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Gilles de la Tourette's syndrome

(also see NINDS Tourette Syndrome Information Page) Etiology: - associated with psychiatric disease - associated with obsessive-compulsive disorder [1] - idiopathic Epidemiology: 1) rare 2) affected individuals may be of any level of intelligence 3) male:female ratio of 3:1 4) chilhood onset [1] Genetics: - associated with defects in IMMP2L gene - associated with defectes in SLITRK1 gene Clinical manifestations: 1) widespread motor & vocal tics* 2) behavorial abnormalities - echolalia - coprolalia (uncommon) 3) onset in childhood 4) frequently persists into early adulthood * diagnosis of Tourette's syndrome requires presence of motor & vocal tics for at least 1 year [1] Management: - reassurance often appropriate with mild disease [1] - cognitive behavioral therapy first line [4] - treatment of psychiatric comorbidities [1] - treatment of motor tics with: a) clonidine (1st line) b) clonazepam c) haloperidol (Haldol) d) chlorpromazine e) Botox - deep brain stimulation in severe cases [4]

Related

chlorpromazine (Thorazine, Ormazine, Propaphenin, Sonazine, Chloractil) clonazepam (Klonopin) clonidine (Catapres, Onyda XR) coprolalia (coprophrasia) haloperidol (Haldol, Halperon, Aloperidol, Halomonth)

General

chronic neurologic disease tic disorder syndrome

Database Correlations

OMIM 137580

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  2. DSM-IV
  3. Kurlan RM. Treatment of Tourette syndrome. Neurotherapeutics. 2014 Jan;11(1):161-5. PMID: 24043501 Free PMC Article
  4. Pringsheim T, Okun MS, Muller-Vahl K et al Practice guideline recommendations summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders. Neurology. May 07, 2019; 92 (19) Special Article PMID: 31061208 https://n.neurology.org/content/92/19/896 - Pringsheim T, Holler-Managan Y, Okun MS et al May 07, 2019; 92 (19) Special Article Comprehensive systematic review summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders. Neurology. May 07, 2019; 92 (19) Special Article PMID: 31061209 https://n.neurology.org/content/92/19/907
  5. NINDS Tourette Syndrome Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Tourette-Syndrome-Information-Page