Should Adult Epilepsy Patients Be Told About Unexpected Death Risks?

by U.S. Medicine

May 31, 2017

Elevated Mortality Rates for Veterans Fires Up Debate

A shocking report on substantially higher mortality rates for veterans with epilepsy, as well as new guidelines from key disease groups, have reignited debate on a difficult issue in treating the neurological disorder: How much should epilepsy patients be told about their risks of sudden unexpected death, especially since—as likely with veterans—some of that risk might be unrelated to the condition?

By Brenda L. Mooney

COLUMBUS, OH—A study indicating that recent U.S. veterans with epilepsy were 2.6 times more likely to die over a four-year period than similar veterans without that diagnosis has added fuel to a long debate among clinicians treating the neurological condition.

The title of a session at the American Academy of Neurology meeting this spring said it all: “To Reveal or Conceal? Adult Patient Perspectives on SUDEP Disclosure.” Ohio State researchers took a close look at how much adult patients really want to know about sudden unexpected death in epilepsy patients (SUDEP).1

“The decision to discuss SUDEP with patients and families has created much debate,” wrote the abstract authors. “Many [healthcare practitioners] are hesitant to discuss SUDEP due to the perception of imposing unnecessary patient related fear, while others argue that informing patients about SUDEP may enhance patient compliance, improve seizure control and aide in saving lives.”

In a session at the same conference, newly-released guidelines co-developed by the American Academy of Neurology (AAN) and the American Epilepsy Society and endorsed by the International Child Neurology Association were very clear on their position: Epilepsy patients, especially those with tonic-clonic seizures—should be told of their risk for SUDUP, especially if the knowledge could help reduce that risk. (See sidebar, pg. xx).2

“The extent to which clinicians inform patients of their risk for SUDEP varies greatly,” Mary Jo Pugh, PhD, RN, lead author of the veterans’ study and associate chief of staff for research, South Texas VA Health Care System in San Antonio, told U.S. Medicine. “There is some controversy in the minds of some about the value of this education. My interviews of clinicians in and outside of VA over the past week suggest that some don’t because there is nothing that can really be done about it. Others have had their opinions changed over the past five years or so. They now include a discussion of SUDEP in the first or second visit in part to encourage medication compliance.”

Interestingly, the research earlier this year in the Morbidity and Mortality Weekly Report on veteran death rates emphasized that the role of SUDEP wasn’t clear in that situation and would require further study.3

“Similar to studies of civilian samples, we found that cancer, stroke and cardiac disease were strong predictors of five-year mortality. But, even after controlling for the impact of these comorbid conditions, we still found a substantial effect for epilepsy,” Pugh noted when that study was published.

She added that, because federal databases at the time the study was conducted did not include causes of death, the report does not speculate on whether mortality was linked to suicidality, car accidents, heart attack, cancer or SUDEP.

Despite that, SUDEP remains a serious concern and the topic of much research and discussion. Another study published this year in the journal Epilepsia noted, “All population-based studies reported an increased risk of premature mortality among people with epilepsy compared to general populations.”4

That Emory University-led study added, “Standard mortality ratios are especially high among people with epilepsy aged <50 years, among those whose epilepsy is categorized as structural/metabolic, those whose seizures do not fully remit under treatment, and those with convulsive seizures. Among deaths directly attributable to epilepsy or seizures, important immediate causes include sudden unexpected death in epilepsy (SUDEP), status epilepticus, unintentional injuries, and suicide.”

In the survey conducted by Ohio State University, meanwhile, all of the respondents said that adult epilepsy patients have the right to know about their higher death risk. For that study, 42 patients with epilepsy (PWE) completed a questionnaire based on an information sheet promoted by the National Epilepsy Foundation of America.

Results indicate that 100% responded that adult PWEs had a right to know about SUDEP and 92% felt that HCPs should be required to inform patients.

Despite concerns that the knowledge would frighten them unnecessarily, respondents said instead that SUDEP awareness motivated them for better medication adherence (81%) and management of their seizure triggers (85%). Fear appeared to be more prevalent in the group with generalized tonic clonic seizures, study authors noted.

Some physicians are more likely than others to inform patients, Pugh said, adding, “My VA colleagues unanimously say that patients should be educated on SUDEP and that they personally do that. The issue is that they are epilepsy specialists. Many veterans with epilepsy do not see epilepsy specialists due to the small number of those specialists in (and outside) the VA.”

Why Higher Death Rates?

Research published late last year in The Lancet Neurology provided more information about SUDEP and offered a possible explanation as to why death rates were so much higher in young veterans with epilepsy.5

“Sudden unexpected death in epilepsy (SUDEP) can affect individuals of any age, but is most common in younger adults (aged 20-45 years),” wrote study authors, including researchers from the Iowa City, IA, VAMC. “Generalized tonic-clonic seizures are the greatest risk factor for SUDEP; most often, SUDEP occurs after this type of seizure in bed during sleep hours and the person is found in a prone position.”

Those researchers went on to explain that SUDEP excludes other forms of seizure-related sudden death that might be mechanistically related, such as death after single febrile, unprovoked seizures or status epilepticus. Usually, they pointed out, postictal apnea and bradycardia progress to asystole and death.

Noting that brainstem dysfunction is “a crucial element” in SUDEP, the study authors added. “Dysfunction in serotonin and adenosine signaling systems, as well as genetic disorders affecting cardiac conduction and neuronal excitability, might also contribute. Because generalized tonic-clonic seizures precede most cases of SUDEP, patients must be better educated about prevention. The value of nocturnal monitoring to detect seizures and postictal stimulation is unproven but warrants further study.”

The Epilepsia authors agreed, stating, “Epilepsy-associated premature mortality imposes a significant public health burden, and many of the specific causes of death are potentially preventable. These require increased attention from healthcare providers, researchers, and public health professionals.”

Yet, those comments raised another essential issue: What can be done immediately to reduce or prevent SUDEP?

The answer, according to a study late last year from Stavanger University Hospital in Norway, might very well lie with existing anti-epileptic drugs.

Writing in the journal Seizure, those authors explained, “High frequency of generalized tonic-clonic seizures is the most important risk factor, and effective seizure protection is probably the most important measure to prevent these tragic deaths.”6

In line with that, the recent treatment guidelines strongly advised clinicians to inform patients of their SUDEP risks.

“Educating health professionals and people with epilepsy about SUDEP is an important first step,” emphasized lead author Cynthia Harden, MD, of Mount Sinai Health System. “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.”

The guideline recommended that, with patients who continue to have worrisome seizures, health professionals try to reduce them with medications or epilepsy surgery, whichever seems most appropriate.

“There is consensus that better seizure control is important for SUDEP risk, but there is really no data available to determine the extent to which SUDEP could be mitigated by better seizure control,” Pugh suggests.

The Norwegian authors took it a step further, however. Their review rejected conventional belief that choice of AED therapy has little or no effect on SUDEP risk, pointing out “although it is well known that the efficacy and safety profiles of AEDs may differ significantly when used in the treatment of genetic epilepsy compared to symptomatic or cryptogenic epilepsy, this has generally been overlooked in epidemiologic studies of possible relationships between AEDs and SUDEP. Consequently, important information about drug safety may have been lost. This review challenges the current view that no AED can increase the risk of SUDEP.”

  1. Long L, Hart S, Mindel J. To Reveal or Conceal? Adult Patient Perspectives on SUDEP Disclosure. Adult Patient Perspectives on SUDEP Disclosure, presented at the American Society. American Academy of Neurology Annual Meeting in Boston. April 22-28, 2017.
  2. Harden C, Tomson T, Gloss D, Buchhalter J, et. al. Practice guideline summary: Sudden unexpected death in epilepsy incidence rates and risk factors: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology. 2017 Apr 25;88(17):1674-1680. doi: 10.1212/WNL.0000000000003685. PubMed PMID: 28438841.
  1. Pugh MJ, Van Cott AC, Amuan M, et al. Epilepsy Among Iraq and Afghanistan War Veterans United States, 2002-2015. MMWR Morb Mortal Wkly Rep 2016;65:1224–1227. DOI: http://dx.doi.org/10.15585/mmwr.mm6544a5.
  2. Thurman DJ, Logroscino G, Beghi E, Hauser WA, et. al. Epidemiology Commission of the International League Against Epilepsy. The burden of premature mortality of epilepsy in high-income countries: A systematic review from the Mortality Task Force of the International League Against Epilepsy. 2017 Jan;58(1):17-26. doi: 10.1111/epi.13604. Epub 2016 Nov 26. Review. PubMed PMID: 27888514.
  3. Devinsky O, Hesdorffer DC, Thurman DJ, Lhatoo S, Richerson G. Sudden unexpected death in epilepsy: epidemiology, mechanisms, and prevention. Lancet Neurol. 2016 Sep;15(10):1075-88. doi: 10.1016/S1474-4422(16)30158-2. Epub 2016 Aug 8. Review. PubMed PMID: 27571159.
  1. Aurlien D, Gjerstad L, Taubøll E. The role of antiepileptic drugs in sudden unexpected death in epilepsy. Seizure. 2016 Dec;43:56-60. doi:10.1016/j.seizure.2016.11.005. Epub 2016 Nov 14. Review. PubMed PMID: 27886630.

Related Articles

Despite Criticism of Federal Medicine, Innovation Thrives at VA, MHS

By Brenda L. Mooney Editorial Director ATLANTA — When it comes to American politics, we certainly live in contentious – if not always interesting – times. One of those hot-button issues is a so-called single-payer... View Article

New Tools Help Reduce CVD Risk in Veterans with Diabetes

By Annette M. Boyle The search has been on for decades: A medication that can help reduce the risk of cardiovascular disease in Type 2 diabetes patients. Late last year, an existing drug was approved... View Article


U.S. Medicine Recommends


More From 2017 compendium of federal medicine

2017 Compendium of Federal Medicine

Despite Criticism of Federal Medicine, Innovation Thrives at VA, MHS

WASHINGTON — The process for tracking the DoD’s most serious adverse medical events is “fragmented, impeding the Defense Health Agency’s (DHA) ability to ensure that it has received complete information,” according to a new review.... View Article

2017 Compendium of Federal Medicine

New Tools Help Reduce CVD Risk in Veterans with Diabetes

WASHINGTON — The process for tracking the DoD’s most serious adverse medical events is “fragmented, impeding the Defense Health Agency’s (DHA) ability to ensure that it has received complete information,” according to a new review.... View Article

2017 Compendium of Federal Medicine

How the Brain, Not Just Lung Function, Affects Patient-Perceived COPD Level

WASHINGTON — The process for tracking the DoD’s most serious adverse medical events is “fragmented, impeding the Defense Health Agency’s (DHA) ability to ensure that it has received complete information,” according to a new review.... View Article

2017 Compendium of Federal Medicine

Protein Can Help Identify Which Rashes Are Early Mycosis Fungoides

WASHINGTON — The process for tracking the DoD’s most serious adverse medical events is “fragmented, impeding the Defense Health Agency’s (DHA) ability to ensure that it has received complete information,” according to a new review.... View Article

2017 Compendium of Federal Medicine

Good Treatment Options Emerging for Less-Common Primary Progressive MS

WASHINGTON — The process for tracking the DoD’s most serious adverse medical events is “fragmented, impeding the Defense Health Agency’s (DHA) ability to ensure that it has received complete information,” according to a new review.... View Article

Facebook Comment

Subscribe to U.S. Medicine Print Magazine

U.S. Medicine is mailed free each month to physicians, pharmacists, nurse practitioners, physician assistants and administrators working for Veterans Affairs, Department of Defense and U.S. Public Health Service.

Subscribe Now

Receive Our Email Newsletter

Stay informed about federal medical news, clinical updates and reports on government topics for the federal healthcare professional.

Sign Up