Spasticity, dystonia and tone management: Viva

Branching clinical structured oral on paediatric spasticity, dystonia and tone management covering the Sanger classification of hypertonia, the goal-directed principle, the Modified Tardieu Scale and the GMFCS, the four core modalities of focal botulinum toxin type A, oral baclofen, intrathecal baclofen and selective dorsal rhizotomy, the intrathecal baclofen withdrawal emergency, the dystonia pathway to GPi deep brain stimulation, and the multidisciplinary prevention of musculoskeletal complications.

branching clinical structured oral3 min readVerification in progress

Prompt

A 6-year-old girl with spastic diplegic cerebral palsy at GMFCS II walks with limitations and has adductor and calf spasticity causing scissoring, a tip-toe gait and difficult nappy changing. She has good selective motor control and minimal dystonia. The examiner asks how you classify her tone, how you set the goals and choose the first-line therapy, how botulinum toxin is dosed and timed, how you would decide on selective dorsal rhizotomy, how you manage an intrathecal baclofen withdrawal emergency, and what you tell the family about the long-term plan.

Branch 1: Classifying the tone

A strong candidate starts with the phenotype, because the phenotype drives the drug. This child has spasticity: a velocity-dependent increase in tonic stretch reflexes, felt as a catch then a release, distinct from dystonia, which is an action and overflow driven twisting posture that vanishes in sleep. The candidate names the Sanger framework as the international standard, confirms the velocity-dependent pattern with the Modified Tardieu Scale, and states the GMFCS level of two, which frames the functional goal and the modality, since GMFCS one to three is the orthoses, botulinum toxin and rhizotomy candidate. [1]

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References8Show ledger

[1]Delgado MR, Hirtz D, Aisen M, et al. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review). Neurology, 2010.PMID 20101040
[2]Shaunak M, Kelly VB Cerebral palsy in under 25 s: assessment and management (NICE Guideline NG62). Arch Dis Child Educ Pract Ed, 2018.PMID 29056589
[5]Molenaers G, Fagard K, Van Campenhout A, Desloovere K Botulinum toxin A treatment of the lower extremities in children with cerebral palsy. J Child Orthop, 2013.PMID 24432099
[6]McLaughlin J, Bjornson K, Temkin N, et al Selective dorsal rhizotomy: meta-analysis of three randomized controlled trials. Dev Med Child Neurol, 2002.PMID 11811645
[8]Albright AL, Cervi A, Singletary J Intrathecal baclofen for spasticity in cerebral palsy. JAMA, 1991.PMID 1999883
[9]Albright AL, Barry MJ, Painter MJ, et al. Infusion of intrathecal baclofen for generalized dystonia in cerebral palsy. J Neurosurg, 1998.PMID 9420075
[11]Monbaliu E, de Cock P, Ortibus E, et al. Clinical patterns of dystonia and choreoathetosis in participants with dyskinetic cerebral palsy. Dev Med Child Neurol, 2016.PMID 26173923
[12]Harvey AR, Baker LB, Reddihough DS, et al. Trihexyphenidyl for dystonia in cerebral palsy. Cochrane Database Syst Rev, 2018.PMID 29763510