German registry of paediatric deep brain stimulation in patients with childhood-onset dystonia (GEPESTIM)

Background

Data on paediatric deep brain stimulation (DBS) is limited, especially for long-term outcomes, because of small numbers in single center series and lack of systematic multi-center trials.

What parents think and feel about deep brain stimulation in paediatric secondary dystonia including cerebral palsy: A qualitative study of parental decision-making

Background

Dystonia is characterised by involuntary movements and postures. Deep Brain Stimulation (DBS) is effective in reducing dystonic symptoms in primary dystonia in childhood and to lesser extent in secondary dystonia. How families and children decide to choose DBS surgery has never been explored.

A comparative historical and demographic study of the neuromodulation management techniques of deep brain stimulation for dystonia and cochlear implantation for sensorineural deafness in children

Cochlear 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.

Reactivity of skin blood flow and heart rate to external thermal stimulation in anencephaly

Oscillations of skin blood flow and heart rate can be synchronised using external rhythmic thermal stimulation in healthy adults and infants. We examined the effect of thermal stimulation on the cutaneous circulation and heart rate of an anencephalic neonate using cutaneous laser Doppler flowmetry and ECG monitoring. The results suggest that synchronisation of SBF and HR to thermal stimulation can also be induced in an anencephalic newborn.     

The 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 care

The 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. We describe lessons learnt from available dystonia studies and discuss/propose ways to measure DBS and other dystonia-related intervention outcomes. We aim to report on current available functional outcome measures as well as some impairment-based assessments that can encourage and generate discussion among movement disorders specialists of different backgrounds regarding choice of the most important areas to be measured after DBS and other interventions for dystonia management. Finally, some recommendations for multi-centre collaboration in regards to functional clinical outcomes and research methodologies for dystonia-related interventions are proposed.     

Heart rate responses to non-arousing trigeminal stimulation in infants: effects of sleep position, sleep state and postnatal age

AIMS: The aim of this study was to examine the effects of maternal smoking, sleeping position, sleep state and postnatal age on heart rate changes following non-arousing trigeminal stimulation in infants. SUBJECTS: We studied healthy term infants, 13 of whom were born to mothers who did not smoke and 11 to mothers who smoked during pregnancy. Each infant was studied using daytime polysomnography on 3 occasions: (a) 2-3 weeks, (b) 2-3 months and (c) 5-6 months after birth. Nasal air-jet stimulation was presented in both active sleep (AS) and quiet sleep (QS) when infants slept both prone and supine. RESULTS: We found no difference between infants of smoking and non-smoking mothers in any of the parameters measured. Minimum HR (MinHR) following non-arousing trigeminal stimulation was significantly lower in the supine compared to the prone sleeping position at 2-3 weeks and 2-3 months of age (p<0.05) in AS, and at all 3 ages in QS (p<0.01). MinHR was significantly lower in QS compared to AS at 2-3 months when infants slept prone and at 5-6 months when sleeping supine (p<0.01). In QS, MinHR became lower with increasing postnatal age in both sleep positions (p<0.01). In AS, there was no maturational effect. The normalized bradycardia (DeltaHR%) was significantly greater in AS than in QS at 2-3 weeks of age (p<0.05) when infants slept supine. CONCLUSION: Our study has shown that there was a decrease in heart rate (MinHR) following trigeminal stimulation in infants up to 6 months of age and this was affected by sleep position and sleep state, being larger in the supine sleeping position and the QS state.     

Reactivity of skin blood flow and heart rate to thermal stimulation in infants during the first postnatal days and after a two-month follow-up

The reactivity of skin blood flow and heart rate to periodic thermal stimulation was studied in 10 term small-for-gestational age (SGA) infants, in 9 preterm and 8 term normal-sized infants during the first two postnatal months. During the first postnatal days, the increase in thermally stimulated skin blood flow oscillations was absent in SGA infants but was clearly present in both preterm ( p < 0.01) and term ( p <0.01) normal-sized infants. The responsiveness of SGA infants improved during the follow-up ( p < 0.05). The response of the periodic heart rate variability of SGA and preterm infants was inferior to term normal-sized infants until the age of 2 months. The cardiovascular responsiveness of growth-retarded infants seems abnormal during the first days of life, and may impair their ability to meet stress, e.g. changes of ambient temperature.