Scoping review and expert-based consensus recommendations for assessment and management of psychogenic non-epileptic (functional) seizures (PNES) in children: A report from the Pediatric Psychiatric Issues Task Force of the International League Against Epilepsy.

Limited guidance exists regarding the assessment and management of psychogenic non-epileptic seizures (PNES) in children. Our aim was to develop consensus-based recommendations to fill this gap. The members of the International League Against Epilepsy (ILAE) Task Force on Pediatric Psychiatric Issues conducted a scoping review adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-SR) standards. This was supplemented with a Delphi process sent to pediatric PNES experts. Consensus was defined as ≥80% agreement. The systematic search identified 77 studies, the majority (55%) of which were retrospective (only one randomized clinical trial). The primary means of PNES identification was video electroencephalography (vEEG) in 84% of studies. Better outcome was associated with access to counseling/psychological intervention. Children with PNES have more frequent psychiatric disorders than controls. The Delphi resulted in 22 recommendations: Assessment-There was consensus on the importance of (1) taking a comprehensive developmental history; (2) obtaining a description of the events; (3) asking about potential stressors; (4) the need to use vEEG if available parent, self, and school reports and video recordings can contribute to a "probable" diagnosis; and (5) that invasive provocation techniques or deceit should not be employed. Management-There was consensus about the (1) need for a professional with expertise in epilepsy to remain involved for a period after PNES diagnosis; (2) provision of appropriate educational materials to the child and caregivers; and (3) that the decision on treatment modality for PNES in children should consider the child's age, cognitive ability, and family factors. Comorbidities-There was consensus that all children with PNES should be screened for mental health and neurodevelopmental difficulties. Recommendations to facilitate the assessment and management of PNES in children were developed. Future directions to fill knowledge gaps were proposed.

"Untold Distress" - How did the COVID-19 pandemic affect those who had previously experienced an epilepsy-related bereavement?

PURPOSE: This study explores the impact of the COVID-19 pandemic and lockdown on people with lived experience of sudden bereavement as a consequence of an epilepsy-related death. METHOD: We developed an online survey with fixed choice and open-ended response formats to collect data on grief symptoms and well-being during the pandemic. A total of 275 people bereaved by epilepsy-related deaths between 1980-2020 participated in this study: with 79 participants providing free-text responses for inductive thematic analysis. RESULTS: In total, 84% of participants reported a bereavement following a sudden death of a person aged under 40, with 22% aged 19 and under. The majority (77% of participants) reported they had been thinking more about the person who died compared to before the COVID-19 outbreak and 54% had experienced more distressing flashbacks to the time of death. Additionally, 61% reported more difficulties falling asleep and staying asleep and 88% of participants reported that the outbreak and response measures had negatively impacted upon their mental health. Medication was being taken for a diagnosed mental health condition by 33% of participants at the time of the study. We categorized these negative experiences during COVID in to four main-themes - 'Family', 'Lifestyle', 'Personal Well-being' and 'Health Services and Shielding Populations'. The 'Personal Well-being' theme was inextricably linked to grief symptoms including 'reflection on the death', 're-exposure to feeling', 'grief', 'salience of sudden deaths in the media' and 'inability to commemorate anniversaries and rituals'. These findings were consistent for bereaved people irrespective of the recency of the death. CONCLUSION: This study highlights the impact of the disruption caused by the pandemic on the grief-management of those bereaved by epilepsy-related death. Grief is not static and its management is connected to the psychosocial and formal support structures that were disrupted during the pandemic. The removal of these supports had an adverse effect upon the mental health and well-being of many bereaved. There is an urgent need for a system-wide transformation of epilepsy and mental health services to be inclusive of the needs and experiences of people impacted by sudden deaths in epilepsy and the contribution of the specialist service developed by families and clinicians to meet this gap.

Health checks for adults with intellectual disability and association with survival rates: a linked electronic records matched cohort study in Wales, UK.

OBJECTIVE: To examine if mortality rates are lower in people with intellectual disability who have had a health check compared with those who have not had health checks. SETTING: General practice records of 26 954 people with an intellectual disability in Wales between 2005-2017, of which 7650 (28.4%) with a health check were matched 1:2 with those without a health check. PRIMARY OUTCOME MEASURE: Office of National Statistics mortality data; a Cox regression was utilised to examine time to death adjusted for comorbidities and gender. RESULTS: Patients who had a health check were stratified by those who (1) had a confirmed health check, that is, Read Code for a health check (n=7650 (28.4 %)) and (2) had no evidence of receiving a health check in their medical record. Patients with a health check were matched for age at time of health check with two people who did not have a health check. The health check was associated with improved survival for those with autism or Down's Syndrome (HR 0.58 (95% CI 0.37 to 0.91) and HR 0.76 (95% CI 0.64 to 0.91), respectively). There was no evidence of improved survival for those diagnosed with diabetes or cancer. The people who had a health check were more likely to be older, have epilepsy and less likely to have autism or Down's syndrome. CONCLUSIONS: Health checks are likely to influence survival if started before a person is diagnosed with a chronic condition, especially for people with autism or Down's syndrome.

Determining the role and responsibilities of the Australian epilepsy nurse in the management of epilepsy: a study protocol.

INTRODUCTION: Epilepsy is a common neurological condition affecting between 3% and 3.5% of the Australian population at some point in their lifetime. The effective management of chronic and complex conditions such as epilepsy requires person-centred and coordinated care across sectors, from primary to tertiary healthcare. Internationally, epilepsy nurse specialists are frequently identified as playing a vital role in improving the integration of epilepsy care and enhancing patient self-management. This workforce has not been the focus of research in Australia to date. METHODS AND ANALYSIS: This multistage mixed-method study examines the role and responsibilities of epilepsy nurses, particularly in primary and community care settings, across Australia, including through the provision of a nurse helpline service. A nationwide sample of 30 epilepsy nurses will be purposively recruited via advertisements distributed by epilepsy organisations and through word-of-mouth snowball sampling. Two stages (1 and 3) consist of a demographic questionnaire and semistructured interviews (individual or group) with epilepsy nurse participants, with the thematic data analysis from this work informing the areas for focus in stage 3. Stage 2 comprises of a retrospective descriptive analysis of phone call data from Epilepsy Action Australia's National Epilepsy Line service to identify types of users, their needs and reasons for using the service, and to characterise the range of activities undertaken by the nurse call takers. ETHICS AND DISSEMINATION: Ethics approval for this study was granted by Macquarie University (HREC: 52020668117612). Findings of the study will be published through peer-reviewed journal articles and summary reports to key stakeholders, and disseminated through public forums and academic conference presentations. Study findings will also be communicated to people living with epilepsy and families.

Paediatric sudden unexpected death in epilepsy: A parental report cohort.

BACKGROUND: Sudden unexpected death in epilepsy (SUDEP) accounts for a large percentage of deaths in children with epilepsy. Contributing factors to paediatric SUDEP are incompletely understood. AIMS OF STUDY: The Epilepsy Deaths Register (EDR) is an anonymized register that compiles information on deaths related to epilepsy, across all ages and epilepsy classifications. Using the EDR, we sought to identify key risk factors for SUDEP in children to assist the development of preventive measures. METHODS: All registrations between the ages of 1 and 16 years were reviewed to identify definite or probable SUDEP. These cases were analysed to identify common demographics, comorbidities, monitoring, treatments and circumstances near to the deaths. RESULTS: We identified forty-six cases (27 males) of definite or probable SUDEP. Paediatric SUDEP is more common in a 12- to 16-year age group and in those with neuro-disability. Most paediatric SUDEP occurs during apparent sleep. There were four cases with a vagus nerve stimulator. SUDEP can occur early after the onset of seizures. CONCLUSIONS: This is the largest single cohort of SUDEP reported in children. Reports from caregivers can augment population data. Surveillance in sleep is a priority area of development.

The evolution of self-injurious behaviors in people with intellectual disability and epilepsy: A follow-up study.

PURPOSE: Longitudinal studies of the evolution of Self-Injurious Behaviors (SIBs) in people with Intellectual Disability (ID) and epilepsy are not common. This study aimed to analyze the evolution (in terms of remission and persistence) and changes in the type, localization, frequency, and intensity of SIBs. METHODS: SIBs were assessed in a sample of 52 people with ID and epilepsy, and re-evaluated after a seven-year interval, using the "Scale for the Assessment of Self-Injurious Behaviors". The scale was administered to caregivers (parents or health professionals) through a semi-structured interview conducted by a specifically trained psychologist. RESULTS: The most frequent types of SIBs identified were: self-biting, self-hitting with objects, self-hitting with hand, object-finger in cavities. The main localizations of SIBs were: hands, mouth, head and cheeks. SIBs were found to be maintained after seven years, for type, localization, frequency, and intensity, in 90.4% of the sample. SIB types were stable over time, as were the affected areas. Global SIB frequency and intensity scores were found to be unchanged. Finally, a positive correlation was found between the frequency of SIBs and levels of intellectual disability. SIBs (frequency and intensity) and seizure frequency showed no correlation. CONCLUSION: Given the negative impact of SIBs on the adaptation and quality of life of people with ID and epilepsy, we believe that further studies on biological, psychological and environmental aspects are needed in order to identify any potential factors that might explain the persistence of SIBs and to find effective interventions to reduce them.

Living safely with epilepsy: a key learning review.

This review focusses on counselling to reduce the risks of seizures in epilepsy patients. Risk reduction, whilst maximising the independence of persons with epilepsy, is a core element of epilepsy practice. The importance of the issue is reflected by the inclusion of risk assessment and reduction in the recent International League Against Epilepsy Curriculum for epileptology. This article addresses the key elements of epilepsy risk and provides practical guidance for counselling and risk reduction. A review of key publications was performed focusing on: (1) the risk of injury associated with seizures; (2) safety issues related to seizures and lifestyle; (3) SUDEP risk and avoidance; and (4) assessing individual risks. Risk is common in epilepsy and is multifactorial. Clinicians should be aware of the risk factors associated with different epilepsy features, such as seizure type, as well as those related to lifestyle and common indoor and outdoor activities. In addition, transcultural differences should be considered in risk assessment and counselling, e.g. regarding SUDEP or even driving, for which regulations vary across nations. Assessment of individual risk is evolving through a better understanding of risk factors and methodologies which can improve communication and empower patients to help identify and manage their individual risks.

Use of telehealth for facilitating the diagnostic assessment of Autism Spectrum Disorder (ASD): A scoping review.

There is a significant delay between seeking help and a confirmed diagnosis of Autism Spectrum Disorder (ASD). This delay can lead to poor outcomes for both the families and individuals. Telehealth potentially offers a way of improving the diagnostic pathway for ASD. We conducted a scoping review examining which telehealth approaches are used in the diagnosis and assessment of ASD in children and adults, whether they are feasible and acceptable, and how they compare with face-to-face diagnosis and assessment methods. A search for all peer-reviewed articles, combining the terms of autism and telehealth was conducted from 2000 to 2019. A total of 10 studies were identified for inclusion in the review. This review of the literature found there to be two methods of using telehealth: (a) Real-Time method e.g. video conferencing that enables teams in different areas to consult with the families and to assess the child/adult in real time and (b) A Store-and-Forward method as Naturalistic Observation Diagnostic Assessment (NODA) system to upload videos of child's behaviors to a webportal that enables the clinicians to make an assessment remotely. The findings were positive, finding there to be high agreement in terms of the diagnosis between remote methods and face to face methods and with high levels of satisfaction among the families and clinicians. This field is in the very early stages and so only studies with small sample size using surveys and interviews were identified but the findings suggest that there is potential for telehealth methods to improve access to assessment and diagnosis of ASD used in conjunction with existing methods, especially for those with clear autism traits and adults with ASD. Larger randomised controlled trials of this technology are warranted.

Determinants of health and wellbeing in refractory epilepsy and surgery: The Patient Reported, ImpleMentation sciEnce (PRIME) model.

This paper offers a new way of understanding the course of a chronic, neurological condition through a comprehensive model of patient-reported determinants of health and wellbeing: The Patient Reported ImpleMentation sciEnce (PRIME) model is the first model of its kind to be based on patient-driven insights for the design and implementation of initiatives that could improve tertiary, primary, and community healthcare services for patients with refractory epilepsy, and has broad implications for other disorders; PRIME focuses on: patient-reported determinants of health and wellbeing, pathways through care, gaps in treatment and other system delays, patient need and expectation, and barriers and facilitators to high-quality care provision; PRIME highlights that in the context of refractory epilepsy, patients value appropriate, clear, and speedy referrals from primary care practitioners and community neurologists to specialist healthcare professionals based in tertiary epilepsy centers. Many patients also want to share in decisions around treatment and care, and gain a greater understanding of their debilitating disease, so as to find ways to self-manage their illness more effectively and plan for the future. Here, PRIME is presented using refractory epilepsy as the exemplar case, while the model remains flexible, suitable for adaptation to other settings, patient populations, and conditions; PRIME comprises six critical levels: 1) The Individual Patient Model; 2) The Patient Relationships Model; 3) The Patient Care Pathways Model; 4) The Patient Transitions Model; 5) The Pre- and Postintervention Model; and 6) The Comprehensive Patient Model. Each level is dealt with in detail, while Levels 5 and 6 are presented in terms of where the gaps lie in our current knowledge, in particular in relation to patients' journeys through healthcare, system intersections, and individuals adaptive behavior following resective surgery, as well as others' views of the disease, such as family members.

Educational attainment of children born to mothers with epilepsy.

OBJECTIVE: Small prospective studies have identified that children exposed to valproate in utero have poorer scores on cognitive testing. We wanted to identify whether children exposed to antiepileptic drugs (AEDs) in utero have poorer school performance. METHODS: We used anonymised, linked, routinely collected healthcare records to identify children born to mothers with epilepsy. We linked these children to their national attainment Key Stage 1 (KS1) tests in mathematics, language and science at the age of 7 and compared them with matched children born to mothers without epilepsy, and with the national KS1 results. We used the core subject indicator (CSI) as an outcome measure (the proportion of children achieving a minimum standard in all subjects) and the results in individual subjects. RESULTS: We identified 440 children born to mothers with epilepsy with available KS1 results. Compared with a matched control group, fewer children with mothers being prescribed sodium valproate during pregnancy achieved the national minimum standard in CSI (-12.7% less than the control group), mathematics (-12.1%), language (-10.4%) and in science (-12.2%). Even fewer children with mothers being prescribed multiple AEDs during pregnancy achieved a national minimum standard: CSI (by -20.7% less than the control group), mathematics (-21.9%), language (-19.3%) and science (-19.4%). We did not observe any significant difference in children whose mothers were prescribed carbamazepine or were not taking an AED when compared with the control group. CONCLUSIONS: In utero exposure to AEDs in combination, or sodium valproate alone, is associated with a significant decrease in attainment in national educational tests for 7-year-old children compared with both a matched control group and the all-Wales national average. These results give further support to the cognitive and developmental effects of in utero exposure to sodium valproate as well as multiple AEDs, which should be balanced against the need for effective seizure control for women during pregnancy.

Validating epilepsy diagnoses in routinely collected data.

PURPOSE: Anonymised, routinely-collected healthcare data is increasingly being used for epilepsy research. We validated algorithms using general practitioner (GP) primary healthcare records to identify people with epilepsy from anonymised healthcare data within the Secure Anonymised Information Linkage (SAIL) databank in Wales, UK. METHOD: A reference population of 150 people with definite epilepsy and 150 people without epilepsy was ascertained from hospital records and linked to records contained within SAIL (containing GP records for 2.4 million people). We used three different algorithms, using combinations of GP epilepsy diagnosis and anti-epileptic drug (AED) prescription codes, to identify the reference population. RESULTS: Combining diagnosis and AED prescription codes had a sensitivity of 84% (95% ci 77-90) and specificity of 98% (95-100) in identifying people with epilepsy; diagnosis codes alone had a sensitivity of 86% (80-91) and a specificity of 97% (92-99); and AED prescription codes alone achieved a sensitivity of 92% (70-83) and a specificity of 73% (65-80). Using AED codes only was more accurate in children achieving a sensitivity of 88% (75-95) and specificity of 98% (88-100). CONCLUSION: GP epilepsy diagnosis and AED prescription codes can be confidently used to identify people with epilepsy using anonymised healthcare records in Wales, UK.

PNES around the world: Where we are now and how we can close the diagnosis and treatment gaps-an ILAE PNES Task Force report.

An international consensus clinical practice statement issued in 2011 ranked psychogenic nonepileptic seizures (PNES) among the top three neuropsychiatric problems. An ILAE PNES Task Force was founded and initially charged with summarizing the current state of the art in terms of diagnosis and treatment, resulting in two publications. The first described different levels of diagnostic certainty. The second summarized current knowledge of management approaches. The present paper summarizes an international workshop of the ILAE PNES Task Force that focused on the current understanding and management of PNES around the world. We initially provide a knowledge update about the etiology, epidemiology, and prognosis of PNES-in adults and in special patient groups, such as children, older adults, and those with intellectual disability. We then explore clinical management pathways and obstacles to optimal care for this disorder around the world by focusing on a number of countries with different cultural backgrounds and at very different stages of social and economic development (United Kingdom, U.S.A., Zambia, Georgia, China, and Japan). Although evidence-based methods for the diagnosis and treatment of PNES have now been described, and much is known about the biopsychosocial underpinnings of this disorder, this paper describes gaps in care (not only in less developed countries) that result in patients with PNES not having adequate access to healthcare provisions. A range of challenges requiring solutions tailored to different healthcare systems emerges. Continued attention to PNES by the ILAE and other national and international neurologic, psychiatric, and health organizations, along with ongoing international collaboration, should ensure that patients with PNES do not lose out as healthcare services evolve around the world.

Perspectives of family members of people with an intellectual disability to a major reconfiguration of living arrangements for people with intellectual disability in Ireland.

AIM: To document the views of family members of people with an intellectual disability regarding implementation of a personalized model of social support in Ireland. METHOD: Forty family members participated in six focus groups. Data were thematically analysed. RESULTS: Family members' preference for particular types of living arrangements were highly reflective of their lived experience. Facilitators to community living included timely information on proposed moves, adequate staffing, suitable properties and locations and consideration of the characteristics of individuals who share a property. Barriers included high support needs, advanced age, a fear of relinquishing current supports, a fear of the sustainability of newer models of residential support and concerns about community opposition. CONCLUSION: The family perspective to reform is characterized by fear and suspicion of the motivation behind these reforms, with cost efficiencies being perceived as a main driver. Greater information is required to empower families to make informed decisions.

The implications of the new approach to classification: Adults with an intellectual disability.

The new ILAE classification offers the potential for clarity and improved translation of the understanding of the nature of epilepsy in people with an intellectual disability. This is particularly true in the use of the term genetic epilepsy and the removal of the term cryptogenic. However, the definition of the "dyscognitive" nature of seizures needs greater definition in those with coexistent cognitive impairment. This is of particular importance when ameliorating risk associated with impaired consciousness. This article is part of a Special Issue entitled "The new approach to classification: Rethinking cognition and behavior in epilepsy".

Severe Intellectual Disability: Systematic Review of the Prevalence and Nature of Presentation of Unipolar Depression.

BACKGROUND: The diagnosis of depression in severe and profound intellectual disability is challenging. Without adequate skills in verbal self-expression, standardized diagnostic criteria cannot be used with confidence. The purpose of this systematic review was to investigate the assessment and diagnosis of unipolar depression in severe and profound intellectual disability. The review aimed to examine the methods used to assess for depression. The secondary aim was to explore the frequency and symptoms of depression. METHODS: The PRISMA (2009) Checklist for systematic review was followed, and a search of electronic databases was undertaken. Nine studies were included in the qualitative synthesis from over 2000 records identified. RESULTS: The quality of the studies was assessed and scored, with a wide range of results. Individual studies scored between 2 and 7 of a maximum possible score of 8. The diagnostic tools utilized by each of the studies were assessed and compared. CONCLUSIONS: In terms of the methods used to assess for depression, results were varied. This was due to the heterogeneous nature of the individual study designs. The Aberrant Behaviour Checklist consistently showed promise, in particular when combined with other instruments or clinical examination. Qualitative analysis of the selected studies has shown a wide variation in the quality of primary research in this field, with more required to make firm conclusions regarding the diagnosis, frequency and presentation of depression in severe and profound intellectual disability.

Behavioral disorder in people with an intellectual disability and epilepsy: A report of the Intellectual Disability Task Force of the Neuropsychiatric Commission of ILAE.

The management and needs of people with intellectual disability (ID) and epilepsy are well evidenced; less so, the comorbidity of behavioral disorder in this population. "Behavioral disorder" is defined as behaviors that are difficult or disruptive, including stereotypes, difficult or disruptive behavior, aggressive behavior toward other people, behaviors that lead to injury to self or others, and destruction of property. These have an important link to emotional disturbance. This report, produced by the Intellectual Disability Task Force of the Neuropsychiatric Commission of the ILAE, aims to provide a brief review of some key areas of concern regarding behavioral disorder among this population and proposes a range of research and clinical practice recommendations generated by task force members. The areas covered in this report were identified by experts in the field as being of specific relevance to the broad epilepsy community when considering behavioral disorder in persons with epilepsy and ID; they are not intended to be exhaustive. The practice recommendations are based on the authors' review of the limited research in this field combined with their experience supporting this population. These points are not graded but can be seen as expert opinion guiding future research and clinical practice.

Epilepsy and deprivation, a data linkage study.

OBJECTIVE: To investigate whether the link between epilepsy and deprivation is due to factors associated with deprivation (social causation) or factors associated with a diagnosis of epilepsy (social drift). METHODS: We reviewed electronic primary health care records from 2004 to 2010, identifying prevalent and incident cases of epilepsy and recording linked deprivation scores. Logistic and Poisson regression models were used to calculate odds ratios and incidence rate ratios. The change in deprivation was measured 10 years after the initial diagnosis of epilepsy for a cohort of people. RESULTS: Between 2004 and 2010, 8.1 million patient-years of records were reviewed. Epilepsy prevalence and incidence were significantly associated with deprivation. Epilepsy prevalence ranged from 1.13% (1.07-1.19%) in the most deprived decile to 0.49% (0.45-0.53%) in the least deprived decile (adjusted odds ratio 0.92, p < 0.001). Epilepsy incidence ranged from 40/100,000 per year in the most deprived decile to 19/100,000 per year in the least deprived decile (adjusted incidence rate ratio 0.94, p < 0.001). There was no statistically significant change in deprivation index decile 10 years after a new diagnosis of epilepsy (mean difference -0.04, p = 0.85). SIGNIFICANCE: Epilepsy prevalence and incidence are strongly associated with deprivation; the deprivation score remains unchanged 10 years after a diagnosis of epilepsy. These findings suggest that increasing rates of epilepsy in deprived areas are more likely explained by social causation than by social drift. The nature of the association between incident epilepsy and social deprivation needs further exploration.