Reducing epilepsy diagnostic and treatment gaps: Standardized paediatric epilepsy training courses for health care professionals.

AIM: To evaluate improvement in knowledge and clinical behaviour among healthcare professionals after attendance at paediatric epilepsy training (PET) courses. METHOD: Since 2005, 1-day PET courses have taught evidence-based paediatric epilepsy management to doctors and nurses in low-, middle-, and high-income countries. A cohort study was performed of 7528 participants attending 252 1-day PET courses between 2005 and 2020 in 17 low-, middle-, and high-income countries, and which gathered data from participants immediately after the course and then 6 months later. Training outcomes were measured prospectively in three domains (reaction, learning, and behaviour) using a mixed-methods approach involving a feedback questionnaire, a knowledge quiz before and after the course, and a 6-month survey. RESULTS: Ninety-eight per cent (7217 of 7395) of participants rated the course as excellent or good. Participants demonstrated knowledge gain, answering a significantly higher proportion of questions correctly after the course compared to before the course (88% [47 883 of 54 196], correct answers/all quiz answers, vs 75% [40 424 of 54 196]; p < 0.001). Most survey responders reported that the course had improved their epilepsy diagnosis and management (73% [311 of 425]), clinical service (68% [290 of 427]), and local epilepsy training (68% [290 of 427]). INTERPRETATION: This was the largest evaluation of a global epilepsy training course. Participants reported high course satisfaction, showed knowledge gain, and described improvements in clinical behaviour 6 months later. PET supports the global reduction in the epilepsy 'treatment gap' as promoted by the World Health Organization.

Epilepsy deaths in children: Improvements driven by data and surveillance in pediatrics.

Epilepsy-related death in children and young people deserves understanding and intervention along with epilepsy-related deaths in adults. Risk of death from epilepsy varies at different ages, and the specific calculations of risk remains complex and varies between studies. There have been several UK studies examining factors associated with epilepsy-related deaths. A UK national audit with other national initiatives has evidenced improving quality of care and more recently allowed service provision factors associated with reduced epilepsy-related death to be evidenced. A national program of health education, formalized epilepsy networks, commissioned surgical pathways, and patient information resources around risk and participation are examples of quality improvement initiatives. Epilepsy-related death is a key outcome, and there remains many difficulties and opportunities at local, regional, and national level to better understand and improve this outcome for children and young people and the adults that they should become. This paper is for the Special Issue: Prevent 21: SUDEP Summit - Time to Listen.

Association of quality of paediatric epilepsy care with mortality and unplanned hospital admissions among children and young people with epilepsy in England: a national longitudinal data linkage study.

BACKGROUND: Concerns have been raised about variation in care quality and outcomes among children and young people with epilepsies in England. We aimed to investigate the association between quality of paediatric care, hospital admissions, and all-cause deaths among epilepsy patients. METHODS: In this longitudinal data linkage study of paediatric epilepsy services in England, we linked unit-level data from round 1 (2009-11) and round 2 (2013-14) of the Epilepsy12 national clinical audit, with death registrations from the UK Office for National Statistics and data for unplanned hospital admissions from Hospital Episode Statistics. We investigated the association between unit-level performance in involving a paediatrician with epilepsy expertise, an epilepsy specialist nurse, and a paediatric neurologist (where appropriate) in round 1 and the proportion of adolescents (aged 10-18 years) with epilepsy admitted to each unit who subsequently died during the study period (April 1, 2009, to March 31, 2015). We also investigated whether change in Epilepsy12 performance between the two audit rounds was associated with changes in the standardised ratio of observed-to-expected unplanned epilepsy admissions over the same period. FINDINGS: In 99 units with data for the analyses relating to paediatricians with epilepsy expertise and epilepsy specialist nurses, 134 (7%) of 1795 patients died during the study period, 88 (5%) of whom died after the transition to adult service. In 55 units with data for the analyses relating to paediatric neurologists, 79 (7%) of 1164 patients died, 54 (5%) of whom did so after the transition. In regression models adjusting for population, unit, and hospital activity characteristics, absolute reductions in total mortality risk (6·4 percentage points, 95% CI 0·1-12·7) and mortality risk after transition (5·7 percentage points, 0·6-10·8) were found when comparing units where all versus no eligible patients were seen by a paediatric neurologist. Units where all eligible patients were seen by a paediatric neurologist were estimated to have absolute reductions of 4·6 percentage points (0·3-8·9) in total mortality and of 4·6 percentage points (1·2-8·0) in post-transition mortality, compared with units where no or some eligible patients were seen by a paediatric neurologist. There was no significant association between performance on being seen by an epilepsy specialist nurse or by a paediatrician with epilepsy expertise and mortality. In units where access to an epilepsy specialist nurse decreased, the standardised ratio of epilepsy admissions increased by a mean of 0·21 (0·01-0·42). INTERPRETATION: Among adolescents with epilepsy, greater involvement of tertiary specialists in paediatric care is associated with decreased all-cause mortality in the period after transition to adult services. Reduced access to an epilepsy specialist nurse was associated with an increase in paediatric epilepsy admissions. FUNDING: The Health Foundation.

Core Health Outcomes in Childhood Epilepsy (CHOICE): Development of a core outcome set using systematic review methods and a Delphi survey consensus.

OBJECTIVE: Establishing a core set of outcomes to be evaluated and reported in intervention trials aims to improve the usefulness of health research. There is no established core outcome set (COS) for childhood epilepsies. The aim of this study was to select a COS to be used in evaluative research of interventions for children with rolandic epilepsy (RE). METHODS: We followed guidance from the COMET (Core Outcome Measures in Effectiveness Trials) Initiative. First, we identified outcomes that had been measured in research through a systematic review. Second, young people with RE, parents, and professionals were invited to take part in a Delphi survey in which participants rated the importance of candidate outcomes. Last, a face-to-face meeting was convened to seek consensus on which outcomes were critical to include and to ratify the final COS. RESULTS: From 37 eligible papers in the review, we identified and included 48 candidate outcomes in the survey. We sent invitations to 165 people registered to take part in the survey; of these, 102 (62%) completed Round 1, and 80 (78%) completed Round 2 (three young people, 16 parents, 61 professionals). In Round 2 we included four additional outcomes suggested by participants in Round 1. The consensus meeting included two young people, four parents, and nine professionals who were eligible to vote and ratified the COS as 39 outcomes across 10 domains. SIGNIFICANCE: Our methodology was a proportionate and pragmatic approach toward producing a COS for evaluating research on interventions aiming to improve the health of children with RE.

Evaluation of a questionnaire to measure parent/carer and child/young person experience of NHS epilepsy services.

PURPOSE: To validate a patient-reported-experience-measure, PREM, of the NHS paediatric epilepsy service. METHODS: Section 1 of the PREM recorded demographic and clinical characteristics, and Section 2 collected information about the users' experience with the service. Section 2 included eighteen statements around three constructs: communication and provision of information to service users, interpersonal skills of staff, and clinic visits and accessibility to the services. Face validity, construct validity, internal reliability, and internal consistency were used to examine the robustness of these statements. The PREM was completed by parents/carers and also children/young people. RESULTS: PREMs were received from 145 of the 192 audit units; 2335 completed forms were returned; the attitude statements were completed by 750 children/young people and 1550 parents/carers. Face validity of the PREM was good. Construct validity was indecisive; confirmatory factor analysis of the hypothesised construct was weak. Exploratory factor analysis identified a four factor solution for the parent/carers dataset and a five factor solution for the children/young people's dataset. Internal reliability was good for the parent/carers dataset but less good for the children/young people. Internal consistency was moderately good for both datasets. CONCLUSIONS: These findings indicate that the PREM is likely to be a valid tool with the potential to elicit a wide variety of reliable views from parents/carers of children with epilepsy. The construct validity for the PREM should be reassessed with confirmatory factor analysis in a new dataset. More work needs to be undertaken with children/young people to design statements that capture their specific needs.

A UK survey of the experience of service provision for children and young people with epilepsy.

PURPOSE: To survey patient and carer experience for children and young people with epilepsy across the United Kingdom. METHODS: We used a Patient Reported Experience Measure methodology to explore perceived satisfaction with their epilepsy service. A survey collected anonymised proxy data on demography and illness severity, and perceptions of interaction with clinicians, ease of access to the service and the quality and quantity of epilepsy information provided. The questionnaire was completed by the child's or young person's carer or by the young person. RESULTS: Survey questionnaires were distributed across all of the 192 paediatric units providing epilepsy care for children in the UK. 145 units (75%) submitted data and there were 2335 responses. 90% of young people and 86% of carers were satisfied with the care they had received. Using multi-level logistic regression modelling, those factors most strongly affecting satisfaction were determined. While many proxies of illness severity adversely affected satisfaction, comorbidity did not. A dedicated clinic setting, perceived adequate information and guidance on restrictions on their child, if any, all improved satisfaction. However, the significantly strongest factor influencing satisfaction was "ease of access" to the service. CONCLUSIONS: These data demonstrate the feasibility of collecting large population sizes to allow a better understanding of the needs of children and young people accessing an epilepsy service. They allow the identification of factors most closely linked to patient satisfaction and provide potentially valuable information on how to improve the quality of care of children and young people with epilepsy.

Core Health Outcomes In Childhood Epilepsy (CHOICE): protocol for the selection of a core outcome set.

BACKGROUND: There is increasing recognition that establishing a core set of outcomes to be evaluated and reported in trials of interventions for particular conditions will improve the usefulness of health research. There is no established core outcome set for childhood epilepsy. The aim of this work is to select a core outcome set to be used in evaluative research of interventions for children with rolandic epilepsy, as an exemplar of common childhood epilepsy syndromes. METHODS: First we will identify what outcomes should be measured; then we will decide how to measure those outcomes. We will engage relevant UK charities and health professional societies as partners, and convene advisory panels for young people with epilepsy and parents of children with epilepsy. We will identify candidate outcomes from a search for trials of interventions for childhood epilepsy, statutory guidance and consultation with our advisory panels. Families, charities and health, education and neuropsychology professionals will be invited to participate in a Delphi survey following recommended practices in the development of core outcome sets. Participants will be able to recommend additional outcome domains. Over three rounds of Delphi survey participants will rate the importance of candidate outcome domains and state the rationale for their decisions. Over the three rounds we will seek consensus across and between families and health professionals on the more important outcomes. A face-to-face meeting will be convened to ratify the core outcome set. We will then review and recommend ways to measure the shortlisted outcomes using clinical assessment and/or patient-reported outcome measures. DISCUSSION: Our methodology is a proportionate and pragmatic approach to expediently produce a core outcome set for evaluative research of interventions aiming to improve the health of children with epilepsy. A number of decisions have to be made when designing a study to develop a core outcome set including defining the scope, choosing which stakeholders to engage, most effective ways to elicit their views, especially children and a potential role for qualitative research.

Integrated care for childhood epilepsy: ongoing challenges and lessons for other long-term conditions.

Epilepsy care has been identified as a major global issue-and there are many recognised concerns in the UK for children and young people with the condition. A proposed new model could help to increase multisector integration, facilitate better outcomes and offer lessons for improving care of other long-term conditions.

Guidelines and Quality Standards in the Care of Children with Epilepsy.

To provide quality care for children with epilepsy there is a continuing need to synthesize clinical research into forms that reflect best clinical practice. The design of evidence-based guidelines allows the gathering of this information together. This review discusses the components needed to analyze published data and produce recommendations for clinical management. Guideline implementation should be seen as an essential component that is integrated into guideline development. Robust clinical practice guidelines can also form the basis of defining quality standards that in turn should allow the development and measurement of clinical outcomes that lead to direct improvements in patient care.

The current state of epilepsy guidelines: A systematic review.

OBJECTIVE: The International League Against Epilepsy (ILAE) Epilepsy Guidelines Task Force, composed of 14 international members, was established in 2011 to identify, using systematic review methodology, international epilepsy clinical care guidelines, assess their quality, and determine gaps in areas of need of development. METHODS: A systematic review of the literature (1985-2014) was performed in six electronic databases (e.g. Medline, Embase) using a broad search strategy without initial limits to language or study design. Six gray literature databases (e.g., American Academy of Neurology [AAN], ILAE) were also searched to minimize publication bias. Two independent reviewers screened abstracts, reviewed full text articles, and performed data abstraction. Descriptive statistics and a meta-analysis were generated. RESULTS: The search identified 10,926 abstracts. Of the 410 articles selected for full text review, 63 met our eligibility criteria for a guideline. Of those included, 54 were in English and 9 were in other languages (French, Spanish, and Italian). Of all guidelines, 29% did not specify the target age groups, 27% were focused on adults, 22% included only children, and 6% specifically addressed issues related to women with epilepsy. Guidelines included in the review were most often aimed at guiding clinical practice for status epilepticus (n = 7), first seizure (n = 6), drug-resistant epilepsy (n = 5), and febrile seizures (n = 4), among others. Most of the guidelines were therapeutic (n = 35) or diagnostic (n = 16) in nature. The quality of the guidelines using a 1-7 point scale (7 = highest) varied and was moderate overall (mean = 4.99 ± 1.05 [SD]). SIGNIFICANCE: We identified substantial gaps in topics (e.g., epilepsy in the elderly) and there was considerable heterogeneity in methodologic quality. The findings should offer a valuable resource for health professionals caring for people with epilepsy, since they will help guide the prioritization, development, and dissemination of future epilepsy-related guidelines.

Developing clinical practice guidelines for epilepsy: A report from the ILAE Epilepsy Guidelines Working Group.

Clinical practice guidelines (CPGs) contain evidence-based recommendations to guide clinical care, policy development, and quality of care improvement. A recent systematic review of epilepsy guidelines identified considerable variability in the quality of available guidelines. Although excellent frameworks for CPG development exist, processes are not followed uniformly internationally, and resources to develop CPGs may be limited in certain settings. An International League Against Epilepsy (ILAE) working group was charged with proposing methodology to guide the development of future epilepsy-specific CPGs. A comprehensive literature search (1985-2014) identified articles related to CPG development and handbooks. Guideline handbooks were included if they were publicly available, and if their methodology had been used to develop CPGs. The working group's expertise also informed the creation of methodologies and processes to develop future CPGs for the ILAE. Five handbooks from North America (American Academy of Neurology), Europe (Scottish Intercollegiate Guidelines Network & National Institute for Health and Care Excellence), Australia (National Health and Medical Research Council), World Health Organization (WHO), and additional references were identified to produce evidence-based, consensus-driven methodology for development of epilepsy-specific CPGs. Key components of CPG development include the following: identifying the topic and defining the scope; establishing a working group; identifying and evaluating the evidence; formulating recommendations and determining strength of recommendations; obtaining peer reviews; dissemination, implementation, and auditing; and updating and retiring the CPG. A practical handbook and toolkit was developed. The resulting CPG development toolkit should facilitate the development of high-quality ILAE CPGs to improve the care of persons with epilepsy.

Deaths in children with epilepsies: a UK-wide study.

PURPOSE: This UK-wide review of deaths in children with epilepsies was undertaken to ascertain any demographic, clinical, organisational, or management factors associated with the deaths, and to determine the extent to which any of these may have deviated from nationally agreed best practice. METHOD: Paediatricians across the UK were asked to notify any deaths in children with epilepsies over a 10-month period. Hospital and community case notes were reviewed by pairs of case assessors using a structured assessment tool combining holistic and criterion-based approaches. RESULTS: Of 46 deaths notified, case notes were obtained on 33. The majority of children had associated developmental impairments. The majority (24), died of an associated co-morbidity rather than of epilepsy. Seven died of convulsive status epilepticus and seven as sudden unexpected deaths in epilepsy. Twenty four percent of deaths were judged to be preventable; potentially modifiable factors included fragmentation of care, support for families, and recognition of and response to acute illness in the child, including the appropriate management of prolonged seizures. CONCLUSIONS: Although this audit has demonstrated significant improvements in quality of care when compared with the last national audit of epilepsy deaths in 2002, further improvement is still required.

A review of the quality of care following prolonged seizures in 1-18 year olds with epilepsies.

PURPOSE: To review the quality of care of children and young people with epilepsies who, following a prolonged seizure, received high-dependency or intensive care. To identify and learn from clinical, organisational, management or personal issues that contributed to these admissions, in order to inform practice and improve clinical services for children across the UK. METHOD: Notifications were collected from consultant paediatricians in England, Wales, Scotland and Northern Ireland over a 10-month period. For all eligible cases a clinical questionnaire was sent to the notifying clinician. A sample of these cases were selected for a detailed case note review. Case notes were reviewed by paediatrician-nurse pairs using a purpose-built assessment tool derived from national guidelines. RESULTS: Data were collected from 135 clinical questionnaires, and 36 sets of case notes were reviewed. Findings were compared to national standards of care and emerging themes identified. There was evidence of good epilepsy management in many cases. In some cases there was evidence of a lack of clear emergency care plans, of delays in administration of emergency medication, and of deviation from established national guidelines. CONCLUSION: The findings of this review suggest there have been improvements in the care of children and young people with epilepsies presenting with prolonged seizures compared to previous studies. Nevertheless, further improvements are needed, particularly in communication with families and prompt administration of emergency medication.

Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility.

BACKGROUND: The evidence base for management of childhood epilepsy is poor, especially for the most common specific syndromes such as rolandic epilepsy (RE) and Panayiotopoulos syndrome (PS). Considerable international variation in management and controversy about non-treatment indicate the need for high quality randomised controlled trials (RCT). The aim of this study is, therefore, to describe current UK practice and explore the feasibility of different RCT designs for RE and PS. METHODS: We conducted an online survey of 590 UK paediatricians who treat epilepsy. Thirty-two questions covered annual caseload, investigation and management practice, factors influencing treatment, antiepileptic drug preferences and hypothetical trial design preferences. RESULTS: 132 responded (22%): 81% were paediatricians and 95% at consultant seniority. We estimated, annually, 751 new RE cases and 233 PS cases. Electroencephalography (EEG) is requested at least half the time in approximately 70% of cases; MRI brain at least half the time in 40%-65% cases and neuropsychological evaluation in 7%-8%. Clinicians reported non-treatment in 40%: main reasons were low frequency of seizures and parent/child preferences. Carbamazepine is the preferred older, and levetiracetam the preferred newer, RCT arm. Approximately one-half considered active and placebo designs acceptable, choosing seizures as primary and cognitive/behavioural measures as secondary outcomes. CONCLUSIONS: Management among respondents is broadly in line with national guidance, although with possible overuse of brain imaging and underuse of EEG and neuropsychological assessments. A large proportion of patients in the UK remains untreated, and clinicians seem amenable to a range of RCT designs, with carbamazepine and levetiracetam the preferred active drugs.

Guidelines, training, audit, and quality standards in children's epilepsy services: closing the loop.

There has been considerable evolution in epilepsy healthcare for children over the last decade in the United Kingdom. There has been no single explanation for this. The development of national clinical guidelines, locally delivered but nationally designed educational programmes, nation-wide clinical audit, clinical networks and development of designated services have all had complimentary roles in enabling the implementation of national recommendations for the development of epilepsy care. These models may be applicable to other healthcare settings outside the UK.

A pilot of clinical performance indicators for suspected childhood epilepsies.

PURPOSE: In response to continuing concerns regarding the quality and equality of care for children and young people, the British Paediatric Neurology Association (BPNA) has supported the development of practical and meaningful audit to support quality improvement. METHOD: In 2006, the Children's Epilepsy Workstream in Trent (CEWT) coordinated a retrospective multi-service audit of paediatric epilepsy care against NICE and SIGN guidelines. This aimed to both facilitate quality improvements for participating services and act as a pilot for future potential national audits. RESULTS: The audit was achieved in 4 hospital services using prospective and retrospective ascertainment methods. 12 performance indicators were applied to each cohort. Overall 54% (12/22) of children with epilepsy had input from a paediatrician with "expertise" and 23% (5/22) had input from an epilepsy specialist nurse. CONCLUSION: Audit can be developed for epilepsies that delivers standardised quality metrics against national recommendations. As well as supporting local quality improvement initiatives, comparative and aggregate data can be produced to potentially give regional and national perspectives. The results and experience describe the journey towards the 2009-2012 Epilepsy 12 UK multicentre epilepsy audit.

Evolution of the EEG in children with Rasmussen's syndrome.

PURPOSE: The early diagnosis of Rasmussen's syndrome (RS) is often difficult, with differentiation between RS and focal cortical dysplasia (FCD) at epilepsy onset problematic. This study reviewed electroencephalography (EEG) in the two conditions for early indicators of either pathology. METHODS: All children with either suspected RS or with unilateral FCD undergoing evaluation for epilepsy surgery between 1992 and 2005 were identified. Case notes and standard EEG recordings were reviewed. EEG findings were compared where available at <3, 3-6 months, and 3-5 years after seizure onset. KEY FINDINGS: Nineteen children with RS and 17 with FCD were ascertained. In EEG studies performed <3 months after seizure onset, 50% (5/10) of the RS group showed background abnormalities, with 80% of these (4/5) showing persistent high-amplitude delta activity over the affected hemisphere. This compared to 66% (6/9) of the FCD group with 17% (1/6) showing marked background asymmetry. By 3-6 months after seizure onset, independent interictal abnormalities over the nonaffected hemisphere were seen in 25% (2/8) of the RS group and by 3-5 years in 62% (5/8) compared to none in the FCD group at any time points measured. These independent contralateral interictal abnormalities were notably associated with a significant decline in cognitive skills over time. SIGNIFICANCE: No specific EEG changes at diagnosis of epilepsy were identified to help differentiate between RS and FCD. Emerging persistent delta activity over the affected hemisphere with contralateral normal background rhythms, followed in due course by independent interictal epileptiform abnormalities over the unaffected hemisphere may support the diagnosis of RS as the condition evolves, and highlight the risk of overall cognitive dysfunction.

Acute scoliosis in a 3-year-old boy.

The case describes the presentation of a fit and well 3-year-old boy to the emergency department of a district general hospital after he developed an acute scoliosis overnight. There was no history of trauma, his observations were normal and he had non-specific symptoms of lethargy and reduced appetite, but no fevers or respiratory distress. Bloods showed raised inflammatory markers and he was referred to orthopaedics as a septic disc as there was some spinal tenderness. An urgent MRI was considered initially but on further examination there was some reduced air entry on the left lung base which a chest radiograph confirmed as a left-sided pneumonia. A diagnosis of pneumonia and secondary functional scoliosis was made. The child was admitted under paediatrics and made a full recovery on antibiotics. At 8 weeks follow-up there was resolution of scoliosis clinically and radiologically.