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Paeds Topicsneurology-neurodisability-and-neuromuscular

Paeds · neurology-neurodisability-and-neuromuscular

Tics and Tourette syndrome

Also known as Tourette disorder · Tourette syndrome · Chronic tic disorder · Provisional tic disorder · Persistent motor or vocal tic disorder · Childhood tic disorder

Fellowship guide to tics and Tourette syndrome: the DSM-5 spectrum from provisional to persistent tic disorder to Tourette disorder, the defining phenomenology of premonitory urge and suppressibility with waxing and waning, the cortico-striato-thalamo-cortical mechanism and the shared genetics with attention-deficit or hyperactivity disorder and obsessive-compulsive disorder, the high-yield comorbidity screen that drives most of the impairment, the stepped management built on Comprehensive Behavioural Intervention for Tics with alpha-2 agonists and antipsychotics, and the favourable prognosis that tics peak around 10 to 12 years and improve through adolescence.

high12 referencesUpdated 16 July 202626 min readVerification in progress

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Target exams

RACP General PaediatricsRACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General PediatricsRCPSC Pediatrics

Red flags

  • A tic with acute abrupt onset after a streptococcal illness, especially with obsessive-compulsive symptoms, suggests PANS or PANDAS and warrants specialist assessment
  • Onset after age 18, a fixed rather than fluctuating course, or a tic that never eases with distraction argues against a primary tic disorder and for a secondary cause
  • A focal neurological deficit, regression, or a static abnormality on examination is not a primary tic disorder and demands neuroimaging
  • Self-injurious tics, head-banging, or severe rage attacks risk real physical or psychosocial harm and need urgent behavioural and pharmacological management
  • Drug-induced tics after starting a stimulant, an antipsychotic, or a dopamine agent are a secondary cause and demand a careful medication review
  • Obsessive-compulsive disorder, anxiety, or attention-deficit or hyperactivity disorder that is untreated cause more impairment than the tics themselves and must be sought at every visit

Life stages

preschoolschool-ageadolescentyoung-adult-transition

Care settings

preventive-medical-homecommunity-schooloutpatienttelehealth

Clinical exam formats

written-only

Board mappings

  • Applies DSM-5 criteria to distinguish Tourette disorder from provisional and persistent tic disorders
  • Recognises that attention-deficit or hyperactivity disorder and obsessive-compulsive disorder drive most impairment
  • Initiates psychoeducation and reassurance for mild tics and refers for CBIT when tics cause impairment
  • Justifies the stepped pharmacological ladder from alpha-2 agonists to antipsychotics with safety monitoring
  • Distinguishes tics from stereotypies, myoclonus, chorea, and functional tics at the bedside
  • Manages Tourette with comorbid attention-deficit or hyperactivity disorder or obsessive-compulsive disorder and counsels that stimulants do not routinely worsen tics
  • DSM-5 criteria for Tourette disorder and the provisional-versus-persistent tic disorder distinction
  • The premonitory urge, suppressibility, and waxing-and-waning phenomenology that define a tic
  • The stepped management ladder and the role of CBIT, alpha-2 agonists, antipsychotics, and botulinum toxin
  • Takes a focussed tic history including home video and a deliberate comorbidity screen
  • Communicates the reassuring prognosis and that treatment targets impairment not tic elimination
  • Coordinates multidisciplinary care for the comorbidities that determine quality of life
  • Level 1: Recognition of tics and Tourette syndrome and exclusion of secondary causes
  • Level 2: Psychoeducation, referral for CBIT, and first-line pharmacological options
  • Level 3: Management of complex Tourette with comorbid neurodevelopmental and psychiatric conditions
  • DSM-5 criteria for Tourette disorder and the phenomenology that distinguishes tics from mimics
  • Evidence base for CBIT and the stepped pharmacological treatment of tics
  • The high prevalence of attention-deficit or hyperactivity disorder and obsessive-compulsive disorder comorbidity
  • Assessment of the child with tics including home video and a comorbidity-focused history
  • Reassurance and the stepped management plan explained to an anxious family
  • Recognition of when to investigate and when to refer to paediatric neurology or psychiatry
  • American Academy of Neurology 2019 guideline for the treatment of tics in Tourette syndrome
  • CBIT as first-line behavioural treatment and the role of alpha-2 agonists and antipsychotics
  • Recognition and management of comorbid attention-deficit or hyperactivity disorder and obsessive-compulsive disorder
  • Recognition of tics as common, fluctuating, and usually benign, with a clinical diagnosis
  • Stepped management built on psychoeducation, CBIT, and risk-stratified pharmacotherapy
  • Family communication around the favourable prognosis and the central importance of comorbidities
  • Canadian approach to the clinical diagnosis of Tourette syndrome and the restrained use of investigation
  • CBIT and the stepped pharmacological ladder informed by American Academy of Neurology and European guidelines
  • Counselling on the natural history of tic improvement and the parallel treatment of comorbidities
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Practise this topic

  • Short-answer question1
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Target exams

RACP General PaediatricsRACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General PediatricsRCPSC Pediatrics

Red flags

  • A tic with acute abrupt onset after a streptococcal illness, especially with obsessive-compulsive symptoms, suggests PANS or PANDAS and warrants specialist assessment
  • Onset after age 18, a fixed rather than fluctuating course, or a tic that never eases with distraction argues against a primary tic disorder and for a secondary cause
  • A focal neurological deficit, regression, or a static abnormality on examination is not a primary tic disorder and demands neuroimaging
  • Self-injurious tics, head-banging, or severe rage attacks risk real physical or psychosocial harm and need urgent behavioural and pharmacological management
  • Drug-induced tics after starting a stimulant, an antipsychotic, or a dopamine agent are a secondary cause and demand a careful medication review
  • Obsessive-compulsive disorder, anxiety, or attention-deficit or hyperactivity disorder that is untreated cause more impairment than the tics themselves and must be sought at every visit

Life stages

preschoolschool-ageadolescentyoung-adult-transition

Care settings

preventive-medical-homecommunity-schooloutpatienttelehealth

Clinical exam formats

written-only

Board mappings

  • Applies DSM-5 criteria to distinguish Tourette disorder from provisional and persistent tic disorders
  • Recognises that attention-deficit or hyperactivity disorder and obsessive-compulsive disorder drive most impairment
  • Initiates psychoeducation and reassurance for mild tics and refers for CBIT when tics cause impairment
  • Justifies the stepped pharmacological ladder from alpha-2 agonists to antipsychotics with safety monitoring
  • Distinguishes tics from stereotypies, myoclonus, chorea, and functional tics at the bedside
  • Manages Tourette with comorbid attention-deficit or hyperactivity disorder or obsessive-compulsive disorder and counsels that stimulants do not routinely worsen tics
  • DSM-5 criteria for Tourette disorder and the provisional-versus-persistent tic disorder distinction
  • The premonitory urge, suppressibility, and waxing-and-waning phenomenology that define a tic
  • The stepped management ladder and the role of CBIT, alpha-2 agonists, antipsychotics, and botulinum toxin
  • Takes a focussed tic history including home video and a deliberate comorbidity screen
  • Communicates the reassuring prognosis and that treatment targets impairment not tic elimination
  • Coordinates multidisciplinary care for the comorbidities that determine quality of life
  • Level 1: Recognition of tics and Tourette syndrome and exclusion of secondary causes
  • Level 2: Psychoeducation, referral for CBIT, and first-line pharmacological options
  • Level 3: Management of complex Tourette with comorbid neurodevelopmental and psychiatric conditions
  • DSM-5 criteria for Tourette disorder and the phenomenology that distinguishes tics from mimics
  • Evidence base for CBIT and the stepped pharmacological treatment of tics
  • The high prevalence of attention-deficit or hyperactivity disorder and obsessive-compulsive disorder comorbidity
  • Assessment of the child with tics including home video and a comorbidity-focused history
  • Reassurance and the stepped management plan explained to an anxious family
  • Recognition of when to investigate and when to refer to paediatric neurology or psychiatry
  • American Academy of Neurology 2019 guideline for the treatment of tics in Tourette syndrome
  • CBIT as first-line behavioural treatment and the role of alpha-2 agonists and antipsychotics
  • Recognition and management of comorbid attention-deficit or hyperactivity disorder and obsessive-compulsive disorder
  • Recognition of tics as common, fluctuating, and usually benign, with a clinical diagnosis
  • Stepped management built on psychoeducation, CBIT, and risk-stratified pharmacotherapy
  • Family communication around the favourable prognosis and the central importance of comorbidities
  • Canadian approach to the clinical diagnosis of Tourette syndrome and the restrained use of investigation
  • CBIT and the stepped pharmacological ladder informed by American Academy of Neurology and European guidelines
  • Counselling on the natural history of tic improvement and the parallel treatment of comorbidities
The fellowship answer

Tics are sudden, rapid, recurrent, non-rhythmic movements or sounds that are briefly suppressible, preceded by a premonitory urge, and followed by transient relief, and they wax and wane and shift body location over time. Tourette disorder is the severe end of a DSM-5 spectrum defined by duration and by the coexistence of motor and vocal tics: it requires multiple motor tics plus at least one vocal tic, present for over one year, with onset before age 18. Tics are common, affect roughly one per cent of schoolchildren, and are two to four times more frequent in boys.

[2][7]

The diagnosis is entirely clinical, made by history and by home video, and there is no blood test or scan for Tourette syndrome. What decides management is not the tics themselves but their impairment, and the impairment is driven mainly by the comorbidities, attention-deficit or hyperactivity disorder in about 60 per cent and obsessive-compulsive disorder in about 30 per cent. [6]

Management is stepped. Mild tics need only psychoeducation and reassurance. When tics cause impairment, the first active treatment is Comprehensive Behavioural Intervention for Tics, or CBIT, built on habit reversal training, which the Piacentini trial proved reduces tic severity. [1] If a drug is needed, alpha-2 agonists such as clonidine or guanfacine are preferred first, especially when attention-deficit or hyperactivity disorder coexists, and antipsychotics such as risperidone or aripiprazole are the strongest tic suppressants but need safety monitoring. The prognosis is good: tics peak around 10 to 12 years and improve for most children through adolescence, and treatment aims at function, never at tic elimination. [2] [11]

Overview & Definition

Picture the seven-year-old boy whose teacher reports he cannot stop blinking, throat-clearing, and jerking his neck, whose parents notice these movements vanish when he plays a video game and surge when he is tired or anxious, and who is now being asked whether he needs a brain scan and a pill. That child carries the whole teaching point of paediatric tics: the diagnosis is clinical, the prognosis is reassuring, and the thing that most needs treating is usually not the movement at all but the attention-deficit, the anxiety, or the obsessive-compulsive symptom riding alongside it. The clinician who grasps this triage avoids two opposite errors, over-medicalising a benign fluctuating movement and missing the comorbidity that is silently harming the child. [2]

A tic is defined by four phenomenological features rather than by any single movement. It is sudden, rapid, recurrent, and non-rhythmic. It is briefly suppressible, so a child can hold it off for a short time. It is preceded by a premonitory urge, a mounting sensory tension that builds until the tic is performed. And performing the tic brings transient relief before the cycle restarts. Movements that lack the urge, the suppressibility, and the relief are usually not tics, and these four features are what separate a tic from a stereotypy, a myoclonic jerk, a choreiform movement, or a habitual mannerism. [2]

The tics themselves divide into motor and vocal, and each divides again into simple and complex. Simple motor tics involve one muscle group, such as eye-blinking, facial grimacing, shoulder-shrugging, or head-jerking, while complex motor tics are coordinated sequences such as jumping, touching, or copropraxia, an obscene gesture. Simple vocal tics are sounds such as sniffing, grunting, throat-clearing, or coughing, while complex vocal tics include words, echolalia repeating another's words, palilalia repeating one's own words, and coprolalia, the involuntary uttering of socially inappropriate words. The famous feature, coprolalia, is in fact uncommon and is never required to make the diagnosis. [2]

Across Australia and New Zealand, practice follows the Royal Children's Hospital Melbourne approach to tics and Tourette syndrome, the American Academy of Neurology 2019 guideline, and the European clinical guidelines, all of which converge on a clinical diagnosis, a comorbidity-led assessment, and a stepped plan that begins with reassurance and Comprehensive Behavioural Intervention for Tics before pharmacotherapy. Rural and remote presentations rely on telehealth paediatric neurology and a primary-care-led stepped plan, with school accommodation delivered through the child's learning-support team.
[2] [5]
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References12ShowHide
  1. [1]Piacentini J, Woods DW, Scahill L, et al Behavior therapy for children with Tourette disorder: a randomized controlled trial. JAMA, 2010.PMID 20483969
  2. [2]Pringsheim T, Okun MS, Müller-Vahl K, et al. Practice guideline recommendations summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders. Neurology, 2019.PMID 31061208
  3. [3]Pringsheim T, Holler-Managan Y, Okun MS, et al Comprehensive systematic review summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders. Neurology, 2019.PMID 31061209
  4. [4]Andrén P, Jakubovski E, Murphy TL, et al. European clinical guidelines for Tourette syndrome and other tic disorders-version 2.0. Part II: psychological interventions. Eur Child Adolesc Psychiatry, 2022.PMID 34313861
  5. [5]Roessner V, Eichele H, Stern JS, et al European clinical guidelines for Tourette syndrome and other tic disorders-version 2.0. Part III: pharmacological treatment. Eur Child Adolesc Psychiatry, 2022.PMID 34757514
  6. [6]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.PMID 25671412
  7. [7]Khalifa N, von Knorring AL Prevalence of tic disorders and Tourette syndrome in a Swedish school population. Dev Med Child Neurol, 2003.PMID 12729145
  8. [8]Chang K, Frankovich J, Cooperstock M, et al Clinical evaluation of youth with pediatric acute-onset neuropsychiatric syndrome (PANS): recommendations from the 2013 PANS Consensus Conference. J Child Adolesc Psychopharmacol, 2015.PMID 25325534
  9. [9]Leckman JF, Riddle MA, Hardin MT, et al The Yale Global Tic Severity Scale: initial testing of a clinician-rated scale of tic severity. J Am Acad Child Adolesc Psychiatry, 1989.PMID 2768151
  10. [10]Marras C, Andrews D, Sime E, Lang AE Botulinum toxin for simple motor tics: a randomized, double-blind, controlled clinical trial. Neurology, 2001.PMID 11245710
  11. [11]Bloch MH, Peterson BS, Scahill L, et al Adulthood outcome of tic and obsessive-compulsive symptom severity in children with Tourette syndrome. Arch Pediatr Adolesc Med, 2006.PMID 16389213
  12. [12]Hirschtritt ME, Darrow SM, Illmann C, et al Genetic and phenotypic overlap of specific obsessive-compulsive and attention-deficit or hyperactive subtypes with Tourette syndrome. Psychol Med, 2018.PMID 28651666

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