Paeds · neurology-neurodisability-and-neuromuscular

Movement disorders, dystonia and chorea

Also known as Paediatric hyperkinetic movement disorders · Dystonia in children · Chorea in children · Status dystonicus · Sydenham chorea · Dyskinetic cerebral palsy

Fellowship guide to paediatric movement disorders. Defines the hyperkinetic movements of chorea, dystonia, myoclonus, tics and tremor against the hypokinetic pole of parkinsonism, applies the Albanese 2013 two-axis dystonia classification and the Sanger 2003 childhood hypertonia framework, traces the basal ganglia direct and indirect pathway mechanism of dyskinesia, and works through Sydenham chorea as a major Jones criterion needing secondary penicillin prophylaxis, dopa-responsive dystonia and its dramatic levodopa response, Wilson disease screening, glutaric aciduria type 1, Lesch-Nyhan, the iatrogenic acute dystonic reversal with an anticholinergic, the status dystonicus escalation ladder from trigger control through oral drugs to sedation and intrathecal baclofen or deep brain stimulation, the dystonic cerebral palsy ladder of trihexyphenidyl, botulinum toxin, intrathecal baclofen and selective dorsal rhizotomy, the PANDAS and PANS controversy, regional rheumatic fever differences, and the family communication and long-term functional outlook.

high12 referencesUpdated 16 July 202622 min readVerification in progress

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Red flags

A child with escalating, painful, sustained dystonic spasms has status dystonicus and needs immediate triage to intensive care with creatine kinase and rhabdomyolysis surveillance, trigger identification, and escalating anti-dystonia therapyAcute onset of oculogyric crisis, torticollis, or oromandibular spasm within hours of a dopamine-blocking drug such as metoclopramide or an antipsychotic is an acute dystonic reaction that is rapidly reversed by an anticholinergicEvery dystonia of unknown cause in a child must have an empiric low-dose levodopa trial because dopa-responsive dystonia is common, dramatic, and entirely treatableAny child with new chorea or dystonia of unknown cause must be screened for Wilson disease with serum copper, caeruloplasmin, 24-hour urinary copper, and slit-lamp examination, because delayed treatment causes irreversible neurologic injurySydenham chorea is a major criterion of acute rheumatic fever and demands a cardiac assessment, an echocardiogram, and secondary penicillin prophylaxisA child with dystonia plus hepatic, psychiatric, or behavioural change, or a movement disorder that fluctuates and is distractible, may have Wilson disease or a functional movement disorder rather than primary psychiatric disease

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infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

outpatientwarded-acutepicuretrievaltelehealth

Clinical exam formats

written-onlyracp-dce-long-caseracp-dce-short-casemrcpch-short-clinicalmrcpch-history-managementmrcpch-communicationrcpsc-structured-oral

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Defines the hyperkinetic movement disorders seen in children (chorea, dystonia, myoclonus, tics, tremor) and distinguishes a movement disorder from a seizure or a behavioural ticRecognises Sydenham chorea as a major criterion of acute rheumatic fever requiring secondary penicillin prophylaxis and cardiac assessmentIdentifies status dystonicus as a life-threatening emergency needing triage to intensive care, trigger identification, and escalating anti-dystonia therapyApplies the Albanese 2013 two-axis dystonia classification, with clinical characteristics on axis I and aetiology on axis II, to localise the problem and direct the workupJustifies the empirical levodopa trial in any child with dystonia of unknown cause because dopa-responsive dystonia is dramatic and treatable, with the inherited and acquired workup proceeding in parallelRuns the status dystonicus escalation ladder from trigger identification through oral anti-dystonia drugs to sedation and anaesthesia to intrathecal baclofen or deep brain stimulation, with creatine kinase and rhabdomyolysis surveillanceThe five hyperkinetic movement definitions of chorea, dystonia, myoclonus, tics, and tremor and the hypokinetic contrast of parkinsonismThe status dystonicus ladder and the acute dystonic reaction reversal with an anticholinergic such as benztropine, procyclidine, or diphenhydramineSydenham chorea treatment with symptomatic, immunomodulatory, and penicillin prophylaxis options and the weight-based secondary prevention dosing of benzathine penicillin GAssesses and triages a child with acute or subacute abnormal movements, distinguishes a movement disorder from seizure and functional disorder, and orders the targeted workupManages status dystonicus with escalating therapy and coordinates intensive care, genetics, and neurosurgery input for refractory diseaseCounsels the family of a child with dystonic cerebral palsy on the oral, intrathecal baclofen, botulinum toxin, and selective dorsal rhizotomy options and the long-term functional outlookLevel 1: Recognition of common paediatric movement disorders and their distinction from seizures and behavioural phenomenaLevel 2: Classification of dystonia by axis I and II, the targeted investigation of chorea and dystonia, and the management of acute dystonic reaction and status dystonicusLevel 3: Multidisciplinary longitudinal management of dystonic cerebral palsy, selection of patients for intrathecal baclofen and deep brain stimulation, and transition planningThe definitions and causes of chorea and dystonia in children including Sydenham chorea, cerebral palsy, dopa-responsive dystonia, Wilson disease, and glutaric aciduria type 1The differential diagnosis of acute dystonic reaction from metoclopramide and antipsychotics and its reversal with an anticholinergicThe status dystonicus emergency and the escalation ladder including the role of intrathecal baclofen and deep brain stimulationStructured assessment of a child with abnormal movements, with age-appropriate examination and differentiation of organic from functional disorderRecognition and emergency management of status dystonicus and acute dystonic reaction, and the workup of a first presentation of chorea or dystoniaCommunication with the family of a child with a chronic dystonia including the treatment ladder, prognosis, and the role of multidisciplinary rehabilitationDefinitions and causes of chorea and dystonia in children and their distinction from seizures, myoclonus, and ticsAcute management of the dystonia emergencies with acute dystonic reversal by anticholinergic and status dystonicus escalationSydenham chorea as a Jones major criterion with secondary penicillin prophylaxis and cardiac evaluationRecognition and classification of paediatric movement disorders and identification of treatable causes such as dopa-responsive dystonia and Wilson diseaseStepwise management of status dystonicus from trigger control through sedation to neuromodulationMultidisciplinary care of dystonic cerebral palsy with pharmacological, intrathecal, and neurosurgical options and long-term functional surveillanceCanadian approach to the classification and investigation of childhood chorea and dystonia including the empiric levodopa trialStatus dystonicus management with the consensus escalation ladder and rhabdomyolysis surveillanceLongitudinal management of dystonic cerebral palsy and selection of children for intrathecal baclofen and deep brain stimulation

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Saved locally on this device.

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Target exams

RACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General Pediatrics

Red flags

Life stages

infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

outpatientwarded-acutepicuretrievaltelehealth

Clinical exam formats

written-onlyracp-dce-long-caseracp-dce-short-casemrcpch-short-clinicalmrcpch-history-managementmrcpch-communicationrcpsc-structured-oral

Board mappings

Overview & Definition

A child whose hand twists into a fixed posture when they reach for a cup, or who flows with restless, dance-like movements they cannot stop, is showing you a movement disorder. The job in paediatric neurology is to name the movement, work out whether it is a symptom of a treatable disease, and protect the child from the two emergencies that can kill or maim: status dystonicus and the acute dystonic reaction. Movement disorders are conditions in which the motor control system produces either an excess of movement that the child cannot suppress, or a poverty of movement with stiffness and slowness. [3]

The vocabulary is small and worth learning precisely, because the wrong word sends the workup in the wrong direction. Chorea is flowing, unpredictable, semi-purposeful movement. Dystonia is sustained or intermittent involuntary muscle contraction that twists the body into abnormal postures. Athetosis is the slow writhing form, and myoclonus is a brief, sudden, shock-like jerk. Tics are brief, stereotyped, and suppressible movements or sounds. Tremor is a rhythmic oscillation. The opposite pole is parkinsonism, with slowness, rigidity, and a rest tremor. The Albanese 2013 consensus update gives dystonia its modern two-axis classification, and the Sanger 2003 task force gave childhood hypertonia its working framework of spasticity, dystonia, and rigidity that dominates cerebral palsy practice. [1][2]

Three ideas hold this topic together. The movement is named by watching it, not by scanning it, so careful observation and video come before the test list. A single treatable cause, dopa-responsive dystonia, hides among the chronic dystonias and is found only by trialling levodopa in every unexplained case. And the two emergencies, the acute dystonic reaction and status dystonicus, are reversed or controlled by a short, memorable ladder that the exam will reward you for knowing in order. [3][6]

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You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References12Show ledger

[1]Albanese A, Bhatia K, Bressman SB, et al Phenomenology and classification of dystonia: a consensus update. Mov Disord, 2013.PMID 23649720
[2]Sanger TD, Delgado MR, Gaebler-Spira D, Hallett M, Mink JW Classification and definition of disorders causing hypertonia in childhood. Pediatrics, 2003.PMID 12509602
[3]Sanger TD Pediatric movement disorders. Curr Opin Neurol, 2003.PMID 12869814
[4]Gewitz MH, Baltimore RS, Tani LY, et al Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation, 2015.PMID 25908771
[5]Dean SL, Singer HS Treatment of Sydenham's Chorea: A Review of the Current Evidence. Tremor Other Hyperkinet Mov (N Y), 2017.PMID 28589057
[6]Allen NM, Lin JP, Lynch T, King MD Status dystonicus: a practice guide. Dev Med Child Neurol, 2014.PMID 24304390
[7]Vogt LM, Yang K, Tse G, et al Recommendations for the Management of Initial and Refractory Pediatric Status Dystonicus. Mov Disord, 2024.PMID 38619077
[8]Harvey AR, Baker LB, Reddihough DS, Scheinberg A, Williams K Trihexyphenidyl for dystonia in cerebral palsy. Cochrane Database Syst Rev, 2018.PMID 29763510
[9]Bohn E, Goren K, Switzer L, Falck-Ytter Y, Fehlings D Pharmacological and neurosurgical interventions for individuals with cerebral palsy and dystonia: a systematic review update and meta-analysis. Dev Med Child Neurol, 2021.PMID 33772789
[10]Fong AT, Jacob JG, Carroll B, Fernando SL Acute dystonic reaction mimicking angioedema secondary to metoclopramide ingestion. Emerg Med Australas, 2021.PMID 33873238
[11]Wilbur C, Bitnun A, Kronenberg S, Laxer RM, Levy DM, Logan WJ, et al PANDAS/PANS in childhood: Controversies and evidence. Paediatr Child Health, 2019.PMID 30996598
[12]Segawa M Dopa-responsive dystonia. Handb Clin Neurol, 2011.PMID 21496606

Movement disorders, dystonia and chorea

Also known as Paediatric hyperkinetic movement disorders · Dystonia in children · Chorea in children · Status dystonicus · Sydenham chorea · Dyskinetic cerebral palsy

high12 referencesUpdated 16 July 202622 min readVerification in progress

Practise this topic

Overview & Definition

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References12Show ledger

[1]Albanese A, Bhatia K, Bressman SB, et al Phenomenology and classification of dystonia: a consensus update. Mov Disord, 2013.PMID 23649720
[2]Sanger TD, Delgado MR, Gaebler-Spira D, Hallett M, Mink JW Classification and definition of disorders causing hypertonia in childhood. Pediatrics, 2003.PMID 12509602
[3]Sanger TD Pediatric movement disorders. Curr Opin Neurol, 2003.PMID 12869814
[4]Gewitz MH, Baltimore RS, Tani LY, et al Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation, 2015.PMID 25908771
[5]Dean SL, Singer HS Treatment of Sydenham's Chorea: A Review of the Current Evidence. Tremor Other Hyperkinet Mov (N Y), 2017.PMID 28589057
[6]Allen NM, Lin JP, Lynch T, King MD Status dystonicus: a practice guide. Dev Med Child Neurol, 2014.PMID 24304390
[7]Vogt LM, Yang K, Tse G, et al Recommendations for the Management of Initial and Refractory Pediatric Status Dystonicus. Mov Disord, 2024.PMID 38619077
[8]Harvey AR, Baker LB, Reddihough DS, Scheinberg A, Williams K Trihexyphenidyl for dystonia in cerebral palsy. Cochrane Database Syst Rev, 2018.PMID 29763510
[9]Bohn E, Goren K, Switzer L, Falck-Ytter Y, Fehlings D Pharmacological and neurosurgical interventions for individuals with cerebral palsy and dystonia: a systematic review update and meta-analysis. Dev Med Child Neurol, 2021.PMID 33772789
[10]Fong AT, Jacob JG, Carroll B, Fernando SL Acute dystonic reaction mimicking angioedema secondary to metoclopramide ingestion. Emerg Med Australas, 2021.PMID 33873238
[11]Wilbur C, Bitnun A, Kronenberg S, Laxer RM, Levy DM, Logan WJ, et al PANDAS/PANS in childhood: Controversies and evidence. Paediatr Child Health, 2019.PMID 30996598
[12]Segawa M Dopa-responsive dystonia. Handb Clin Neurol, 2011.PMID 21496606