George Washington's step-daughter died of it. So likely did Florence Griffith Joyner, and the sister of 'Seinfeld' actor John O'Hurley.

'It' is sudden unexplained death in epilepsy (SUDEP). SUDEP does not side-step the famous -- it kills people of every age, socioeconomic group and gender. SUDEP is defined as sudden, unexpected, non-traumatic, non-drowning death in an individual with epilepsy. Witnessed or not, in which a postmortem examination does not reveal an anatomical or toxicological cause for the death. Usually the victims' breathing or hearts stop during or just after a generalized tonic-clonic seizure, frequently when they're in bed at night.

The incidence of SUDEP is approximately one per 500-1,000 person-years in people with epilepsy, rising to one per 200 person-years among those with severe cases (Seizure 2003;12:456-464). It is believed to be responsible for 2-18% of all epilepsy related deaths (Seizure 1999;8:347-352), and males aged 15-40 are at highest risk.

However, even these dire statistics may not reflect the true rate of SUDEP -- a survey of 2,997 medical examiners and coroners across the United States revealed only 31% declared SUDEP to be the cause of death in >50% of the cases in which an autopsy revealed no other possible causes (Epilepsia 2003;44[Suppl.9]:181 [Abstract 2.015]).

"It is an intellectual disconnect -- they steer around SUDEP, even though they know it exists," says primary investigator Paul Schraeder, MD, professor of neurology, Drexel University College of Medicine, Philadelphia. "So at least in the U.S., there's probably a significant tendency to under-report the phenomenon."

To Tell Or Not To Tell?

Neurogenic cardiac arrhythmias and pulmonary edema are key suspects in SUDEP, but neither has ever been 'caught in the act' of causing a death. Hence, virtually nothing is known about what can be done to prevent SUDEP. Each paper on the topic includes a different menu of risk factors. Some experts point to early onset of seizures, refractory seizures, poor drug compliance, polytherapy, or learning disabilities, while others systematically rule each of these out (see for example Seizure 2003;12:456-464). The general consensus is that optimization of treatment and hence minimization of seizures is the best course of action.

The vast majority of neurologists and epileptologists in North America do not discuss the possibility of SUDEP with patients who have been newly diagnosed with epilepsy, nor with their families.

"The question of whether you bring this up with families that don't ask is very controversial," says Elizabeth Donner, MD, staff neurologist, Hospital for Sick Children, Toronto, who has published the largest series to date on pediatric SUDEP (Neurology 2001;57:4309-434). "Most families who have lost a child say, 'You should have told us [that this could happen].' But nothing I can see in the literature [establishes what] can be done to prevent SUDEP. So I don't know anyone who brings it up with newly diagnosed patients." Only if Dr. Donner is asked by parents whether epilepsy is life-threatening does she tell them about SUDEP.

Gregory L. Holmes, MD, raises the issue with every set of parents of children with epilepsy. Dr. Holmes, chief of neurology, Dartmouth Medical School, Lebanon, N.H., and first vice-president of the American Epilepsy Society, tells them SUDEP is rare, and that they should not become overly concerned and hyper-vigilant with their children.

"I bring it up as part of the discussion with the parents of the risks associated with epilepsy. I tell them it's more likely that they'll get hit by a car driving home from the appointment [with me]," Dr. Holmes tells CNS News. "I think people with epilepsy have enough problems as it is -- it's a disorder that still carries a scarlet 'E'. So to add to that social burden by bringing up SUDEP -- it's a judgement call."

Lawrence J. Hirsch, MD, associate clinical professor of neurology, Comprehensive Epilepsy Center, Department of Neurology, Columbia University, New York, takes a position between those of Drs. Donner and Holmes.

"That's the last thing a general neurologist needs -- a long, serious discussion during his 15-minute follow-up with a recently diagnosed patient with epilepsy about something rare that they probably cannot prevent," says Dr. Hirsch. "For patients with refractory epilepsy or those who are non-compliant with medication, SUDEP should be discussed. For everyone else, I'd recommend providing comprehensive information via brochures or Web sites that include discussions of SUDEP."

Jeanne Donalty, board member of Chicago-based Citizens United for Research on Epilepsy (CURE), is incensed that epilepsy patients are rarely told about the possibility of SUDEP. Mrs. Donalty's son Christopher died of SUDEP in February 2002, at the age of 21. Christopher was in his senior year at Stetson University in DeLand, Fla., when he died at his girlfriend's apartment. She arrived home after school one evening to find his body. Neither Mrs. Donalty nor Christopher had ever been told about SUDEP.

"I can't bring my son back, it's too late. But from a parent's point of view, I think that if Chris had known about this, he would have made different decisions. He wouldn't have made a decision to go to a place where he'd be alone if he had a seizure and could die," Mrs. Donalty says. "For a person to not have all the information they need to make their decisions -- it's unconscionable. It's like this dirty little secret in epilepsy. And unless you talk about it, nothing's going to be done to reduce the rate of SUDEP."

However, medical ethicist Bernard Dickens, PhD, LLD, when contacted for an opinion about whether physicians should tell people with epilepsy about SUDEP, said it is not advisable to routinely tell all newly diagnosed epilepsy patients about the phenomenon.

"It could be that targeted, patient-specific information is not helpful," commented Dr. Dickens, professor emeritus of health law and policy, University of Toronto. "Communication to the general public might be more helpful, and then if they wish, particular individuals may seek more information from their physicians."

U.K. Epilepsy Guidelines Urge Disclosure of SUDEP Facts

The authors of a new set of English epilepsy guidelines appear not to agree with the non-disclosure position. The guidelines were released in October 2004 by the National Institute for Clinical Excellence (NICE), (see here). The guidelines follow on the heels of a U.K. SUDEP audit in 2002, which uncovered many serious deficits in the treatment of epilepsy and in the awareness of SUDEP among clinicians (see here and commentary in Lancet 2002;359[9320]:1790-1791).

Jane Hanna, director of Epilepsy Bereaved -- a U.K.-based organization that has persistently fought since 1990 to increase awareness of SUDEP among the public and physicians -- headed the committee that wrote the audit report, and was also on the committee that created the new epilepsy guidelines.

"Given the controversy over the years about recognition of SUDEP, to now have specific recognition of SUDEP, and to have a list of the clinical issues that have to be addressed, is absolutely critical," said Ms. Hanna, whose partner Alan died of SUDEP at age 27.

Some of the main points relating to SUDEP in the NICE guidelines are:
- Healthcare professionals should provide newly diagnosed patients with information about all aspects of epilepsy, ranging from general facts about epilepsy to discussion of the small but definite risk of SUDEP. Literature on SUDEP should be provided, as should tailored information on each individual patient's risk of SUDEP.
- Physicians should be aware of the higher risks of mortality among people with learning disabilities and epilepsy, and discuss these risks with the families and/or care givers.
- Patients with both epilepsy and learning disabilities should be assessed for the risks posed to them by such factors as bathing, cooking, using electrical equipment, and SUDEP.
- The risk of SUDEP can be minimised by optimising seizure control and being aware of the potential consequences of nocturnal seizures.

Dr. Holmes commented that this last point is "possible, but not proven. Patients can have SUDEP without a history of nocturnal seizures, and it also should not be assumed that good seizure control eliminates the risk of SUDEP."

However, Henry Smithson, MBChB, MSc, the chair of the new epilepsy-guidelines committee and spokesperson on epilepsy for the Royal College of General Practitioners, sticks with his team's recommendations.

"The evidence suggests -- not in children, in whom SUDEP is rare, but in adults, particularly young adults -- that almost all cases of SUDEP occur during a seizure," Dr. Smithson tells CNS News. "So perhaps by giving those individuals and their families and friends some information about SUDEP, people with epilepsy can make better-informed decisions about their epilepsy and their lives -- they may be more likely to take their medicine as indicated, get enough sleep, not drink to excess, etcetera."

All of this activity in the U.K. may have a ripple effect in the States and Canada. The AES's Dr. Holmes tells CNS News that release of the NICE guidelines may spur the AES, American Academy of Neurology and American Academy of Pediatrics to develop new guidelines on epilepsy, incorporating information on SUDEP.

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Last Modified: 06/30/2006 10:53:56 AM