Abstracts

Rationale: SUDEP (Sudden Unexplained Death in Epilepsy) can be defined as death in people diagnosed with epilepsy that occurs unexpectedly during normal activities while in a reasonable state of health. In addition, there must be no obvious medical cause of death during postmortem examination. However, often medical examiners do not use SUDEP as a cause of death (COD), as patients with epilepsy have comorbidities that present alternative causes of death. In addition, epilepsy itself can be listed as the COD, which may be too broad and can contribute to a substantial underestimation of SUDEP incidence. Risk factors for SUDEP include refractory convulsions, early age of seizure onset, young age, polytherapy and non-adherence to medication regimen. There are numerous options now available to patients with pharmacoresistant epilepsy, including epilepsy surgery (eg. resection of epileptogenic zone, corpus callosotomy, laser surgery), responsive neurostimulator and the vagal nerve stimulator. Despite these advances in treatment, SUDEP remains the most frequent cause of epilepsy related death and the second leading neurologic cause of productive life-years lost. Numerous studies have shown that the rates of SUDEP decrease two years after VNS implant. Here we present the first report of a patient who had Aspire SR VNS, significantly improved seizure control and SUDEP. Methods: 29 year old woman who has a 24 year history of refractory epilepsy, characterized by auras before going to sleep followed by nocturnal generalized tonic-clonic seizures (GTCs). Her medication regimen included levetiracetam, lacosamide, oxcarbazepine, and zonisamide which all were titrated to tolerability. She had epilepsy surgery evaluation, including intracranial EEG monitoring and was not considered a candidate for surgical resection due to the presence of eloquent cortex in the epileptogenic zone. She continued to have 1-2 GTCs per month and had one episode of status epilepticus requiring intubation and ICU management. Results: After the episode of status epilepticus, decision was made to proceed with insertion of a VNS. She was implanted with the AspireSR vagal nerve stimulator (VNS) in SEP2015. She had one convulsion two days before the device was turned on. After VNS was turned on, she reported marked improvements in quality of life and no convulsions for over two months. She had a single convulsion on 12/29/15 and on 1/7/16 (4 months after VNS implant), she was found face down and dead in her bed. The COD, as determined by a medical examiner, was SUDEP. Her brain was transferred to New York University for enrollment in epilepsy death study protocol. VNS was retrieved and sent to LivaNova for evaluation. There was no evidence of device malfunction. Conclusions: This is the first reported case of SUDEP in a patient with data recorded by Aspire SR VNS after FDA approval of the aspire VNS. Post-mortem analysis of this patient's brain along with recordings from the VNS may provide insight into the mechanism of SUDEP. The current laxity in the criteria for death in patients with epilepsy has likely resulted in a gross underestimation of the incidence of SUDEP. Analysis of this case strongly suggests SUDEP as the COD and highlights the importance of a well-developed and recognized criteria for deaths directly related to epilepsy (SUDEP, status epilepticus, drowning, motor vehicle accidents, etc.) versus deaths indirectly due to epilepsy (aspiration pneumonia, suicide, cardiovascular disease). This case also highlights the importance of getting post-mortem examination on people with death related to epilepsy. Funding: None.