Sudden Unexpected Death in Epilepsy (SUDEP) in Sturge-Weber Syndrome

OCTOBER 3, 2014

What is SUDEP (Sudden Unexpected Death in Epilepsy)? What causes it? Does this happen to people with Sturge-Weber syndrome?

Response: SUDEP is the unexpected death of a person with epilepsy who is otherwise healthy, and an autopsy does not demonstrate another reason. It usually happens at night and the cause of it is not well understood. There are theories which may partially account for it. Often the patient is found the next morning face down and therefore suffocation is thought to have some role. Other factors may include heart rhythm problems. Some individuals show evidence of having had a recent seizure.

Each year, one in 1,000 patients with epilepsy die of SUDEP, and about one in 150 patients with uncontrolled epilepsy die of SUDEP. It is rare in children, and most common in young adults. SUDEP has not been shown to occur in those with only staring or myoclonic seizures, and those with uncontrolled generalize tonic-clonic seizures seem to be at highest risk.

I have known one patient with Sturge-Weber syndrome who died SUDEP. He was a young boy with uncontrolled seizures who was getting ready to be evaluated for hemispherectomy when he died. This is out of about 200+ patients I have cared for with Sturge-Weber syndrome brain involvement over the years.

Research has shown that the most important predictor of SUDEP is uncontrolled seizures. Therefore, it is important to always take medication as directed and to work closely with your physician to attain as close to complete seizure control as possible.  Try to identify your/ your child’s seizure triggers and avoid them. Research has shown that having someone else in the room at night to help, should a seizure occur, can reduce the risk of SUDEP. Therefore, those with uncontrolled seizures may want to consider this option.

SUDEP has recently become a focus of research, increased recognition, and understanding. Patients are urged to discuss this topic with their doctors, particularly if their seizures are uncontrolled. More information can be found on the Epilepsy Foundation website. In addition to the research databases listed at that website, if it is important to you that our center maintains a research database on SUDEP in Sturge-Weber syndrome, please let me know.

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