Sudden Unexpected Death In Epilepsy

Nitin K Sethi, MD

Assistant Professor of Neurology

New York-Presbyterian Hospital

Weill Cornell Medical Center

New York, NY 10021

I recently read about the tragic death of John Travolta’s son from a reported seizure while on a family vacation. The news got extensive coverage on prime time television networks but unfortunately none of the news segments provided any credible information about death from a seizure or epilepsy in general.  While I respect medical journalists and the work they do, I feel they owe a bigger moral obligation not just in getting news across to the public but also going a step forward in researching the topic and utilizing the news story  to spread awareness about a disease. The tragic death of Mr. Travolta’s son limelights epilepsy and its at times unexpected tragic consequences.

When people think of a seizure or epilepsy, death as a possible consequence does not come to mind as conjured by other illnesses like cancer. Seizures usually are discreet episodes, guaranteed they are frightening to witness (if you happen to be a family member or a bystander). The patient falls down (if standing at the time of seizure onset), shakes and jerks violently, eyes roll up, drools and may bite his or her tongue. After a minute or two (which for the caregiver or bystander may seem like an eternity), the person stops shaking and may infact appear to fall asleep and breathe loudly. Emergency medical staff are there by then and take the person to the hospital.

ALL’S WELL THAT ENDS WELL YOU MAY SAY!!!

Well yes and no. As I tell most of my patients and their concerned family members, most seizures end on their own and do not need any “active intervention” (meaning giving them some drug to stop the seizure). By the time EMS arrive, the seizure is already over and the patient is confused and disoriented (we call this the post-ictal stage meaning the stage after the ictus/seizure is over). There are a few patients in whom the seizure may not stop or in whom one seizure is followed in rapid succession by another seizure without regaining consciousness in between. These patients are said to be in “status epilepticus” and need urgent medical attention to abort the onoing seizures. These are the patients who the EMS give intravenous medications to stop the seizure (you can read about this more on my website http://braindiseases.info), once these patients reach the ER, intravenous medications to abort the seizure are administered.

But coming back to where I started most seizures do abort on their own. That is what I tell my patients and their caregivers. As a physician the seizure itself does not worry me so much, it is the circumstances surrounding the seizure which can prove to be fatal. When a person is having a seizure, his or her brain is malfunctioning (think of it as a massive short circuit in the neural pathways), he is thus unable to fend for himself.  Seizures are usually associated with a loss of body tone, a standing patient thus may fall and injure himself. A hard fall on the head may result in a fatal head injury due to intracranial hemorrhage. A person standing next to a subway line in New York City may fall onto the tracks after a seizure episode and get hit by an oncoming train, he or she may suffer a fatal car crash if the seizure happens to occur while they are driving (this of course poses a risk to other motorists and pedestrians who share the road. There are rules with respect to driving with epilepsy and these vary from state to state). Deaths have occurred due to submersion and drowning if the patient has a seizure while swimming or while taking a bath in a tub.

So what I tell my patients is this

“YOU DO NOT WANT TO GET CAUGHT WITH A SEIZURE AT THE WRONG PLACE AND AT THE WRONG TIME”.

There is a further entity called “Sudden Unexplained Death in Epilepsy” also called SUDEP. SUDEP refers to patients with epilepsy/ seizure disorder who are found dead due to no “apparent reason”.  These usually are epilepsy patients who on the surface seem to be fine and one day are found dead. At times they may go to bed okay but are found in bed dead the next morning. A lot of research and studies have looked into SUDEP to try to determine its etiology and thus help in better identification of those epilepsy patients who are more prone to SUDEP. While we still do not know what causes SUDEP, we now do know certain facts.

There is neural control of heart rate (meaning parts in the brain control our heart rate). Hence seizures which arise from certain areas in the brain such as the insular cortex may at times be accompanied with bradycardia (that is the heart slows down and in extreme cases may even stop for a few seconds to minutes). Thus ictal bradycardia or ictal asystole may be one of the mechanism underlying SUDEP.  In other documented cases of SUDEP, patient was found to have severe laryngeal muscle spasm  thus impairing respiratory effort.

So who are the patients who are at high risk for SUDEP? These are usually patients who have poorly controlled epilepsy (seizure control is inadequate on current therapy). Patients who have seizures associated with ictal bradycardia and/or laryngeal spasm remain at exceptionally high risk.

Epilepsy is a relatively common neurological disorder. It imposes a high price in terms of socio-economic costs and quality of life issues. Every attempt should be made to attain good seizure control (thankfully we now have many options both medical therapy ( anti-seizure medications) as well as surgical, you can read about them on my website http://braindiseases.info).

The battle is still to be won but together we can!!!