Intrathecal baclofen pumps in the management of hypertonia in childhood: a UK and Ireland wide survey.

BACKGROUND: Intrathecal baclofen (ITB) is a useful treatment for hypertonia where non-invasive treatments have been ineffective or poorly tolerated. There is an absence of national guidance on selection criteria and a lack of literature regarding patient characteristics and treatment details for children and young people (CYP) receiving ITB therapy in the UK and Ireland. We aimed to gather patient and treatment characteristics for CYP receiving ITB in the UK and Ireland. METHODS: An electronic survey was sent to all paediatric ITB centres in the UK and Ireland. Anonymised data were returned between December 2019 and April 2020. CYP >16 years and those awaiting ITB pump removal were excluded from the dataset. RESULTS: 176 CYP were identified as receiving ITB therapy across the UK and Ireland. The majority of CYP with ITB pumps were non-ambulant (93%) with a diagnosis of cerebral palsy (79%). Median age of ITB insertion was 9 years; median current age was 14 years. 79% of CYP had significant spasticity, 55% had significant dystonia. The most commonly used ITB dosing modes were continuous (73%) and flexible (23%). CONCLUSIONS: ITB pumps were most frequently used for non-ambulant CYP with cerebral palsy and existence of spasticity and/or dystonia in the UK and Ireland. Most CYP were receiving a continuous dose of ITB. There is significant variation in the number of paediatric ITB pumps across UK and Ireland. There is a need for development of nationally accepted paediatric referral criteria and clinical standards for ITB use.

Outcome of paediatric acute flaccid myelitis associated with enterovirus D68: a case series.

Enterovirus D68 (EV-D68) is an emerging infection associated with acute flaccid myelitis (AFM). Cases of AFM associated with EV-D68 infection have increased in recent years and the evidence for a causal link is growing. However, our understanding of the epidemiology, clinical features, prognosis, and neurological sequelae of EV-D68 requires ongoing surveillance and investigation. We report five cases of AFM in previously typically developing children (2-6y) from South East Scotland during September and October 2016 after infection with EV-D68 (all detected in the nasopharyngeal aspirates). All cases presented with significant neurological symptoms, which were severe in two cases requiring intensive care support because of respiratory paralysis. At 18-month follow-up, two cases remain ventilator-dependent with other cases requiring ongoing community rehabilitation. These cases represent one of the largest reported paediatric cluster of AFM associated with EV-D68 in Europe. The epidemiology and clinical information add to the knowledge base and the 18-month outcome will help clinicians to counsel families. WHAT THIS PAPER ADDS: Nasopharyngeal aspirate is more sensitive for viral isolation and isolated in all cases. Clinical outcome at 18 months after enterovirus D68 with acute flaccid myelitis provides information on extent of recovery and level of disability.

The long-term health, social, and financial burden of hypoxic-ischaemic encephalopathy.

Infants who suffer hypoxic-ischaemic encephalopathy (HIE) at term are at risk of dying or developing severe cerebral palsy (CP). Children with severe CP often have other neurodevelopmental disabilities, which may affect their quality of life as much as the CP itself. New treatments for HIE, such as cooling, may improve motor outcomes, but affected infants may still have significant cognitive or communication problems. Infants who have experienced HIE and develop CP will require significant medical input throughout childhood and adult life. The costs of this medical input are high, but the indirect costs to the child, his or her family, and the relevant social services and education systems are many times greater. When demonstrating the cost-effectiveness of interventions aimed at preventing or treating HIE, these additional costs should be taken into account.

Limbic encephalitis associated with elevated antithyroid antibodies.

Immune-mediated limbic encephalitis affects both adults and children. Patients typically present with seizures, memory problems, and imaging changes in the medial temporal lobes. Both paraneoplastic and nonparaneoplastic forms have been described in which the antibody to the voltage-gated potassium channel-complex associated protein, leucine-rich glioma-inactivated 1, is most commonly reported. Elevated antithyroid antibodies have also been reported in a range of neurological syndromes with encephalopathy, such as limbic encephalitis, often collectively termed Hashimoto encephalopathy, a condition whereby corticosteroids responsiveness with a complete recovery is commonly observed. Here we describe 3 children presenting with limbic encephalitis with elevated thyroid antibodies that did not respond to corticosteroids alone and required more aggressive immunotherapy, mirroring the slower treatment response that is more frequently seen in other immune-mediated forms of limbic encephalitis.

Consensus on the appropriate use of intrathecal baclofen (ITB) therapy in paediatric spasticity.

Among features of motor disorders in children, spasticity is associated with considerable morbidity and problems in care, particularly in severely affected patients. Intrathecal baclofen (ITB) has been increasingly used as a relatively specific treatment modality for spasticity. To date, most of the evidence for its use in paediatric patients has come from retrospective and uncontrolled studies, although randomised, controlled trials of screening ITB and ITB therapy itself have recently been published. This consensus statement on the use of ITB in paediatric patients with spasticity was developed on the basis of currently available evidence, with the aim of providing information for clinicians, promoting an expert opinion and a consistent approach to the management of these patients and emphasising the need for further prospective, large-scale studies.

Intravenous diazepam infusion in the management of planned intrathecal baclofen withdrawal.

Intrathecal baclofen is used in the management of spasticity associated with cerebral palsy (CP). Sudden cessation of intrathecal baclofen may give rise to the potentially life-threatening baclofen withdrawal syndrome. We report three cases in which intravenous diazepam is used effectively in the management of planned intrathecal withdrawal. All three patients had CP. In the first patient, a 19-year-old male with quadriplegia, the intrathecal catheter had to be removed. The other two patients, an 11-year-old female with diplegia and a 16-year-old male with quadriplegia, both required the entire device to be removed. The purpose of this case report is to illustrate the potential management for elective cases in a similar situation.

Severe progressive late onset myelopathy and arachnoiditis following neonatal meningitis.

This case series describes four children who had meningitis in the neonatal period. After a stable period of years, they developed a myelopathy caused by chronic arachnoiditis. The myelopathy was precipitated by a fall in two cases, and in two cases there was an acute deterioration after surgery. A history of neonatal meningitis should be taken into consideration before planning surgery or anaesthesia. Careful intra-operative positioning, immobilisation of the neck, and maintenance of blood pressure is important but may not prevent this complication.

Maturation of finger-synchronization skills.

The ability to move two fingers at the same time was measured in young and older adults. This study measured the accuracy with which 45 normally developing children (23 boys, 22 girls; mean ages 8.4 and 8.3 years respectively) and 49 young adults (25 males, 24 females; mean ages 19.8 and 20.5 years respectively) could synchronize movements of two fingers when making or breaking contact with circular metal discs. A portable electronic instrument displayed the timings. To measure 'in-phase' skills, attempts were made to contact both discs simultaneously with the two index fingers or to break both contacts; asynchrony was only a few milliseconds and differences between children and adults were insignificant. To measure 'antiphase' skills, attempts were made with the index and middle fingers to make contact simultaneously with one disc while breaking contact with the other; asynchrony was larger. Usually one contact was made and later the other was broken; for a while both fingers touched (overlap). Rarely one contact was broken before the other was made; for a while neither finger touched (gap). Boys' periods of overlap were longer than those of men and women; on both sides these differences were highly significant statistically (p<0.0001). Boys' errors on the right side were also significantly greater than those of girls. On both sides, mean errors of girls were higher than those of men and women: three of the four sets of data reached statistical significance. While boys' performance improved with age, that of girls was static. Children achieved an essentially adult level of control for in-phase skills but with antiphase skills the children, and especially the boys, had clearly poorer performances.