Epilepsy patients who experience three or more generalized tonic-clonic seizures (GTCS) per year have a significantly increased risk of sudden, unexpected death, according to new guidelines presented at the American Academy of Neurology (AAN) Annual Meeting on April 24.

The guidelines, co-developed by the AAN and the American Epilepsy Society, state that epilepsy patients who experience frequent GTCS—a type of seizure that involves a loss of consciousness and violent muscle contractions—are 15 times more likely to experience unexpected death in epilepsy (SUDEP) than those who experience few or no GTCS.

This risk is increased for adults, with one in 4,500 children and one in every 1,000 adults dying unexpectedly from epilepsy. This translates to up to 18 in 1,000 deaths per year for people with frequent GTCS. While this phenomenon is relatively rare, it is still important that healthcare professionals talk to their patients with epilepsy about the risk of SUDEP and the importance of managing frequent GTCS, said Cynthia L. Harden, M.D, lead author of the guideline and a professor of neurology at The Mount Sinai Hospital.

“The guideline shows that being free of seizures, particularly tonic-clonic seizures, is strongly associated with decreased risk,” said Harden during an AAN Annual Meeting press conference on the guidelines. “Educating health professionals and people with epilepsy about SUDEP is an important first step.”

Guideline recommendations

The guideline’s outline steps that health professionals and patients should take to reduce their risk of SUDEP.

For patients with epilepsy who continue to experience GTCS, the guidelines recommend  clinicians should continue to actively manage epilepsy therapies to reduce seizure occurrences and the risk of SUDEP, while incorporating patient preferences and weighing the risks and benefits of any new approach. Healthcare professionals need to stress the importance of adhering to medication and learning to manage seizure triggers with their patients, said Elizabeth Donner, M.D., co-author of the guidelines, associate professor at the University of Toronto and pediatric neurologist at the Hospital for Sick Children in Toronto.

“People need to understand that the risk of generalized tonic-clonic seizures is not only related to maintaining a driver’s license, maintaining work or other things like that, it’s actually related to risk of death,” said Donner at the AAN press event. “Further, I would hope that this can be a motivator to pursue treatments beyond medications when medications are not successful at treating seizures.

“We know that for a certain population of people living with epilepsy, medication, which is our first approach, does not work. In those cases, we need people to feel safe and motivated to work with their healthcare team to find other treatments, like surgery and other approaches to managing seizures”

The guidelines also state  unwitnessed seizures that occur at night may increase the risk of unexpected death from epilepsy. For patients with frequent GTCS and nocturnal seizures, some patients and families may want to consider having a secondary person present in the bedroom during sleep or using a remote listening device to reduce SUDEP risk.

Both Harden and Donner stated these guidelines are not meant to scare patients, but  that most patients or families of patients with epilepsy want to be informed of factors that are associated with an increased risk of a catastrophic event such as SUDEP. It is a doctor’s responsibility to make sure their patients and families are getting information from them about risks, and not from other sources, said Donner.

“I see parents that have seen their children have a particularly convulsive, body shaking-seizure and are terrified,” said Donner. “For most of these parents it has run through their head that their child could die from this. They have looked it up, they have Googled it, and it is almost the elephant in the room.

“So it is not difficult to talk about the risk of death associated with an illness when people are already thinking about it. In fact, if we as health care practitioners don’t share high quality information with people, they will find other information from other sources. When someone comes to see their health care practitioner, they really need to get information about their disorder in its entirety.”