Spasticity, dystonia and tone management

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Target exams

Red flags

• Intrathecal baclofen withdrawal is a medical emergency: fever, rebound rigidity and spasticity, seizures and rhabdomyolysis can progress to multi-organ failure, so restore the infusion immediately and give enteral baclofen and benzodiazepines
• Never stop oral baclofen abruptly: sudden withdrawal causes seizures, rebound spasticity and hallucinations, so always taper
• Treat the tone only when it is the rate-limiting problem: high tone that does not limit function, comfort, care or hygiene should be observed, not medicalised
• Botulinum toxin products are not interchangeable: onabotulinumtoxinA abobotulinumtoxinA and incobotulinumtoxinA units differ and must never be dose-converted between brands
• Selective dorsal rhizotomy is for the ambulant spastic diplegic child with good selective motor control and minimal dystonia: it fails and may harm the dystonia-dominant or the most severe child
• A child whose pain or function suddenly worsens between injections may have a hip that has subluxed or dislocated, not just tone that has returned: image the hips

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises that hypertonia in a child is usually an upper motor neuron sign, separates velocity-dependent spasticity from action-induced dystonia, and names cerebral palsy as the commonest cause
• States the goal-directed principle that tone is treated only when it limits function, comfort, care or participation, and names the four core modalities of botulinum toxin, oral baclofen, intrathecal baclofen and selective dorsal rhizotomy
• Names the Gross Motor Function Classification System from GMFCS I to V and uses it to frame prognosis and the choice of tone therapy
• Explains the Sanger framework for hypertonia and distinguishes spasticity from dystonia, rigidity and mixed tone using the Modified Tardieu Scale and a careful history of provocation and overflow
• Matches the tone modality to the goal and the phenotype: focal botulinum toxin for focal spasticity, oral then intrathecal baclofen for generalised spasticity, selective dorsal rhizotomy for the ambulant spastic diplegic child, and a dystonia pathway from trihexyphenidyl to GPi deep brain stimulation
• Runs the multidisciplinary team that prevents contracture, hip subluxation, scoliosis and pain, and manages the intrathecal baclofen withdrawal syndrome as a medical emergency
• The Sanger classification of hypertonia into velocity-dependent spasticity and action-induced dystonia, with the mixed picture common in cerebral palsy
• The four core modalities of botulinum toxin type A, oral baclofen, intrathecal baclofen and selective dorsal rhizotomy with their indications and their key dangers
• The goal-directed principle that tone is treated only when it limits function, comfort, care or hygiene, and the GMFCS levels I to V that frame prognosis and therapy choice
• Assesses the child with cerebral palsy, separates the spastic from the dystonic phenotype, sets SMART goals with the family, and chooses the modality that matches the goal
• Manages botulinum toxin injection cycles with correct non-interchangeable dosing and a twelve-week minimum interval, and counsels on the intrathecal baclofen withdrawal syndrome
• Coordinates the multidisciplinary surveillance for hip subluxation, scoliosis, contracture and pain across childhood and into transition
• Level 1: Recognition that high tone in a child is usually an upper motor neuron sign, that spasticity and dystonia differ, and that cerebral palsy is the commonest cause
• Level 2: Application of the goal-directed principle and the four core modalities with the Modified Tardieu Scale and the GMFCS levels
• Level 3: Coordination of lifelong multidisciplinary care, the intrathecal baclofen and selective dorsal rhizotomy pathways, and the prevention of musculoskeletal complications
• Spasticity as a velocity-dependent increase in tonic stretch reflexes and dystonia as involuntary sustained or intermittent muscle contraction causing twisting postures and overflow, with the mixed picture common in cerebral palsy
• The four core tone modalities of botulinum toxin type A, oral baclofen, intrathecal baclofen and selective dorsal rhizotomy with their indications and key dangers
• The goal-directed principle that tone is treated only when it limits function comfort or care, and the prevention of contracture hip subluxation scoliosis and pain
• Structured assessment of a child with cerebral palsy using the Modified Tardieu Scale and the GMFCS, separating spasticity from dystonia
• Choice of the tone modality matched to the goal and the phenotype with the botulinum toxin and intrathecal baclofen and selective dorsal rhizotomy evidence
• Communication with the family on goal setting, the intrathecal baclofen withdrawal danger, and the lifelong multidisciplinary plan
• Spasticity and dystonia in cerebral palsy as upper motor neuron signs, with the Sanger classification of hypertonia and the GMFCS levels I to V
• The four core tone modalities with the AAN evidence-based practice parameter and the botulinum toxin and selective dorsal rhizotomy trial evidence
• The goal-directed principle and the multidisciplinary prevention of musculoskeletal complications across childhood
• Recognition of the child with hypertonia, separation of spasticity from dystonia, and identification of the tone that limits function comfort or care
• Stepwise management from focal botulinum toxin to oral and intrathecal baclofen to selective dorsal rhizotomy, matched to the goal and the phenotype
• Coordination of the multidisciplinary team and the management of the intrathecal baclofen withdrawal syndrome as an emergency
• Canadian approach to cerebral palsy tone management with the goal-directed principle and the four core modalities
• The selective dorsal rhizotomy pathway for the ambulant spastic diplegic child and the intrathecal baclofen pathway for the severe generalised child
• Coordination of lifelong multidisciplinary care and the prevention of contracture hip subluxation scoliosis and pain