AAN Releases Guidelines for Sudden Unexpected Death in Epilepsy

Speaking at a press conference at the American Academy of Neurology (AAN), Cynthia L. Harden, MD, clinical epilepsy services at Mount Sinai Health System, and Elizabeth Donner, MD (pictured) of the University of Toronto, announced a new set of guidelines from the American Academy of Neurology regarding sudden unexpected death in epilepsy, or SUDEP.

“There is an uncommon risk of death that people with epilepsy, and their loved ones, may not know about,” Harden opened in her introduction of the condition. “SUDEP is when someone with epilepsy but otherwise healthy dies suddenly with no known cause.”

The guideline is a collaboration between the AAN and the American Epilepsy Society, and is endorsed by the International Child Neurology Association. Harden says it is intended to “bring clarity to the discussion, giving healthcare providers practical information they can use.”

Donner followed with a detailing of the prevalence of SUDEP. Following extensive reviews, they found that the condition was rare in children, only impacting 1 in 4500 children in epilepsy per year, though the number is higher at 1 in 1000 adults. The most major risk factor that the team identified was generalized tonic-clonic seizure, “the kind of seizure that most people think about when they think about seizures,” she said.

Those who suffer 3 or more per year are 15 times more likely to die suddenly than those who did not have such seizures, according to the team’s research. “In total, this translates to up to 18 in 1000 deaths per year in people with epilepsy who have generalized tonic-clonic seizures.”

The guidelines recommend encouraging physicians to make those with epilepsy aware of the risk, particularly those prone to such seizures, and to encourage preventative measures.

“The guideline shows that being free of seizures, particularly tonic-clonic seizures, is strongly associated with decreased risk,” Harden followed, emphasizing that the guideline was meant to educate both health providers and patients as well as reinforce the importance treatment adherence can have in reducing the risk of SUDEP.

“The team did look at other potential risk factors for SUDEP,” Donner said, “but the evidence was not strong enough to make recommendations regarding these risk factors.”

“Really, more research is needed regarding risk factors for SUDEP so we can focus future studies on finding ways to reduce how often SUDEP occurs,” she added,

While the awareness and emphasis on reducing overall seizures to reduce risk seem like common sense, Donner reinforced the importance of putting together these guidelines. “I would hope that this can be a motivator to pursue treatments beyond medication when medication isn’t successful at treating seizures...we need people to feel safe and motivated to work with their health care team to find other treatments, like surgery and other approaches to managing seizures.”

“Using the AAN guideline methodology we were able to provide nuance to those risk factors,” Harden said, adding that the importance was being able to separate out risk factors based on the strength of evidence supporting them. Delivering the information to patients is important, says Harden, who “couches” SUDEP as “the most severe risk of epilepsy.”

Donner, who is a pediatric neurologist, emphasized the difficulty of discussing epilepsy and death, and the importance not only of addressing the topic in practice but also creating the guidelines. “Parents who have witnessed their child have a particularly convulsive, full-body-shaking seizure are terrified…it’s almost the elephant in the room.”

“If we, as health care practitioners, 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,” she closed.

The research was published today in the AAN’s journal Neurology.