The major risk factor for sudden unexplained death in epilepsy (SUDEP) was found to be generalized tonic-clonic seizures. This risk is 15 times higher in people who have three or more of this type of seizure per year. This translates to up to 18 in 1000 deaths per year in this high-risk population.
The new SUDEP guidelines, co-developed by the American Academy of Neurology and American Epilepsy Society and presented this week at the AAN Annual Meeting, is endorsed by the International Child Neurology Association.
“Using the AAN guideline methodology, we were able to provide nuance to those risk factors – which ones are well established, which ones have strong evidence. . .Even though this information is available, neurologists in general don’t take the time or have the ability to synthesize which ones are truly important for their patients in order to reduce the risk,” said guideline author Cynthia Harden, MD, Mount Sinai Health System, New York, NY. She explained that while some risk factors are not modifiable, the guidelines provide an actionable plan for health care professionals to help their patients reduce risk of SUDEP.
After reviewing all available evidence on SUDEP, researchers found moderate evidence that SUDEP is rare in children, affecting one in 4500 children every year. In addition, SUDEP is uncommon in adults, affecting one in 1000 adults each year.
“It is important that the rate of occurrence of SUDEP and the specific risk factors for SUDEP are communicated to persons and families affected by epilepsy,” said Harden. “Our guideline brings clarity to the discussion, giving health care providers practical information they can use to help people with epilepsy reduce their risk.”
Therefore, health professionals should tell people with epilepsy that controlling seizures, especially tonic-clonic seizures, may reduce the risk of SUDEP. Being free of seizures, particularly tonic-clonic seizures, is strongly associated with a decreased risk of SUDEP.
Health care providers should advise patients who continue to have tonic-clonic seizures to try to reduce them with medications or epilepsy surgery, being sure to actively weigh the risks and benefits of any new approach.
“Educating health professionals and people with epilepsy about SUDEP is an important first step,” said Harden. “This guideline makes the conversation much easier with information that may motivate people to take their medications on time, to never skip taking their medications, and to learn and manage their seizure triggers so they can work toward reducing seizures. People who follow their medication schedule or pursue other treatments such as surgery may be more likely to become seizure free.”
Guideline author Elizabeth Donner, MD, The Hospital for Sick Children, Toronto, Canada added, “I would hope that this could be a motivator [for patients] to pursue treatments beyond medication when medication is not successful in treating seizures. . .in those cases we need people to feel safe and motivated to work with their health care team to find other treatments” to manage their seizures.
When asked how to approach a discussion of SUDEP with patients, both Harden and Donner advise a direct approach. “I think how we share information about disease risk is part of a comprehensive education around epilepsy,” said Donner. She explained it’s not difficult to bring up risk of death associated with epilepsy because patients are already thinking about it. “If we as health care providers don’t share high quality information with people, they will find other information through other sources. We’re their most trusted source of information and we should be the ones providing it.”
Although the guideline authors examined other potential risk factors for SUDEP, the evidence was not strong enough to support recommendations regarding these risk factors in the medical management of persons with epilepsy.
“More research is now needed to identify other preventable risk factors so that future studies can focus on finding ways to reduce just how often SUDEP occurs,” Harden said. Developing a powerful animal model to test interventions will be important, as will increasing awareness of SUDEP, particularly getting the term SUDEP into the lexicon of medical examiners, to help verify the incidence of SUDEP.
The guidelines were published online in Neurology.
From materials presented at the AAN Annual Meeting, Boston, MA. Press conference. April 24, 2017.