Abstract

In children, dystonia presents almost always as a severe, generalized and rapidly progressive condition. Response to pharmacological therapies is poor, making surgical treatment a better option. Originally, this was limited to basal ganglia neuroablative lesion surgery, but currently, Deep Brain Stimulation (DBS), a more conservative intervention which can be tailored to treat individual patient symptoms has become the standard of care worldwide, in particular GPi DBS, which is highly efficient in primary dystonia. Historical developments in children's deep brain stimulationEuropean Journal of Paediatric NeurologyVol. 21Issue 1PreviewHeterogeneous by the underlying pathobiology and clinical presentation, childhood onset dystonia is most frequently progressive, with related disability and limitations in functions of daily living. Consequently, there is an obvious need for efficient symptomatic therapies. Full-Text PDF Open AccessThe International Classification of Functioning (ICF) to evaluate deep brain stimulation neuromodulation in childhood dystonia-hyperkinesia informs future clinical & research priorities in a multidisciplinary model of careEuropean Journal of Paediatric NeurologyVol. 21Issue 1PreviewThe multidisciplinary team (MDT) approach illustrates how motor classification systems, assessments and outcome measures currently available have been applied to a national cohort of children and young people with dystonia and other hyperkinetic movement disorders (HMD) particularly with a focus on dyskinetic cerebral palsy (CP). The paper is divided in 3 sections. Firstly, we describe the service model adopted by the Complex Motor Disorders Service (CMDS) at Evelina London Children's Hospital and King's College Hospital (ELCH-KCH) for deep brain stimulation. Full-Text PDF Open AccessDeep brain stimulation in cerebral palsy: Challenges and opportunitiesEuropean Journal of Paediatric NeurologyVol. 21Issue 1PreviewCerebral palsy (CP) is the most common cause for acquired dystonia in childhood. Pharmacological treatment is often unsatisfactory and side effects are frequently dose-limiting. Data on outcome of DBS in paediatric patients with dyskinetic CP is very limited and heterogeneous. Reasons for the variability in responses are not entirely known yet. Interestingly, some CP-patients seem to improve subjectively on pallidal stimulation but without measurable changes in impairment scales. Besides dystonia scales, the use of sensitive age-dependent assessments tools is therefore reasonable to capture the full effect. Full-Text PDF A comparative historical and demographic study of the neuromodulation management techniques of deep brain stimulation for dystonia and cochlear implantation for sensorineural deafness in childrenEuropean Journal of Paediatric NeurologyVol. 21Issue 1PreviewCochlear implants for sensorineural deafness in children is one of the most successful neuromodulation techniques known to relieve early chronic neurodisability, improving activity and participation. In 2012 there were 324,000 recipients of cochlear implants globally. Full-Text PDF

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