Judith Helen Cross

• University College London

Objective This study aimed to identify the determinants of intellectual and developmental outcomes following pediatric hemispherotomy in a large, contemporary multicenter cohort. Methods We retrospectively analyzed the intellectual and developmental outcomes of 296 children and adolescents who underwent hemispherotomy between 2000 and 2016 and rec...

This review summarizes content presented at the Pediatric State of the Art Symposium held during the American Epilepsy Society's annual meeting in December 2024. The symposium focused on Lennox-Gastaut syndrome (LGS), a severe developmental and epileptic encephalopathy that emerges in childhood. Despite its diverse etiologies, LGS is defined by a c...

Background Sudden gains (rapid, large, stable improvements in symptoms) are common in psychological therapy and are associated with favourable outcomes, but no studies have investigated sudden gains in children and young people (CYP) with a chronic physical condition. Methods Within‐group study nested in the Mental Health Intervention for Children...

Developmental and epileptic encephalopathies (DEEs) are the most severe group of epilepsies, characterized by drug‐resistant seizures and developmental slowing or regression. DEEs encompass many epilepsy syndromes, although not all patients with a DEE can be classified into a specific syndrome. Our understanding of the etiologies of DEEs has been r...

Advances in genomic technologies have revolutionized the diagnosis of rare genetic diseases, leading to the emergence of precision therapies. However, there remains significant effort ahead to ensure the promise of precision medicine translates to improved outcomes. Here, we discuss the challenges in advancing precision child health and highlight h...

Objectives Approximately 80% of people with epilepsy live in low- and middle-income countries (LMICs), where limited resources and stigma hinder accurate diagnosis and treatment. Clinical machine learning models have demonstrated substantial promise in supporting the diagnostic process in LMICs by aiding in preliminary screening and detection of po...

Introduction The prevalence of epilepsy in sub-Saharan Africa varies considerably, and the exact estimate for Ghana remains unclear, particularly in peri-urban areas where data are scarce. More community-based studies are required to understand better the actual burden of epilepsy in these areas and the difficulties in accessing healthcare. Object...

The interaction between basic science epilepsy researchers and clinical epileptologists is a longstanding issue. Efforts to provide opportunities for a dialogue between preclinical and clinical epilepsy professionals are crucial to reduce the knowledge gap between them and improve the translational success of neurobiology‐based research. The Intern...

Objectives Research has shown that children with epilepsy often experience mental health disorders but face barriers to effective care. One solution is to train healthcare professionals within paediatric epilepsy services to deliver psychological interventions. The aim of this paper was to examine aspects of treatment integrity of the ‘Mental Healt...

Background The Global Burden of Disease Study (GBD) produces prevalence estimates for ‘idiopathic epilepsy’ (ie, of unknown aetiology) and ‘secondary epilepsy’ (ie, with known aetiology) but does not report prevalence by underlying aetiologies for ‘secondary epilepsy’. Methods We used nationwide, population-based register data from Denmark to iden...

Background Malformations of cortical development (MCDs) in children with focal epilepsy pose significant diagnostic challenges, and a precise radiological diagnosis is crucial for surgical planning. New MRI sequences and the use of artificial intelligence (AI) algorithms are considered very promising in this regard, yet studies evaluating the relat...

Background Many infancy-onset epilepsies have a poor prognosis for seizure control and neurodevelopmental outcome. Ketogenic diets can improve seizures in older children and adults unresponsive to antiseizure medicines. We aimed to determine the effectiveness of the ketogenic diet in reducing seizure frequency compared to further antiseizure medici...

Dravet syndrome (DS) is a severe childhood onset developmental and epileptic encephalopathy which leads to life-long disability. Symptoms usually manifest in the first year of life and include prolonged severe seizures, developmental delay and severe intellectual disability. DS patients have an increased mortality rate, including sudden unexpected...

Objective To characterize the experience of people with epilepsy and aligned healthcare workers (HCWs) during the first 18 months of the COVID‐19 pandemic and compare experiences in high‐income countries (HICs) with non‐HICs. Methods Separate surveys for people with epilepsy and HCWs were distributed online in April 2020. Responses were collected...

Epilepsy affects at least 50 million people worldwide, 80–85% of whom live in low-income and middle-income countries (LMICs). 1 Around 70% of people with epilepsy can be rendered seizure free with appropriate anti-seizure medications (ASMs). The WHO Intersectoral Global Action Plan on Epilepsy and Other Neurological Disorders (IGAP) 2 sets a global...

Objective Evaluate the cognitive, behavioural and affective processes involved in therapeutic change for young people with epilepsy and mental health difficulties receiving an integrated mental health intervention. Methods As part of a mixed methods convergent design, qualitative data were gathered in parallel to quantitative data at two timepoint...

Progressive inflammation of one hemisphere characterises Rasmussen’s encephalitis (RE), but contralesional epileptiform activity has been repeatedly reported. We aimed to quantify contralesional epileptiform activity in RE and uncover its functional and structural underpinnings. We retrospectively ascertained people with RE treated between 2000 and...

Aim To identify research priorities regarding the effectiveness of interventions for children and young people (CYP) with childhood neurological conditions (CNCs). These include common conditions such as epilepsies and cerebral palsy, as well as many rare conditions. Method The National Institute for Health and Care Research (NIHR) and the James L...

Fenfluramine (FFA), an antiseizure medication (ASM) with serotonergic and sigma‐1 receptor activity, is used to manage patients with developmental and epileptic encephalopathies (DEEs). It is approved in the US for treating seizures associated with Dravet syndrome (DS) and Lennox–Gastaut syndrome (LGS) in patients ≥2 years old and as add‐on therapy...

The implementation and potential of ketogenic dietary therapies (KDTs) have changed over time. The organization of KDT services, the availability of multidisciplinary teams, resources and support for patients and families still vary widely around the world. This diversity is reflected by a lack of consistency in reported outcomes, optimization of u...

Developmental and epileptic encephalopathies (DEEs) are characterized by pharmacoresistant seizures and developmental delay. Patients with DEEs experience multiple seizure types, including tonic–clonic seizures (TCS) that can be generalized tonic–clonic (GTCS) or focal evolving to bilateral tonic–clonic (FBTCS). Fenfluramine (FFA) has demonstrated...

Electroencephalography (EEG) and magnetoencephalography (MEG) non-invasively measure human brain electrophysiology. They differ in nature; MEG offers better performance while EEG (a wearable platform) is more practical. They are also complementary, with studies showing that concurrent MEG/EEG provides advantages over either modality alone, and cons...

At present, there is no internationally accepted set of core outcomes or measurement methods for epilepsy clinical practice. The International Consortium for Health Outcomes Measurement (ICHOM) convened an international working group of experts in epilepsy, people with epilepsy, and their representatives to develop minimum sets of standardized outc...

At present, there is no internationally accepted set of core outcomes or measurement methods for epilepsy clinical practice. Therefore, the International Consortium for Health Outcomes Measurement (ICHOM) convened an international working group of experts in epilepsy, people with epilepsy and their representatives to develop minimum sets of standar...

Ketogenic diet therapy (KDT) is a safe and effective treatment for epilepsy and glucose transporter type 1 (GLUT1) deficiency syndrome in infancy. Complete weaning from breastfeeding is not required to implement KDT; however, breastfeeding remains uncommon. Barriers include feasibility concerns and lack of referrals to expert centres. Therefore, pr...

Neuropsychological impairments are common in children with drug-resistant epilepsy. It has been proposed that epilepsy surgery may alleviate these impairments by providing seizure freedom; however, findings from prior studies have been inconsistent. We mapped long-term neuropsychological trajectories in children before and after undergoing epilepsy...

Objective The objective was to analyze seizure semiology in pediatric frontal lobe epilepsy patients, considering age, to localize the seizure onset zone for surgical resection in focal epilepsy. Methods Fifty patients were identified retrospectively, who achieved seizure freedom after frontal lobe resective surgery at Great Ormond Street Hospital...

Background and purpose Primary mitochondrial diseases (PMDs) are common inborn errors of energy metabolism, with an estimated prevalence of one in 4300. These disorders typically affect tissues with high energy requirements, including heart, muscle and brain. Epilepsy may be the presenting feature of PMD, can be difficult to treat and often represe...

Purpose : Anecdotal evidence suggests that children with epilepsy (CWE) are limited in the frequency of their daily physical activity (PA). However, there is limited research utilizing device-based measures of PA. We compared levels of PA and sedentary behavior in CWE (11–15 y) and age- and gender-matched healthy controls. Method : Participants (n...

Objectives Approximately 80% of people with epilepsy live in low- and middle-income countries (LMICs), where limited resources and stigma hinder accurate diagnosis and treatment. Clinical machine learning models have demonstrated substantial promise in supporting the diagnostic process in LMICs without relying on specialised or trained personnel. H...

This study utilized a qualitative design to explore dietitians’ perceptions regarding Ketogenic Diet Therapy (KDT) for patients with drug-resistant epilepsy in Kenya. Dietitians from Kenya were selected and consented. Audio-recorded interviews were conducted, followed by thematic analysis of verbatim transcripts to identify recurring patterns. The...

Fully elucidating the burden that Lennox–Gastaut syndrome (LGS) places on individuals with the disease and their caregivers is critical to improving outcomes and quality of life (QoL). This systematic literature review evaluated the global burden of illness of LGS, including clinical symptom burden, care requirements, QoL, comorbidities, caregiver...

Introduction: Lennox Gastaut syndrome (LGS) as an electroclinical diagnosis has been utilized as a clinical entity for more than 70 years. However, with the recognition of other distinct electroclinical epilepsy syndromes, no consistent single etiology, and the variability of criteria used in clinical trials, the clinical utility of such a diagnos...

When planning for epilepsy surgery, multiple potential sites for resection may be identified through anatomical imaging. Magnetoencephalography (MEG) using optically pumped sensors (OP-MEG) is a non-invasive functional neuroimaging technique which could be used to help identify the epileptogenic zone from these candidate regions. Here we test the u...

Background Although levetiracetam and phenytoin are widely used antiseizure medications (ASM) in neonates, their efficacy on seizure freedom is unclear. We evaluated electroencephalographic (EEG) seizure freedom following sequential levetiracetam and phenytoin in neonatal seizures unresponsive to phenobarbital. Methods We recruited neonates born ≥...

Background and objective: Patients with presumed nonlesional focal epilepsy-based on either MRI or histopathologic findings-have a lower success rate of epilepsy surgery compared with lesional patients. In this study, we aimed to characterize a large group of patients with focal epilepsy who underwent epilepsy surgery despite a normal MRI and had...

A variety of terms, such as “antiepileptic,” “anticonvulsant,” and “antiseizure” have been historically applied to medications for the treatment of seizure disorders. Terminology is important because using terms that do not accurately reflect the action of specific treatments may result in a misunderstanding of their effects and inappropriate use....

Dravet syndrome (DS) and Lennox–Gastaut syndrome (LGS) are rare developmental and epileptic encephalopathies associated with seizure and nonseizure symptoms. A comprehensive understanding of how many individuals are affected globally, the diagnostic journey they face, and the extent of mortality associated with these conditions is lacking. Here, we...

Objective Although effectiveness of ketogenic dietary therapy (KDT) as a treatment for drug-resistant epilepsy has been demonstrated in clinical trials, key research questions remain; many best addressed by a collaborative approach. We aim to establish an international registry of individuals with epilepsy referred for KDT to determine long-term cl...

Objective Focal epilepsy is common in low‐ and middle‐income countries. The frequency and nature of possible underlying structural brain abnormalities have, however, not been fully assessed. Methods We evaluated the possible structural causes of epilepsy in 331 people with epilepsy (240 from Kenya and 91 from South Africa) identified from communit...

Objective We aimed to evaluate determinants of functional outcome after pediatric hemispherotomy in a large and recent multicenter cohort. Methods We retrospectively investigated the functional outcomes of 455 children who underwent hemispherotomy in five epilepsy centers in 2000–2016. We identified determinants of unaided walking, voluntary grasp...

Background: Most neonatal and infantile-onset epilepsies have presumed genetic aetiologies, and early genetic diagnoses have the potential to inform clinical management and improve outcomes. We therefore aimed to determine the feasibility, diagnostic yield, and clinical utility of rapid genome sequencing in this population. Methods: We conducted...

Objective This study was undertaken to assess the safety and efficacy of fenfluramine in the treatment of convulsive seizures in patients with Dravet syndrome. Methods This multicenter, randomized, double‐blind, placebo‐controlled, parallel‐group, phase 3 clinical trial enrolled patients with Dravet syndrome, aged 2–18 years with poorly controlled...

Aim To evaluate a lesion detection algorithm designed to detect focal cortical dysplasia (FCD) in children undergoing stereoelectroencephalography (SEEG) as part of their presurgical evaluation for drug‐resistant epilepsy. Method This was a prospective, single‐arm, interventional study (Idea, Development, Exploration, Assessment, and Long‐Term Fol...

Objective Neurosurgery is a safe and effective form of treatment for select children with drug‐resistant epilepsy. Still, there is concern that it remains underutilized, and that seizure freedom rates have not improved over time. We investigated referral and surgical practices, patient characteristics, and postoperative outcomes over the past two d...

Background and purpose: Prior studies have found an association between calcification and the epileptogenicity of tubers in tuberous sclerosis complex. Quantitative susceptibility mapping is a novel tool sensitive to magnetic susceptibility alterations due to tissue calcification. We assessed the utility of quantitative susceptibility mapping in i...

Objective The accurate prediction of seizure freedom after epilepsy surgery remains challenging. We investigated if (1) training more complex models, (2) recruiting larger sample sizes, or (3) using data‐driven selection of clinical predictors would improve our ability to predict postoperative seizure outcome using clinical features. We also conduc...

The global burden of neurological disorders is substantial and increasing, especially in low-resource settings. The current increased global interest in brain health and its impact on population wellbeing and economic growth, highlighted in the World Health Organization's new Intersectoral Global Action Plan on Epilepsy and other Neurological Disor...

Purpose: This retrospective chart review study (GWEP20052) evaluated plant-derived highly purified cannabidiol (CBD; Epidyolex®; 100 mg/mL oral solution) use without clobazam as add-on therapy in patients aged ≥2 years with Lennox-Gastaut syndrome (LGS) or Dravet syndrome (DS) enrolled in a European Early Access Program. Methods: Data were extra...

Background: Identification of convulsive epilepsy in sub-Saharan Africa relies on access to resources that are often unavailable. Infrastructure and resource requirements can further complicate case verification. Using machine-learning techniques, we have developed and tested a region-specific questionnaire panel and predictive model to identify p...

Objective We aimed to assess determinants of seizure outcome following pediatric hemispherotomy in a contemporary cohort. Methods We retrospectively analyzed the seizure outcomes of 457 children who underwent hemispheric surgery in five European epilepsy centers between 2000 and 2016. We identified variables related to seizure outcome through mult...

Objective: To survey attitudes towards, and knowledge about, epilepsy among school staff in a defined geographical region in the United Kingdom. Methods: School staff (n = 160) from 18 schools (56% of eligible schools) where children with epilepsy were currently attending were surveyed. Surveys were developed in collaboration with educational pr...

Objective: The accurate prediction of seizure freedom after epilepsy surgery remains challenging. We investigated if 1) training more complex models, 2) recruiting larger sample sizes, or 3) using data-driven selection of clinical predictors would improve our ability to predict post-operative seizure outcome. We also conducted the first external va...

Worldwide, People with Epilepsy (PWE) are confronted with several barriers to face-to-face consultations. These obstacles hamper appropriate clinical follow-up and also increase the treatment gap for Epilepsy. Telemedicine holds the potential to enhance management as follow-up visits for PWE are focused on more on clinical history and counselling r...

Objective To evaluate whether fenfluramine (FFA) is associated with improvement in everyday executive function (EF)—self-regulation—in preschool-aged children with Dravet syndrome (DS). Methods Children with DS received placebo or FFA in one of two phase III studies (first study: placebo, FFA 0.2 mg/kg/day, or FFA 0.7 mg/kg/day added to stiripento...

https://cdn.who.int/media/docs/default-source/essential-medicines/2023-eml-expert-committee/applications-for-addition-of-new-medicines/a24_levetiracetam.pdf?sfvrsn=eac59651_2

Objective: To gain an understanding of the views of school-aged children with epilepsy, their parents, and school staff regarding the impact of epilepsy on sleep. Methods: As part of the What I Need in School (WINS) study, school-aged children (n = 18) with 'active epilepsy' (taking Anti-Seizure Medications, ASMs, for epilepsy), their parents (n...

Objective Antiseizure medications (ASMs) remain the mainstay of epilepsy treatment. These ASMs have mainly been tested in trials in adults with epilepsy, which subsequently led to market authorization (MA). For treatment of – especially young – children with epilepsy, several ASMs do not have a MA and guidelines are lacking, subsequently leading to...

Background The relationship between COVID-19 and epilepsy is uncertain. We studied the potential association between COVID-19 and seizures or epilepsy in the six months after infection. Methods We applied validated methods to an electronic health records network (TriNetX Analytics) of 81 million people. We closely matched people with COVID-19 infe...

We propose an instructive figure that summarized the classification of epilepsy syndromes according to the 2022 report of the ILAE Task Force on Nosology and Definitions. Our aim is to present on the same figure different concepts such as the names of epilepsy syndromes, their extreme and classical ages of onset, their epilepsy types (generalized,...

Major disruption in the delivery of healthcare services can occur in exceptional situations such as natural disasters, conflicts, periods of severe economic hardship, and epidemics. These disruptions typically affect to the greatest extent the most vulnerable segments of the population, including people with epilepsy. Inability to access healthcare...

In times of severe antiseizure medication (ASM) shortage due to emergency situations (e.g., disasters, conflicts, sudden disruption to international supply chains), management of people with epilepsy with available ASMs can be difficult. A group of experts was brought together by the International League Against Epilepsy (ILAE) to formulate recomme...

Objective To determine whether an ictal electroencephalographic (EEG) recording as part of presurgical evaluation of children with a demarcated single unilateral magnetic resonance imaging (MRI) lesion is indispensable for surgical decision‐making, we investigated the relationship of interictal/ictal EEG and seizure semiology with seizure‐free outc...

Aim To investigate the link between sleep disruption and cognitive impairment in childhood epilepsy by studying the effect of epilepsy on sleep homeostasis, as reflected in slow‐wave activity (SWA). Method We examined SWA from overnight EEG‐polysomnography in 19 children with focal epilepsy (mean [SD] age 11 years 6 months [3 years], range 6 years...

Background: Over recent years, epilepsy surgery has gained its place as the standard of care for many cases of drug-resistant focal epilepsy. Our paper aims to briefly summarize major achievements in epilepsy surgery in children and discuss emerging aspects. Methods: Our review has a narrative and a systematic part. Articles included in the systema...

Aim To investigate the rate of successful withdrawal of antiseizure medication (ASM) after starting the ketogenic diet in children and identify predictive factors. Method We retrospectively reviewed data of children with epilepsy, who were treated with the ketogenic diet for 6 months or longer at our institution, over a 5‐year period. We defined s...

One outstanding challenge for machine learning in diagnostic biomedical imaging is algorithm interpretability. A key application is the identification of subtle epileptogenic focal cortical dysplasias (FCDs) from structural MRI. FCDs are difficult to visualize on structural MRI but are often amenable to surgical resection. We aimed to develop an op...

Background and objectives ATP1A3 is associated with a broad spectrum of predominantly neurological disorders, that continues to expand beyond the initially defined phenotypes of Alternating Hemiplegia of Childhood (AHC), Rapid-onset Dystonia Parkinsonism (RDP) and Cerebellar ataxia, Areflexia, Pes cavus , Optic atrophy, Sensorineural hearing loss s...

Epilepsy surgery is the treatment of choice for patients with drug‐resistant seizures. A timely evaluation for surgical candidacy can be life‐saving for patients who are identified as appropriate surgical candidates, and may also enhance the care of nonsurgical candidates through improvement in diagnosis, optimization of therapy, and treatment of c...