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The devastating effects of sudden unexpected death in epilepsy ( SUDEP) can be difficult to navigate, even for experienced clinicians. Mounting evidence supports full disclosure of the risks of epilepsy to those affected and their caregivers, and recommendations from regulatory and professional groups encourage the same. Following a death, families are faced with tragedy, guilt, and sometimes anger. Clinicians are often called upon to provide information and support. The development of a comprehensive approach to SUDEP education requires careful consideration of the people living with epilepsy, facts about SUDEP and known risk factors, as well as experiences of families and care providers. In this article, we share the experiences of those working in SUDEP education and epilepsy care, including the voluntary sector. We explore the experience of bereaved families and clinicians, derive lessons from published research, highlight areas where more research is needed, and report on preliminary data from a nationwide study from France. [ABSTRACT FROM AUTHOR]

Abstract: Importance: Sudden unexpected death in epilepsy (SUDEP) is a common cause of mortality in patients with the disease, but it is unknown how neurologists disclose this risk when counseling patients. Objective: This study aimed at examining SUDEP discussion practices of neurologists in the U.S. and Canada. Design: An electronic, web-based survey was sent to 17,558 neurologists in the U.S. and Canada. Survey questions included frequency of SUDEP discussion, reasons for discussing/not discussing SUDEP, timing of SUDEP discussions, and perceived patient reactions. We examined factors that influence the frequency of SUDEP discussion and perceived patient response using multivariate logistic regression. Participants: The participants of this study were neurologists who completed postgraduate training and devoted >5% of their time to patient care. Results: There was a response rate of 9.3%; 1200 respondents met eligibility criteria and completed surveys. Only 6.8% of the respondents discussed SUDEP with nearly all (>90% of the time) of their patients with epilepsy/caregivers, while 11.6% never discussed it. Factors that independently predicted whether SUDEP was discussed nearly all of the time were the following: number of patients with epilepsy seen annually (OR=2.01, 95% CI=1.20–3.37, p <0.01) and if the respondent had a SUDEP case in the past 24months (OR=2.27, 95% CI=1.37–3.66, p <0.01). A majority of respondents (59.5%) reported that negative reactions were the most common response to a discussion of SUDEP. Having additional epilepsy/neurophysiology training was associated with an increased risk of a perceived negative response (OR=1.36, 95% CI=1.02–1.82, p =0.038), while years in practice (OR=0.85, 95% CI=0.77–0.95, p <0.005) and seeing both adults and children were associated with a decreased likelihood of negative response (OR=0.15, 95% CI=0.032–0.74, p =0.02). Conclusions: U.S. and Canadian neurologists rarely discuss SUDEP with all patients with epilepsy/caregivers though discussions are more likely among neurologists who frequently see patients with epilepsy or had a recent SUDEP in their practice. Perceived negative reactions to SUDEP discussions are common but not universal; more experienced neurologists may be less likely to encounter negative reactions, suggesting that there may be ways to frame the discussion that minimizes patient/caregiver distress. [Copyright &y& Elsevier]

Sudden death is over 20 times more frequent in people with epilepsy than the general population. The literature on clinical risk factors is now able to define individuals at the highest risk. Despite these advances in our understanding of risk, the mechanism of sudden unexpected death in epilepsy remains elusive. While it is unlikely that a single mechanism will be found to explain all deaths, there have been recent advances that identify factors that play a critical role. This review provides an update on new advances in the understanding of sudden unexpected death in epilepsy. [ABSTRACT FROM AUTHOR]

Sudden unexpected death in epilepsy (SUDEP) is the most tragic potential outcome of epilepsy. Despite recommendations from epilepsy organizations in the United Kingdom and the United States, many neurologists choose not to discuss the risk of SUDEP with their patients with epilepsy. Yet, the literature clearly demonstrates that people with epilepsy and their caregivers want to know more about SUDEP. When health care providers do not provide information, people with epilepsy turn to other sources, risking misinformation and potentially increasing anxiety and distress. Sharing accurate information about SUDEP can optimize epilepsy self-management and engage the person with epilepsy as a partner in their own care. Information about SUDEP must be part of the comprehensive education given to all people with epilepsy. [ABSTRACT FROM AUTHOR]

We compared sudden unexpected death in epilepsy (SUDEP) diagnosis rates between North American SUDEP Registry (NASR) epileptologists and original death investigators, to determine degree and causes of discordance. In 220 SUDEP cases with post‐mortem examination, we recorded the epileptologist adjudications and medical examiner‐ and coroner‐ (ME/C) listed causes of death (CODs). COD diagnosis concordance decreased with NASR's uncertainty in the SUDEP diagnosis: highest for Definite SUDEP (84%, n = 158), lower in Definite Plus (50%, n = 36), and lowest in Possible (0%, n = 18). Rates of psychiatric comorbidity, substance abuse, and toxicology findings for drugs of abuse were all higher in discordant cases than concordant cases. Possible SUDEP cases, an understudied group, were significantly older, and had higher rates of cardiac, drug, or toxicology findings than more certain SUDEP cases. With a potentially contributing or competing COD, ME/Cs favored non–epilepsy‐related diagnoses, suggesting a bias toward listing CODs with structural or toxicological findings; SUDEP has no pathognomonic features. A history of epilepsy should always be listed on death certificates and autopsy reports. Even without an alternate COD, ME/Cs infrequently classified COD as "SUDEP." Improved collaboration and communication between epilepsy and ME/C communities improve diagnostic accuracy, as well as bereavement and research opportunities. [ABSTRACT FROM AUTHOR]

Abstract Background Sudden Unexpected Death in Epilepsy (SUDEP) is a significant cause of death in childhood epilepsy, and causes considerable concern to patients and their families. Despite this, the condition remains poorly understood. This systematic review investigates the risk factors, pathophysiology, and circumstances associated with childhood SUDEP. It aimed to explore the etiology of SUDEP and inform clinicians approaching SUDEP risk disclosure. Methods A structured electronic database search of MEDLINE, CENTRAL, EMBASE, and ISI web of science was conducted. Studies were included if they described clinical details of one or more patients, aged 18 years of age and below, who had SUDEP. Two reviewers independently reviewed each article for data extraction and quality assessment. Results Information on 108 cases of pediatric SUDEP was extracted from 22 included studies. These comprised five cohort studies, four retrospective case control studies, seven case series, and five case reports. Factors that appeared to be linked to pediatric SUDEP included those associated with severe epilepsy (early age of onset, high seizure frequency, intellectual impairment and developmental delay, multiple antiepileptic drug therapy, and structural abnormalities). The majority of included studies was noncomparative and had significant risk of bias. Conclusions There is currently insufficient evidence to determine the etiology of pediatric SUDEP. Current best practice to prevent pediatric SUDEP is to optimize the management of epilepsy. A national SUDEP registry would provide invaluable high-quality data and insights into modifiable risk factors, genetic predispositions, and novel prevention strategies. Highlights • Very little is known about the pathophysiology, risk factors and circumstances associated with childhood SUDEP. • In this systematic review, 22 studies were included with information on 108 cases of pediatric SUDEP. • Factors which appeared to be linked to pediatric SUDEP included those associated with severe epilepsy. • This is the first systematic review of childhood SUDEP in the literature, drawn from analysis of the largest compilation of pediatric SUDEP cases. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 14-year-old girl and 12-year-old boy suffering from epilepsy. The girl had history of myoclonic jerks and diagnosed with juvenile myoclonic epilepsy for which she was treated with valproic acid. The boy had history of drug-resistant epilepsy, moderately severe cognitive impairment and features of autism. It further mentions their death due to seizure.

Objective: Sudden unexpected death in epilepsy (SUDEP) is a diagnosis of exclusion; the definition includes individuals with epilepsy who die suddenly without an identifiable toxicological or anatomical cause of death. Limited data suggest underidentification of SUDEP as the cause of death on death certificates. Here, we evaluate the autopsy‐reported cause of death in a population‐based cohort of SUDEP cases. Methods: Case summaries of forensic autopsies conducted in Ontario, Canada between January 2014 and June 2016 were retrospectively screened using a language processing script for decedents with a history of epilepsy or seizures. After manual review for potential SUDEP cases, two neurologists independently examined the autopsy reports and classified deaths by Nashef criteria. Demographic characteristics and consideration by the forensic pathologist of the role of epilepsy, seizure, and SUDEP in death were summarized. Results: One hundred and eight Definite, 34 Definite Plus, and 22 Possible SUDEP cases were identified. Seventy‐five percent of Definite/Definite Plus SUDEP cases identified by the neurologists were attributed to SUDEP, epilepsy, or seizure disorder in the autopsy report. There was a significant association between the proportion of cases listed in the autopsy report as SUDEP, epilepsy, or seizure disorder and neurologists' SUDEP classification (86% of Definite, 38% of Definite Plus, 0% of Possible). Age was significantly associated with SUDEP classification; Definite cases were younger than Definite Plus, which were younger than Possible SUDEP cases. Significance: Most SUDEP cases identified by neurologists were classified concordantly by forensic pathologists in Ontario, Canada; however, concordance decreased with increased case complexity. Although the role of epilepsy/seizures was considered in most Definite/Definite Plus cases, this study highlights the need for autopsy report review of potential SUDEP cases in research studies and assessments of the public health burden of SUDEP. The relationship between age and SUDEP classification has important public health implications; SUDEP incidence may be underappreciated in older adults. [ABSTRACT FROM AUTHOR]

Preclinical data report within subject modifiable ailments emerge weeks prior to SUDEP, including sleep disorders and cardiorespiratory changes; findings which support anecdotal clinical data. Here, we bridge preclinical findings with future clinical/preclinical studies, and survey whether caretakers or family members of victims noticed transient changes prior to SUDEP. The aim of this pilot study is to identify potential modifiable changes that may synergistically increase SUDEP risk for future research. A mobile electronic survey was posted on SUDEP community websites. The survey queried whether changes in seizures, sleep, physical well-being, emotional well-being, cognition, breathing, or heart rate were noticed before SUDEP. The most profound finding was that 85% of victims had multiple transient ailments prior to SUDEP. Changes in seizures (28/54), and sleep (30/58) occurred in more than 50% of the victims and represent the most influential changes identified. The second and third most influential changes were a reduction in physical well-being (25/57) and emotional well-being (26/56). Changes were observed within the last two months of life in approximately one third of the cases, and more than four months prior to SUDEP in approximately one third of cases, indicating a potential time frame for proactive preventative strategies. Respondents also noted changes in cognition (16/55), breathing (9/54) or heart rate (8/55). Data indicate these changes may be associated with increased SUDEP risk within subject. Study limitations include the responses were based on memory, there was a potential for data to be over reported, and caretakers were not prompted to observe changes a priori, thus some existing changes may have gone unnoticed. Data support the preclinical findings that transient, subclinical (i.e., not severe enough to require medical intervention), modifiable ailments may increase risk of SUDEP. This suggests that just as an epilepsy type can change over a lifetime and epilepsy type-specific treatments can reduce SUDEP risk, further personalization of SUDEP risk will improve our understanding as to whether variables contribute to risk differently across lifespan. Thus, with a dynamic capacity to change, differing factors may contribute to the distribution of risk probability within an individual at any given time. Understanding whether different combinations of transient changes are specific to epilepsy type, age, or sex needs to be determined to move the field forward in hopes of developing a personalized approach to preventative strategies. • Loved ones of victims were asked whether they noticed any changes before SUDEP. • 85% of SUDEP victims had multiple subclinical comorbidities prior to SUDEP. • Seizure worsening and sleep problems emerged in the months prior to SUDEP in more than half of the victims. • Changes in emotional and physical well-being were observed in more than 40% of SUDEP cases. • Whether changes are specific to epilepsy type needs to be determined to move towards personalized preventative strategies. [ABSTRACT FROM AUTHOR]

Summary: Objective: To examine the consistency of applying the Nashef et al (2012) criteria to classify sudden unexpected death in epilepsy (SUDEP). Methods: We reviewed cases from the North American SUDEP Registry (n = 250) and Medical Examiner Offices (n = 1301: 698 Maryland, 457 New York City, 146 San Diego). Two epileptologists with expertise in SUDEP and epilepsy‐related mortality independently reviewed medical records, scene investigation, autopsy, and toxicology and assigned a SUDEP class. Results: Major areas of disagreement arose between adjudicators concerned differentiating (1) Definite SUDEP Plus Comorbidity from Possible SUDEP and (2) Resuscitated (Near) SUDEP from SUDEP. In many cases, distinguishing between contributing and competing causes of death when trying to classify Definite SUDEP Plus Comorbidity versus Possible SUDEP is ambiguous and relies on judgement. Similarly, determining if an intervention was lifesaving or not (Resuscitated SUDEP or Not SUDEP), or if resuscitation merely delayed SUDEP (Resuscitated SUDEP or SUDEP) is often a judgement call and can differ between experienced adjudicators. Given these persisting ambiguities, we propose more explicit criteria for distinguishing these categories. Significance: Accurate and consistent classification of cause of death among individuals with epilepsy remains a dire public health concern. SUDEP is likely underestimated in national health statistics. Greater standardization of criteria among epilepsy researchers, medical examiners, and epidemiologists to determine cause and classify death will lead to more accurate tracking of SUDEP and other epilepsy‐related mortalities. [ABSTRACT FROM AUTHOR]

The North American SUDEP Registry (NASR) is a repository of clinical data and biospecimens in cases of sudden unexpected death in epilepsy (SUDEP), a leading cause of epilepsy-related deaths. We assessed whether bereaved families were aware of SUDEP before their family member's death and their preferences for SUDEP disclosure. At enrollment, next-of-kin of SUDEP cases completed an intake interview, including questions assessing premorbid SUDEP discussions. Only 18.1% of the 138 next-of-kin recalled a previous discussion of SUDEP with a healthcare provider or support resource. Of the 112 who did not recall such a discussion, 72.3% wished it was discussed, 10.7% were satisfied it was not discussed, and 17% were unsure. A history of status epilepticus predicted SUDEP discussion. Rates of SUDEP discussion were not significantly higher among SUDEPs after 2013 (the approximate study midpoint) compared with those before then. Our study suggests that SUDEP remains infrequently discussed with family members of persons with epilepsy. Nearly three-quarters of family members wished they had known of SUDEP before the death. However, some were indifferent or were satisfied that this discussion had not occurred. We must balance more systematic education of patients and families about SUDEP while respecting individual preferences about having this discussion. [ABSTRACT FROM AUTHOR]

Objective: To determine the incidence rates of sudden unexpected death in epilepsy (SUDEP) in different epilepsy populations and address the question of whether risk factors for SUDEP have been identified. Methods: Systematic review of evidence; modified Grading Recommendations Assessment, Development and Evaluation process for developing conclusions; recommendations developed by consensus. Results: Findings for incidence rates based on 12 Class I studies include the following: SUDEP risk in children with epilepsy (aged 0-17 years) is 0.22/1,000 patient-years (95% CI 0.16-0.31) (high confidence in evidence). SUDEP risk increases in adults to 1.2/1,000 patient-years (95% CI 0.64-2.32) (low confidence in evidence). The major risk factor for SUDEP is the occurrence of generalized tonic-clonic seizures (GTCS); the SUDEP risk increases in association with increasing frequency of GTCS occurrence (high confidence in evidence). Recommendations: Level B: Clinicians caring for young children with epilepsy should inform parents/guardians that in 1 year, SUDEP typically affects 1 in 4,500 children; therefore, 4,499 of 4,500 children will not be affected. Clinicians should inform adult patients with epilepsy that SUDEP typically affects 1 in 1,000 adults with epilepsy per year; therefore, annually 999 of 1,000 adults will not be affected. For persons with epilepsy who continue to experience GTCS, clinicians should continue to actively manage epilepsy therapies to reduce seizures and SUDEP risk while incorporating patient preferences and weighing the risks and benefits of any new approach. Clinicians should inform persons with epilepsy that seizure freedom, particularly freedom from GTCS, is strongly associated with decreased SUDEP risk. [ABSTRACT FROM AUTHOR]

Our aim was to describe the risk factors known to be related to sudden unexpected death in epilepsy (SUDEP) that can be extracted from patients that utilizes an online seizure diary tool (SeizureTracker™). We conducted a descriptive analysis of SeizureTracker™ users across factors relevant to SUDEP risk. We also compared our app-using cohort to published SUDEP case-control studies. We report across seven risk factors from 30,813 users of SeizureTracker™ who had a median length of time using the app of 5.69 years (range from 1 month to 15 years). We found that they are at greater risk for SUDEP than groups from published studies (p <.00001) based on the risk factor of generalized tonic-clonic seizures. We demonstrated that the population using the SeizureTracker™ tool can be a valuable population for expanding investigation of SUDEP risk factors and is a first step towards establishing a large sample with a method to ascertain data prospectively that might be critical to developing a SUDEP risk algorithm. • Risk factors related to SUDEP that can be obtained from an epilepsy cohort that utilizes an online seizure diary tool. • We found that this app-using cohort is at risk for SUDEP based on number of GTCs, even more than published studies. • This is a viable approache to expanding our investigation of SUDEP risk factors. [ABSTRACT FROM AUTHOR]

Invitation to participate in a prospective case-control study of sudden unexpected death in epilepsy New people with epilepsy can join the cohort during the course of the study, and only people who are alive at the time the study starts can be included. The EpiNet study group is undertaking a case-control study of sudden unexpected death in epilepsy (SUDEP) and is inviting physicians looking after people with epilepsy to participate. [Extracted from the article]

The American Epilepsy Society and the Epilepsy Foundation jointly convened a task force to assess the state of knowledge about sudden unexplained death in epilepsy (SUDEP). The task force had five charges: (1) develop a position statement describing if, when, what, and how SUDEP should be discussed with patients and their families and caregivers; (2) design methods by which the medical and lay communities become aware of the risk of SUDEP; (3) recommend research directions in SUDEP; (4) explore steps that organizations can take to perform large-scale, prospective studies of SUDEP to identify risk factors; and (5) identify possible preventive strategies for SUDEP. Some of the major task force recommendations include convening a multidisciplinary workshop to refine current lines of investigation and to identify additional areas of research for mechanisms underlying SUDEP; performing a survey of patients and their families and caregivers to identify effective means of education that will enhance participation in SUDEP research; conducting a campaign aimed at patients, families, caregivers, coroners, and medical examiners that emphasizes the need for complete autopsy examinations for patients with suspected SUDEP; and securing infrastructure grants to fund a consortium of centers that will conduct prospective clinical and basic research studies to identify preventable risk factors and mechanisms underlying SUDEP. For now, the principal effort in preventing SUDEP should be prompt and optimal control of seizures, especially generalized convulsive seizures. [ABSTRACT FROM AUTHOR]