Seizure control via pH manipulation: a phase II double-blind randomised controlled trial of inhaled carbogen as adjunctive treatment of paediatric convulsive status epilepticus (Carbogen for Status Epilepticus in Children Trial (CRESCENT)).

Background: Paediatric convulsive status epilepticus is the most common neurological emergency presenting to emergency departments. Risks of resultant neurological morbidity and mortality increase with seizure duration. If the seizure fails to stop within defined time-windows, standard care follows an algorithm of stepwise escalation to more intensive treatments, ultimately resorting to induction of general anaesthesia and ventilation.

It's easier to relearn gross motor skills than learn them for the first time after injury: Empirical evidence informing the age at injury debate.

The effect of age at injury on outcomes after brain injury has long been debated. Many have argued that the greater plasticity of the immature brain aids in its recovery from trauma, but others (notably Donald Hebb) have argued that early injury can impair the future ability of the brain to acquire new capabilities. This is difficult to assess empirically due to the presence of many age-dependent confounders.

Early mobilisation and rehabilitation in the PICU: a UK survey.

Objective: To understand the context and professional perspectives of delivering early rehabilitation and mobilisation (ERM) within UK paediatric intensive care units (PICUs).

Design: A web-based survey administered from May 2019 to August 2019.

Setting: UK PICUs.

Rehabilitation after paediatric acquired brain injury: Longitudinal change in content and effect on recovery.

Aim: To describe cross-sectional and longitudinal variation in neurorehabilitation content provided to young people after severe paediatric acquired brain injury (pABI) and to relate this to observed functional recovery.

Method: This was an observational study in a cohort of admissions to a residential neurorehabilitation centre. Recovery was described using the Pediatric Evaluation of Disability - Computer Adaptive Testing instrument.

Prion-like α-synuclein pathology in the brain of infants with Krabbe disease.

Krabbe disease is an infantile neurodegenerative disorder resulting from pathogenic variants in the GALC gene that causes accumulation of the toxic sphingolipid psychosine. GALC variants are also associated with Lewy body diseases, an umbrella term for age-associated neurodegenerative diseases in which the protein α-synuclein aggregates into Lewy bodies. To explore whether α-synuclein in Krabbe disease has pathological similarities to that in Lewy body disease, we performed an observational post-mortem study of Krabbe disease brain tissue (n = 4) compared to infant controls (n = 4) and identified widespread accumulations of α-synuclein.

Rasch Properties of the Cognitive and Linguistic Scale and Optimization for Outcome Trajectory Modeling in Pediatric Acquired Brain Injury.

Objectives: To (1) determine if items on the Cognitive and Linguistic Scale (CALS) follow a Rasch distribution and (2) explore the relationship between Rasch-derived Cognitive Ability Estimates and outcome trajectory parameters using a nonlinear mixed-effects modeling approach.

Design: Retrospective study.

Setting: Pediatric inpatient rehabilitation hospital.

Demonstration of functional rehabilitation treatment effects in children and young people after severe acquired brain injury.

Purpose: To examine relationships between functional outcomes after pediatric acquired brain injury (ABI) and measures of rehabilitation dose.

Methods: An observational study of children receiving residential neurorehabilitation after severe ABI.

Results: Basic total rehabilitation dose shows a paradoxical relationship to global outcome.

Use of Disease-Modifying Therapies in Pediatric Relapsing-Remitting Multiple Sclerosis in the United Kingdom.

Objectives: To compare the real-world effectiveness of newer disease-modifying therapies (DMTs) vs injectables in children with relapsing-remitting multiple sclerosis (RRMS).

Methods: In this retrospective, multicenter study, from the UK Childhood Inflammatory Demyelination Network, we identified children with RRMS receiving DMTs from January 2012 to December 2018. Clinical and paraclinical data were retrieved from the medical records.

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.

Treatment of MOG-IgG-associated disorder with rituximab: An international study of 121 patients.

Objective: To assess the effect of anti-CD20 B-cell depletion with rituximab (RTX) on relapse rates in myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD).

Methods: Retrospective review of RTX-treated MOGAD patients from 29 centres in 13 countries. The primary outcome measure was change in relapse rate after starting rituximab (Poisson regression model).

Network reorganisation following anterior temporal lobe resection and relation with post-surgery seizure relapse: A longitudinal study.

Objective: To characterise temporal lobe epilepsy (TLE) surgery-induced changes in brain network properties, as measured using diffusion weighted MRI, and investigate their association with postoperative seizure-freedom.

Methods: For 48 patients who underwent anterior temporal lobe resection, diffusion weighted MRI was acquired pre-operatively, 3-4 months post-operatively (N = 48), and again 12 months post-operatively (N = 13). Data for 17 controls were also acquired over the same period.

Investigating Brain Network Changes and Their Association With Cognitive Recovery After Traumatic Brain Injury: A Longitudinal Analysis.

Traumatic brain injury (TBI) can result in acute cognitive deficits and diffuse axonal injury reflected in white matter brain network alterations, which may, or may not, later recover. Our objective is to first characterize the ways in which brain networks change after TBI and, second, investigate if those changes are associated with recovery of cognitive deficits. We aim to make initial progress in discerning the relationships between brain network changes, and their (dys)functional correlates.

Seizure pathways change on circadian and slower timescales in individual patients with focal epilepsy.

Personalized medicine requires that treatments adapt to not only the patient but also changing factors within each individual. Although epilepsy is a dynamic disorder characterized by pathological fluctuations in brain state, surprisingly little is known about whether and how seizures vary in the same patient. We quantitatively compared within-patient seizure network evolutions using intracranial electroencephalographic (iEEG) recordings of over 500 seizures from 31 patients with focal epilepsy (mean 16.

Complement factor I deficiency: A potentially treatable cause of fulminant cerebral inflammation.

Objective: To raise awareness of complement factor I (CFI) deficiency as a potentially treatable cause of severe cerebral inflammation.

Methods: Case report with neuroradiology, neuropathology, and functional data describing the mutation with review of literature.

Results: We present a case of acute, fulminant, destructive cerebral edema in a previously well 11-year-old, demonstrating massive activation of complement pathways on neuropathology and compound heterozygote status for 2 pathogenic mutations in CFI which result in normal levels but completely abrogate function.

Early deviation from normal structural connectivity: A novel intrinsic severity score for mild TBI.

Objective: Studies of outcome after traumatic brain injury (TBI) are hampered by the lack of robust injury severity measures that can accommodate spatial-anatomical and mechanistic heterogeneity. In this study we introduce a Mahalanobis distance measure () as an intrinsic injury severity measure that combines in a single score the many ways a given injured brain's connectivity can vary from that of healthy controls. Our objective is to test the hypotheses that is superior to univariate measures in (1) discriminating patients and controls and (2) correlating with cognitive assessment.

Synaptic Scaling Improves the Stability of Neural Mass Models Capable of Simulating Brain Plasticity.

Neural mass models offer a way of studying the development and behavior of large-scale brain networks through computer simulations. Such simulations are currently mainly research tools, but as they improve, they could soon play a role in understanding, predicting, and optimizing patient treatments, particularly in relation to effects and outcomes of brain injury. To bring us closer to this goal, we took an existing state-of-the-art neural mass model capable of simulating connection growth through simulated plasticity processes.

Paediatric traumatic brain injury.

Purpose Of Review: To provide a summary of recent developments in the field of paediatric traumatic brain injury (TBI).

Recent Findings: The epidemiology of paediatric TBI with falling rates of severe TBI, and increasing presentations of apparently minor TBI. There is growing interest in the pathophysiology and outcomes of concussion in children, and detection of 'significant' injury, arising from concern about risks of long-term chronic traumatic encephalopathy.

Long-term outcomes of functional neurological disorder in children.

Objectives: To establish the incidence and long-term outcomes (up to 21 years) of children presenting to a University hospital paediatric neurology service with symptoms due to functional neurological disorder (FND) with particular reference to occurrence of FND or similar symptoms in adulthood.

Methods: Retrospective chart review to determine characteristics of the original paediatric FND presentation plus record-linkage with providers of Child and Adolescent Mental Health Services. Chart review of adult medical records for documentation of functional symptoms in adulthood.

Computer modelling of connectivity change suggests epileptogenesis mechanisms in idiopathic generalised epilepsy.

Patients with idiopathic generalised epilepsy (IGE) typically have normal conventional magnetic resonance imaging (MRI), hence diagnosis based on MRI is challenging. Anatomical abnormalities underlying brain dysfunctions in IGE are unclear and their relation to the pathomechanisms of epileptogenesis is poorly understood. In this study, we applied connectometry, an advanced quantitative neuroimaging technique for investigating localised changes in white-matter tissues in vivo.