Abstracts

To describe the clinical characteristics of patients with childhood-onset epilepsy who died from sudden unexplained death in epilepsy (SUDEP) at a comprehensive epilepsy center. The charts of all patients with onset of epilepsy less than age 18 years who suffered SUDEP between August 1992 and April 2004 were studied. The autopsy reports and circumstances of death were reviewed. Deaths were classified as possible, probable, or definite SUDEP based upon previously established criteria. Seventeen epilepsy patients (10 females and 7 males) suffered SUDEP. The average age of seizure onset was 4.3 [plusmn] 1.4 years (range 2 months to 17 7/12 years). Three patients presented with febrile seizures; all were less than or equal to 1 year of age. The average age of death was 12.6 [plusmn] 2.1 years (range 22 months to 27 years). The average duration of seizures was 9.2 [plusmn] 2.2 years (range 15 months to 26 years). There were 1 possible, 11 probable, and 5 definite cases of SUDEP. The results of 5 full autopsies were available; one child only had histopathological examination of the brain. All 5 patients with full autopsies were diagnosed with SUDEP. Seven patients (41%) had structural lesions by imaging or pathology. The lesions included a previously resected dysembryoplastic neuroepithelial tumor, prior repaired nasal encephalocele, hypoplastic optic nerves with a pericallosal lipoma, cortical dysplasia (2 patients), and right mesial temporal sclerosis. One patient had radiation necrosis and strokes in the right hemisphere following treatment for a left choroid plexus carcinoma. This case was classified as possible SUDEP due to other potential causes of death. Another patient had a prior right temporal lobectomy (pathology was not available); she had been seizure-free 2 years prior to her death. Another 5 patients (29%) had cognitive delay. Eleven children (65%) were found dead in bed. One patient was found pulseless and apneic 6 months prior to her death. All patients had generalized tonic-clonic (GTC) seizures (secondarily [10 patients], symptomatic [6 patients], or febrile seizures [1 patient]). Three patients had undergone a complete callosotomy for intractable generalized seizures. The average number of AEDs (antiepileptic drugs) given during the course of epilepsy was 5.5 [plusmn] 0.9 (range 1 to 13). At the time of death, patients were on an average of 1.6 [plusmn] 0.2 AEDs (range 0 to 3). Of the 3 autopsies with AED levels, all were subtherapeutic. Three patients had functioning vagus nerve stimulators. Although most of the children began having seizures before age five years, age at death was evenly distributed in childhood. Most children died in their sleep. The majority of patients (71%) had structural lesions, cognitive delay, or both. All patients had GTC seizures (secondarily, symptomatic, or febrile seizures). Some had successful epilepsy surgery and still suffered SUDEP.