National Association of Medical Examiners position paper: Recommendations for the investigation and certification of deaths in people with epilepsy.

Sudden unexpected death of an individual with epilepsy can pose a challenge to death investigators, as most deaths are unwitnessed, and the individual is commonly found dead in bed. Anatomic findings (eg, tongue/lip bite) are commonly absent and of varying specificity, thereby limiting the evidence to implicate epilepsy as a cause of or contributor to death. Thus it is likely that death certificates significantly underrepresent the true number of deaths in which epilepsy was a factor. To address this, members of the National Association of Medical Examiners, North American SUDEP Registry, Epilepsy Foundation SUDEP Institute, American Epilepsy Society, and the Centers for Disease Control and Prevention constituted an expert panel to generate evidence-based recommendations for the practice of death investigation and autopsy, toxicological analysis, interpretation of autopsy and toxicology findings, and death certification to improve the precision of death certificate data available for public health surveillance of epilepsy-related deaths. The recommendations provided in this paper are intended to assist medical examiners, coroners, and death investigators when a sudden unexpected death in a person with epilepsy is encountered.

National Association of Medical Examiners Position Paper: Recommendations for the Investigation and Certification of Deaths in People with Epilepsy.

Sudden unexpected death of an individual with epilepsy (SUDEP) can pose a challenge to death investigators, as most deaths are unwitnessed and the individual is commonly found dead in bed. Anatomic findings (e.g., tongue/lip bite) are commonly absent and of varying specificity, limiting the evidence to implicate epilepsy as a cause of or contributor to death. Thus, it is likely that death certificates significantly underrepresent the true number of deaths in which epilepsy was a factor. To address this, members of the National Association of Medical Examiners, North American SUDEP Registry, Epilepsy Foundation SUDEP Institute, American Epilepsy Society, and the Centers for Disease Control and Prevention convened an expert panel to generate evidence-based recommendations for the practice of death investigation and autopsy, toxicological analysis, interpretation of autopsy and toxicology findings, and death certification to improve the precision of death certificate data available for public health surveillance of epilepsy-related deaths. The recommendations provided in this paper are intended to assist medical examiners, coroners, and death investigators when a sudden, unexpected death in a person with epilepsy is encountered.

A survey of medical examiner death certification of vignettes on death in epilepsy: Gaps in identifying SUDEP.

OBJECTIVE: Lack of standardized terminology on death certificates (DCs) of SUDEP type cases may obscure the presence of epilepsy in these deaths. Most DCs for individuals dying unexpectedly with epilepsy are certified by medical examiners (MEs). The purpose of this study was to gauge death certification practices of MEs when interpreting SUDEP cases and assess implications for valid surveillance of SUDEP. MATERIALS AND METHODS: A survey consisting of clinical vignettes describing deaths in individuals with epilepsy was sent to medical examiners. Respondents were asked to indicate how they would certify death on a DC. Similar text responses were aggregated and coded according to the 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) coding system. RESULTS: A total of 847 responses on 11 cases were received. Depending upon the vignette, the proportion of responses within each case that did not have an ICD-10 seizure code ranged from 3% to 62%. G40.9 (Epilepsy, unspecified) resulted from 43% of responses, and R56.8 (Other and unspecified convulsion) resulted from 38% of responses. CONCLUSION: The survey indicates that a high proportion of DCs do not have a seizure code and would not be identified utilizing these ICD-10 codes. The complicated nature of deaths in SUDEP, unclear circumstances surrounding a given death, and the lack of familiarity with SUDEP by surviving relatives may all contribute to variable terminology used to certify SUDEP deaths. Our results emphasize the need for collaboration between neurologists and forensic pathologists to develop a more uniform approach to death certification in SUDEP that will facilitate SUDEP research and inform relatives of individuals who die of SUDEP.

Implications of Death Certification on Sudden Unexpected Death in Epilepsy (SUDEP) Research.

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in individuals with chronic, uncontrolled epilepsy. Epidemiologists use information on death certificates to study SUDEP. Certification of seizure-related deaths varies. Multiple classification schemes have been proposed to categorize SUDEP type deaths. Nashef et al. recently proposed categorizing death into Definite SUDEP, Definite SUDEP Plus, Probable SUDEP, Possible SUDEP, Near-SUDEP, and Not SUDEP. This study analyzes certification of seizure-related deaths by our office and considers how it relates to Nashef's classifications. Investigative reports from 2011-2015 from the archives of the Jefferson County Coroner/Medical Examiner's Office were searched for the terms "seizure(s)" and "epilepsy." Cases (N=61) were categorized as Definite SUDEP (n=13), Definite SUDEP Plus (n=12), Probable SUDEP (n=1), Possible SUDEP (n=2), and Not SUDEP (n=33). The term SUDEP was only used in one case of Definite SUDEP. The other 12 cases were certified with variations of terms "seizure" and "epilepsy." Cases categorized as Definite SUDEP Plus were overwhelmingly certified as deaths due to heart disease. Categories Probable SUDEP or Possible SUDEP comprised three cases, and in one of those a seizure-related term was used on the death certificate. Thirty-three cases were classified as Not SUDEP. The finding that the majority of cases of Definite SUDEP were certified as some variation of "seizure" or "epilepsy" but not "SUDEP" has important implications for SUDEP research. Our study also suggests that cases of Definite SUDEP Plus would be difficult for epidemiologists to identify because cardiovascular diseases are more frequently implicated.

Characteristics of epilepsy patients and caregivers who either have or have not heard of SUDEP.

OBJECTIVE: Describe the characteristics of persons with epilepsy (PWEs) and caregivers that have or have not heard of sudden unexpected death in epilepsy (SUDEP) prior to completing a survey through the Internet or in the clinical setting. METHODS: An online survey for adult PWEs and caregivers was solicited by e-mail and newsletter to Epilepsy Therapy Project members. A similar survey was implemented in a clinic setting of a community hospital. The survey asked about seizure characteristics, epilepsy management, fear of death, and familiarity with the term SUDEP. Respondents that never heard of SUDEP read a definition and responded to questions about their initial reactions. RESULTS: Surveys from 1,392 PWEs and 611 caregivers recruited through an epilepsy Website and a clinic demonstrated that Internet respondents were much more likely to have heard about SUDEP than the clinic population (71.1% vs. 38.8%; p < 0.001), and caregivers of PWEs were more likely to have heard about SUDEP than PWEs (76.2% vs. 65.2%; p < 0.001). Prior awareness was related to an increased level of education, more severe and longer duration of epilepsy, and having an epileptologist as the primary care provider. Although most PWEs and caregivers reported feelings of fear, anxiety, and sadness after first hearing of SUDEP, they wanted to discuss it with their doctor. Persons with epilepsy, and especially their caregivers, often worry that the PWEs may die of epilepsy or seizures. This worry escalated with knowledge of SUDEP and increased epilepsy severity. Approximately half of PWEs and caregivers believed that knowledge about SUDEP would influence their epilepsy management. SIGNIFICANCE: Our results may help epilepsy care providers determine when to facilitate a discussion about epilepsy-related mortality and SUDEP among patients and caregivers, and to educate those at high risk about the importance of seizure control as well as reduce fears about death in patients with well-controlled and nonconvulsive epilepsies.

Sudden unexpected death in epilepsy: knowledge and experience among U.S. and Canadian neurologists.

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 24 months (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.