Abstracts

Rationale: Sudden unexpected death in epilepsy (SUDEP) is an important cause of mortality in patients with epilepsy. Potential mechanisms include cardiac causes such as ictal arrhythmia, bradycardia, and asystole; ictal apnea leading to hypoxemia or hypercarbia; and neurogenic cardiopulmonary dysfunction. Ictal asystole is well-described in patients with epilepsy. It is often treated with a permanent cardiac pacemaker with the thought of lessening risk for SUDEP, but the degree of benefit from this intervention is unclear. Methods: Case report. Results: A 41-year-old man with epilepsy since age 1 secondary to meningitis presented for evaluation of increasing seizure frequency despite treatment with phenytoin and phenobarbital.  Seizure semiologies included cephalic aura followed by bilateral arm movements with loss of awareness, and nocturnal convulsions. He underwent video EEG monitoring for consideration of surgical treatment. He had two seizures originating from the left frontocentral area. Interictal EEG showed left greater than right frontocentral slowing and sharp waves. He chose not to undergo surgery and continued to have seizures, with relatively poor adherence to antiepileptic drugs. Six years later he underwent repeat EEG monitoring for reconsideration of surgery.14 seizures from the same onset zone were captured, two of which were associated with asystole lasting between 10 and 14 seconds. Upon repeat review of his EEG from the initial admission, it was noted that he had ictal asystole at that time as well (Figure 1). A permanent pacemaker was placed. He again declined epilepsy surgery and continued to have seizures. Six years later, he died unexpectedly and was found by his family the next day, facing down. Autopsy showed moderate coronary atherosclerosis but no obvious medical cause of death. Electrophysiologic interrogation of the pacemaker showed intact function and minimal need for pacing.  Conclusions: The patient fulfilled criteria for definite SUDEP. Ictal asystole in this patient was inadvertently untreated for at least six years, suggesting that ictal asystole itself may not necessarily be a malignant condition. Moreover, his death demonstrates that a permanent pacemaker is not effective in preventing SUDEP. It is unclear whether a defibrillator would have been effective. While pacemaker placement may remain a necessary treatment in patients with ictal asystole, additional research is needed. Patients and clinicians should avoid feeling falsely reassured that a pacemaker ensures protection against SUDEP. Funding: None