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Neurology Today:
doi: 10.1097/01.NT.0000346248.45962.39
Article

A Teenager's Death Turns Spotlight on Sudden Unexplained Death in Epilepsy

TALAN, JAMIE

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In January, John Travolta's teenage son, Jett, died after suffering an epileptic seizure, reminding neurologists of the importance of controlling seizures to reduce the risk of unexplained sudden death.

The complete autopsy results and the medical history of the 16-year-old have not been released to the public, but experts say that there is mounting evidence that the risk of dying is increased in epilepsy patients whose seizures are not controlled by available treatments.

While these deaths are rare, epilepsy specialists advise their colleagues to do everything possible to educate patients about the importance of taking anticonvulsant medicines, keeping a record of seizure activity, and sharing the information with their doctors.

There is no need to talk about this risk unless a patient is not compliant with treatment or continues to have uncontrolled seizures, experts said in interviews with Neurology Today.

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RISK FOR SUDEP

An estimated 0.05- to 1.0-percent of those with refractory epilepsy die of sudden unexplained death in epilepsy (SUDEP). About 30 to 40 percent of epilepsy patients continue to have seizures, even with the available treatments.

“People with well-controlled epilepsy have no greater risk of dying than anyone else,” said Michael R. Sperling, MD, the Baldwin Keyes Professor of Neurology and director of the Jefferson Comprehensive Epilepsy Center at Thomas Jefferson University Hospital in Philadelphia.

Long-term studies suggest that people with uncontrolled seizures die more often than expected. The causes vary somewhat but anywhere between 15 and 50 percent of the excess mortality among uncontrolled epilepsy patients is due to SUDEP. Other causes of death are attributed to head injury, car accidents, pneumonia, heart disease, or accidental drowning of patients in the throes of a seizure, said Dr. Sperling.

Figure. DR. MICHAEL ...
Figure. DR. MICHAEL ...
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Epilepsy patients may also be at higher risk for some cancers, and while no one knows why, Dr. Sperling and others speculate that the disease or treatment could alter immune function. What's more, many patients with epilepsy have only limited social and educational opportunities, which could lead to a lower socio-economic status and behaviors that put them at risk for other conditions.

The risk of death can also increase among patients with other life-threatening diseases that trigger seizures, such as brain tumors. Dr. Sperling sees about one to three unexplained deaths a year among his epilepsy patients.

Grand mal (tonic-clonic) seizures are associated with greater risk than other types of seizures. Also, neurological co-morbidities increase the risk for sudden death even more.

In a prospective study of 1,478 people in Sweden, people with epilepsy, mental retardation, and cerebral palsy had a 40-to 50-times higher risk of dying than those without epilepsy. The risk was about five times higher than normal in those with uncontrolled seizures, according to the 1996 study in Epilepsia.

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REASONS FOR INCREASED RISK

But why? There is evidence from animal studies and human autopsies that cardiac dysrhythmia during a seizure can trigger a sudden cardiac arrest. Additionally, abnormal electrical activity in the form of interictal spikes is generated between seizures, which could provoke alterations in cardiac rhythms. Autonomic functions are disturbed in patients with epilepsy, including variations in heart rate.

In fact, in one study of 43 patients, Maromi Nei, MD, associate professor of neurology at Thomas Jefferson University, and her colleagues found that about 40 percent had an arrhythmia. Thirteen percent of these cases were potentially lethal although none of the patients in that study died, according to the 2000 report in Epilepsia.

In another British study, reported in 2004 in The Lancet, 20 patients were followed for a year with continuous cardiac monitoring and doctors ended up putting pacemakers in four patients. Seizures might also trigger respiratory arrest in some patients, which could be another mechanism of SUDEP, Dr. Sperling said.

Epidemiological evidence suggests that more people die who live alone than with someone, according to a 2007 report in Epilepsia. Many SUDEP cases occur in the night while patients are asleep; often these people are found lying on their stomach rather than their back, suggesting that they may have suffocated during the seizure.

While the data are building to make a case for an increased risk of sudden death among epilepsy patients, the biggest stumbling block is that it is so rare.

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STRATEGIES FOR AVERTING THE CRISIS

Carl W. Bazil, MD, PhD, professor of clinical neurology at Columbia University College of Physicians and Surgeons, said that SUDEP should lead neurologists to consider epilepsy surgery when patients' seizures are not well-controlled with available drugs. Dr. Bazil added that epilepsy patients with status epilepticus can have a continuous seizure lasting 30 minutes and not regain consciousness. During such an event, the first line treatment should be intravenous use of benzodiazepine and, if needed, other intravenous anticonvulsants.

“Telling people with epilepsy that they may die unexpectedly is not beneficial,” said Dr. Sperling. Patients should be informed when knowledge of SUDEP can influence their treatment; for example, when discussing reliably taking medication or considering surgery. He added that surgical control of seizures reduces the risk of sudden death back to normal.

Dr. Bazil stressed that patients need to be clear about the importance of complete seizure control. Many patients do not report seizures because they are afraid of some state laws that mandate reporting to motor vehicle departments. This leads to an incentive to hide ongoing seizure activity, he said.

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ARTICLE IN BRIEF

Following the death of John Travolta's son Jett due to a seizure, epileptologists advise physicians to do everything possible to educate patients about the importance of taking anticonvulsant medicines, keeping a record of seizure activity, and sharing the information with their doctors.

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References

Forsgren L, Edvinsson SO, Blomquist HK, et al. Influence of epilepsy on mortality in mental retardation: An epidemiologic study. Epilepsia 1996;37(10):956–963.

Nei M, Ho R, Sperling MR. EKG abnormalities during partial seizures in refractory epilepsy. Epilepsia 2000;41:542–548.

Nei M, Ho RT, Sperling MR, et al. EEG and ECG in sudden unexplained death in epilepsy. Epilepsia 2004;45(4):338–345.

Rugg-Gunn FJ, Simister RJ, Duncan JS. Cardiac arrhythmias in focal epilepsy: a prospective long-term study. Lancet 2004;364:2212–2219.

Nashef L, Hindocha N, Makoff A. Risk factors in sudden death in epilepsy (SUDEP): The quest for mechanisms. Epilepsia 2007;48:859–871.

©2009 American Academy of Neurology

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