Sudden Death Still a Threat to People with Epilepsy

Anthony, a teenager, and Olivia, a mother of two young children, had a few things in common: They had epilepsy that was difficult to control, and they both died alone, probably as a result of a seizure.

Their stories, said Elizabeth J. Donner, MD, assistant professor of neurology, University of Toronto, are representative of the devastating cases of sudden, unexpected death in persons with epilepsy (SUDEP).

“Sudden death is 24 times more likely in someone with epilepsy than in the normal population,” Dr. Donner said in her plenary lecture “Sudden Unexplained Death in Epilepsy,” at the 61st Annual Meeting of the American Academy of Neurology.

She described the case history of Anthony who had an uncomplicated, unremarkable delivery but began developing staring spell-type seizures at age 3 years. By age 12 years, Anthony was experiencing multiple seizures, including generalized tonic clonic seizures as often as 8 times a day. He was diagnosed with Lennox-Gastaut Syndrome and proved medically intractable. Although also autistic and still suffering from cluster headaches, Anthony was attending school in a special needs 10th grade class. At age 16 years, his mother found him face down in his bed one morning. Resuscitation failed.

Olivia also had an uncomplicated pregnancy and was well until seizures were experienced when she was age 11 years. By age 19 years, her seizures were worsening, requiring multidrug treatment, but she was less than compliant, due to adverse effects of the medicine. Despite the seizures, which were poorly controlled, Olivia married, had two children, and held an office manager position. A room was set aside for her use if she felt poorly. When she was age 32 years, she went to the room, and when she didn’t return after two hours, co-workers went to the room and found her dead.

Dr. Donner said that while some people succumb to epilepsy because the seizure occurs while swimming or doing some other activity in which the seizure is responsible for the death, Anthony and Olivia appear to be cases of SUDEP.

“Epilepsy affects 1% of children,” Dr. Donner said, “but SUDEP accounts for 34% of sudden deaths in childhood.” And she said that SUDEP may be under-reported because coroners and medical examiners may not be aware of the condition. Autopsy rates in these cases are low, she noted.

She said her studies have estimated that SUDEP occurs in 0.2 per 1000-patient years in children.

Dr. Donner said risk factors of SUDEP include epilepsy severity, increased frequency of generalized tonic clonic seizures, longer duration of epilepsy, the use of polytherapy, and the lack of medication.

“Over the last few generations, we have emphasized that people with epilepsy can live nearly normal lives,” said Andrew Wilner, MD, an epileptologist in Newport, RI, “but we have to remember that people do die of this disease.”

Dr. Wilner emphasized that “those at most risk of fatality are people with intractable seizures.”