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Expert Interview Epilepsy
Introducing New Guidelines on Sudden
Unexpected Death in Epilepsy
An Expert Interview with Cynthia L Harden
Department of Neurology, Mount Sinai Health System, New York, NY, US
Cynthia L Harden
Cynthia L Harden, MD, received her medical degree at the University of Wisconsin. She trained in internal medicine at Mount Sinai
St Luke’s Hospital and neurology at Mount Sinai Hospital, both in New York City, and in clinical neurophysiology at Albert Einstein College
of Medicine in the Bronx. She served most of her career at Weill Cornell College of Medicine, where she became Professor of Neurology.
Dr Harden serves as Chair of the Guideline Development, Dissemination and Implementation Subcommittee of the American Academy
of Neurology (AAN). In 2016, she was also elected Chair of AAN’s Epilepsy Section for a 2-year term.
Keywords Epilepsy, guidelines, sudden unexpected death,
SUDEP Disclosure: Cynthia L Harden has nothing to declare in
relation to this article. This is an expert interview and as
such, has not undergone the journal's standard peer
review process. No funding was received in the publication
of this article.
Authorship: All named authors meet the International
Committee of Medical Journal Editors (ICMJE) criteria
for authorship of this manuscript, take responsibility
for the integrity of the work as a whole, and have
given final approval for the version to be published.
Open Access: This article is published under the Creative
Commons Attribution Noncommercial License, which
permits any noncommercial use, distribution, adaptation,
and reproduction provided the original author(s) and
source are given appropriate credit.
Received: June 12, 2017
Published Online: July 24, 2017
Citation: US Neurology, 2017;13(2):65–6
Corresponding Author: Cynthia L Harden, Mount Sinai
Health System, Mount Sinai Beth Israel Phillips Ambulatory
Care Center, 10 Union Square East, Suite 5D, New York, NY
10003, US. E: Cynthia.Harden@mountsinai.org
TOU CH MED ICA L MEDIA
N ew guidelines from the American Academy of Neurology (AAN) and the American Epilepsy
Society on sudden unexpected death in epilepsy (SUDEP) were presented at the 69th Annual
AAN Meeting held in Boston, MA, US, May 28–22, 2017 in parallel with their publication in
Neurology. 1 In an expert interview, Dr Cynthia L Harden of Mount Sinai discusses SUDEP, why the new
guidelines were needed and the key recommendations.
Q. What is the incidence of SUDEP?
Based on moderate confidence in the evidence from three Class I studies, SUDEP risk in children with
epilepsy is 0.22/1,000 patient-years (95% confidence interval [CI] 0.16–0.31). 1 Based on low confidence
in the evidence, due to considerable imprecision across five Class I studies, SUDEP risk increases in
adults to 1.2/1,000 patient-years (95% CI 0.64–2.32). 1
These rates were derived according to the AAN guideline process, as follows: in the comprehensive
analysis of the literature, the authors included reports that met AAN criteria to be categorized as
Class I, which means they are less likely to have selection bias than reports determined to be Class
II-IV. 2 The Class I articles surveyed a population-representative group of people with epilepsy, and
captured at least 80% of the deaths in the study population, and of the deaths, at least 80% were
systematically evaluated for death due to SUDEP. A random-effects meta-analysis was performed
across age groups, due to variation in SUDEP incidence rates across studies. The three papers in
children had close agreement as to the SUDEP incidence per 1,000 epilepsy patient-years, therefore
the confidence in the evidence is “moderate”, meaning that future studies are unlikely to change this
incidence rate. The five papers in adults with epilepsy had more variation in their findings, resulting
in the confidence in the evidence as “low”, meaning that future studies may change this SUDEP
incidence rate. These rates are based on an accurate documentation of the SUDEP diagnosis, so that
the SUDEP occurrences were either “definite”, meaning an autopsy was performed, or “probable”,
meaning that the SUDEP criteria were met, but no autopsy was performed. Overall, the incidence
appears to increase after age 18. Patients over age 55 were often excluded in the reports since
competing causes of death e.g., cardiovascular-related, could not be reliably excluded in this mature
age group. These results suggest that ongoing research to accurately capture SUDEP diagnoses at the
point of contact with the medical examiner, is still needed.