It is known that about 1 in 150 persons with poorly controlled epilepsy dies of SUDEP (sudden unexpected death in epilepsy) every year. Reports of the few cases of SUDEP that have occurred in patients admitted to hospitals and epilepsy monitoring units have identified several seizure-related mechanisms for SUDEP, including breathing difficulties, heart arrhythmias, and prolonged coma following seizures, thus leading to impaired protective reflexes. However, these few cases may not be a representative group of all those who die from SUDEP because of the artificial circumstances surrounding their death, including tonic-clonic seizures induced by rapid withdrawal of medication during hospitalization. There are cases of SUDEP in people without known tonic-clonic seizures or that have seemingly good seizure control. A better understanding of the true incidence, mechanisms and risks for SUDEP requires a population-based approach to the condition. That is, by examining all of the SUDEP cases that occur in a predetermined geographic area, one obtains a more accurate understanding of the risk factors and possible mechanisms, than if one only looked at cases that occurred at a few epilepsy centers. In addition, population-based studies are the best way to get an accurate estimate of the true incidence of SUDEP.
While it is the ideal way to study a disease or outcome, population-based studies of SUDEP are difficult to conduct, especially in the United States. Medical examiners or coroners investigate most sudden and unexpected deaths. However, there is a great deal of misunderstanding among the death investigation community regarding SUDEP. A survey undertaken by Paul Schraeder and colleagues showed that while many coroners and medical examiners acknowledge that SUDEP is a legitimate cause of death, only a minority uses the diagnosis on the death certificate. This makes it very difficult to get an accurate assessment of the true incidence of SUDEP in the general population. Furthermore, medical examiners, especially those who practice in urban areas, are more likely to be familiar with SUDEP than coroners. Medical examiners are typically forensic pathologists whereas coroners are typically non-specialists and, in many jurisdictions, not medical professionals. This varied system, often run by people who are overworked and understaffed, makes methodical study of something like SUDEP very difficult. In order to develop a better understanding of the issues surrounding the medico-legal investigation of epilepsy deaths and develop strategies for surveillance of epilepsy-related mortality, the SUDEP Institute of the Epilepsy Foundation convened a SUDEP Surveillance Workshop on June 19, 2013, in New York City. This workshop had representation from key stake-holders including medical examiners, among them leadership of the National Association of Medical Examiners (NAME), coroners, epidemiologists, neurologists, representatives from the National Institute of Neurological Disease and Stroke (NINDS) and the Centers for Disease Control and Prevention (CDC), parents, and Epilepsy Foundation representatives. The major topics discussed were how to enhance SUDEP awareness among the death investigation community, how to improve the assessment and the reporting of SUDEP, and how to develop a framework to “map” existing definitions of SUDEP to the reporting requirements of medical examiners and coroners, who must complete death certificates to comply with medico-legal and public health needs.
The need for a unified approach to SUDEP reporting is a pressing matter. Through the efforts of patient advocacy groups, legislation has been passed or is under consideration in some states to mandate SUDEP surveillance. In New Jersey, which has a unified medical examiner system, a bill to require SUDEP education for medical examiners, standardize reporting, and facilitate tissue donation was recently signed into law and became effective January 1, 2014. In Illinois, which has a mixed coroner and medical examiner system, the Danny Stanton SUDEP law, named after a child who died from SUDEP, passed the state legislature in May 2013 thanks to the efforts of the Danny Did Foundation and local Foundation affiliates. The law, which advocates education about SUDEP, inquiry into the possibility that any sudden death was related to epilepsy, and standardized reporting of the deaths, also went into effect January 1, 2014. In other states, including New York, epilepsy advocates are working with the medical examiner community to investigate public health policy changes to improve SUDEP surveillance.
In order for this mandated surveillance to provide meaningful data, the death investigation community and epilepsy professionals must work together to develop guidelines for investigation, coding and reporting of deaths thought to be related to SUDEP. The collaborative groups facilitated by this SUDEP Institute initiative will continue to work together to improve the ability to study SUDEP and other causes of death in patients with epilepsy and to increase awareness of SUDEP among the diverse death investigation communities in the U.S.
Schraeder PL, Delin K, McClelland RL, So EL. Coroner and medical examiner documentation of sudden unexplained deaths in epilepsy. Epilepsy Res, 68(2), 137-143 (2006).