July 25, 2019

SUDEP: Untangling the Causes

Article featuring the work of CURE Grantee Dr. William Nobis

Earlier this month, 20-year-old Disney star Cameron Boyce died suddenly from what was most likely sudden unexpected death in epilepsy, or SUDEP. In this rare complication from epilepsy, a patient who is otherwise healthy dies during or following a seizure, and postmortem examination reveals no structural or toxicological cause for death. Boyce’s family confirmed he had epilepsy.

SUDEP occurs in about 1-2 per 1,000 patients with chronic epilepsy and 3-9 per 1,000 in those with severe, refractory seizures, though its frequency is difficult to calculate. It is most common in patients 20 to 40 years old.

“SUDEP is difficult to detect; it is often unobserved when it happens and autopsy doesn’t reveal any changes,” said William Nobis, M.D., an instructor in the Department of Neurology at Vanderbilt University Medical Center. “No definitive cause has been discovered, but we believe that respiratory dysfunction leads to cardiac abnormalities and arrest.”

While the brain regions responsible for a seizure’s role in inducing respiratory depression are unclear, “we have some clues that the pathways involve the amygdala and the brainstem,” Nobis said. In a new study published in the Journal of Neurosurgery, Nobis and colleagues at Northwestern University suggest that activation of amygdalar networks is correlated with central apnea during seizures.

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