of age
  • Lasting > 1 year
  • Not explained by other medical conditions or substance use (e.g., cocaine) [3]

Differential diagnoses

  • Other tic disorders [4]
    • Sporadic transient tic disorder: a subtype of tic disorder particularly common in children, which manifests with motor or vocal tics that occur over a period of less than one year before resolving.
      • Motor and/or vocal tics
      • Age of onset: < 18 years
      • Symptoms occur for less than 1 year (resolve spontaneously)
      • Not explained by any other medical conditions or substance abuse
      • Do not meet the criteria for Tourette syndrome
    • Persistent motor tic disorder or persistent vocal tic disorder are subtypes of tic disorder particularly common in children, which manifests with motor or phonic tics only.
      • Motor or vocal tics (not both)
      • Age of onset: < 18 years
      • Persist > 1 year
      • Not explained by any other medical conditions or substance abuse
      • Do not meet the criteria for Tourette syndrome
  • Stereotypic movement disorder
    • Stereotypic, uncontrolled, repetitive movements lasting ≥ 4 weeks
    • More common in children, especially boys, with neurological disorders and developmental retardation
    • Includes rocking movements, hair pulling, hair twisting, and self-destructive behavior (e.g., head banging, picking at skin, hitting oneself) [5][6]
  • Tardive dyskinesia
  • Huntington disease

The differential diagnoses listed here are not exhaustive.

Treatment

Treatment of associated conditions (i.e., ADHD or OCD) can improve symptoms.

Prognosis

  • Symptoms improve during adolescence. [2]
  • May resolve spontaneously by 18 years of age (50% of cases) [10]
  • 1. Du JC, Chiu TF, Lee KM, et al. Tourette syndrome in children: an updated review. Pediatrics and neonatology. 2010; 51(5): pp. 255–64. doi: 10.1016/S1875-9572(10)60050-2.
  • 2. Bloch MH, Leckman JF. Clinical course of Tourette syndrome. J Psychosom Res. 2009; 67(6): pp. 497–501. doi: 10.1016/j.jpsychores.2009.09.002.
  • 3. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. DSM library; 2013.
  • 4. Jones KS, Ramphul K. Tourette Syndrome And Other Tic Disorders. StatPearls. 2020. pmid: 29763133.
  • 5. Ollendick TH, Hersen M. Handbook of Child Psychopathology. New York, NY: Springer Science & Business Media; 2013.
  • 6. Freeman RD, Soltanifair A, Baer S. Stereotypic movement disorder: easily missed. Dev Med Child Neurol. 2010; 52(8): pp. 733–738. doi: 10.1111/j.1469-8749.2010.03627.x.
  • 7. Tourette Association of America . Pharmacology. https://www.tourette.org/research-medical/pharmacology/. Accessed May 15, 2017.
  • 8. Thenganatt MA, Jankovic J. Recent advances in understanding and managing tourette syndrome. F1000Research. 2016; 5: p. 152. doi: 10.12688/f1000research.7424.1.
  • 9. Savica R, Stead M, Mack KJ, Lee KH, Klassen BT. Deep brain stimulation in tourette syndrome: a description of 3 patients with excellent outcome. Mayo Clinic proceedings. 2012; 87(1): pp. 59–62. doi: 10.1016/j.mayocp.2011.08.005.
  • 10. Leckman JF, Zhang H, Vitale A, et al. Course of tic severity in Tourette syndrome: the first two decades. Pediatrics. 1998; 102(1 Pt 1): pp. 14–9. doi: 10.1542/peds.102.1.14.
last updated 11/03/2020
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