Movement disorders, dystonia and chorea

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Target exams

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 therapy
• Acute 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 anticholinergic
• Every dystonia of unknown cause in a child must have an empiric low-dose levodopa trial because dopa-responsive dystonia is common, dramatic, and entirely treatable
• Any 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 injury
• Sydenham chorea is a major criterion of acute rheumatic fever and demands a cardiac assessment, an echocardiogram, and secondary penicillin prophylaxis
• A 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

Life stages

Care settings

Clinical exam formats

Board mappings

• Defines the hyperkinetic movement disorders seen in children (chorea, dystonia, myoclonus, tics, tremor) and distinguishes a movement disorder from a seizure or a behavioural tic
• Recognises Sydenham chorea as a major criterion of acute rheumatic fever requiring secondary penicillin prophylaxis and cardiac assessment
• Identifies status dystonicus as a life-threatening emergency needing triage to intensive care, trigger identification, and escalating anti-dystonia therapy
• Applies 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 workup
• Justifies 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 parallel
• Runs 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 surveillance
• The five hyperkinetic movement definitions of chorea, dystonia, myoclonus, tics, and tremor and the hypokinetic contrast of parkinsonism
• The status dystonicus ladder and the acute dystonic reaction reversal with an anticholinergic such as benztropine, procyclidine, or diphenhydramine
• Sydenham chorea treatment with symptomatic, immunomodulatory, and penicillin prophylaxis options and the weight-based secondary prevention dosing of benzathine penicillin G
• Assesses and triages a child with acute or subacute abnormal movements, distinguishes a movement disorder from seizure and functional disorder, and orders the targeted workup
• Manages status dystonicus with escalating therapy and coordinates intensive care, genetics, and neurosurgery input for refractory disease
• Counsels 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 outlook
• Level 1: Recognition of common paediatric movement disorders and their distinction from seizures and behavioural phenomena
• Level 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 dystonicus
• Level 3: Multidisciplinary longitudinal management of dystonic cerebral palsy, selection of patients for intrathecal baclofen and deep brain stimulation, and transition planning
• The definitions and causes of chorea and dystonia in children including Sydenham chorea, cerebral palsy, dopa-responsive dystonia, Wilson disease, and glutaric aciduria type 1
• The differential diagnosis of acute dystonic reaction from metoclopramide and antipsychotics and its reversal with an anticholinergic
• The status dystonicus emergency and the escalation ladder including the role of intrathecal baclofen and deep brain stimulation
• Structured assessment of a child with abnormal movements, with age-appropriate examination and differentiation of organic from functional disorder
• Recognition and emergency management of status dystonicus and acute dystonic reaction, and the workup of a first presentation of chorea or dystonia
• Communication with the family of a child with a chronic dystonia including the treatment ladder, prognosis, and the role of multidisciplinary rehabilitation
• Definitions and causes of chorea and dystonia in children and their distinction from seizures, myoclonus, and tics
• Acute management of the dystonia emergencies with acute dystonic reversal by anticholinergic and status dystonicus escalation
• Sydenham chorea as a Jones major criterion with secondary penicillin prophylaxis and cardiac evaluation
• Recognition and classification of paediatric movement disorders and identification of treatable causes such as dopa-responsive dystonia and Wilson disease
• Stepwise management of status dystonicus from trigger control through sedation to neuromodulation
• Multidisciplinary care of dystonic cerebral palsy with pharmacological, intrathecal, and neurosurgical options and long-term functional surveillance
• Canadian approach to the classification and investigation of childhood chorea and dystonia including the empiric levodopa trial
• Status dystonicus management with the consensus escalation ladder and rhabdomyolysis surveillance
• Longitudinal management of dystonic cerebral palsy and selection of children for intrathecal baclofen and deep brain stimulation