Sudden death, febrile seizures, and hippocampal and temporal lobe maldevelopment in toddlers: A new entity

Hannah C. Kinney, Amy E. Chadwick, Laura A. Crandall, Marjorie Grafe, Dawna L. Armstrong, William J. Kupsky, Felicia L. Trachtenberg, Henry F. Krous

Research output: Contribution to journalArticle

45 Scopus citations

Abstract

Recently, we reported hippocampal and temporal lobe abnormalities in 5 toddlers with sudden unexplained death in childhood (SUDC). The association of these anomalies with a high incidence (40%) of individual/ family histories of simple febrile seizures in the cases raised concern that febrile seizures can be associated with death. In a series of 64 toddlers with sudden death, we tested the hypothesis that an SUDC subset is characterized by hippocampal and temporal lobe maldevelopment and an individual and/or family history of simple familial seizures. Cases of sudden and unexplained death in children aged 1.0 to 5.9 years (median 1.7 years) were divided into groups based upon a history of febrile or nonfebrile seizures, familial febrile seizures, and autopsy classification of cause of death. Forty-nine of the 64 cases (77%) were classified as SUDC, of which 40% had an individual/family history of febrile seizures. Of the 26 SUDC cases with available hippocampal sections, 62% (16/26) had hippocampal and temporal lobe anomalies, including 82% (9/11) of cases with an individual/family history of febrile seizures. Cases with these anomalies were all found dead during a sleep period, typically in the prone (87%) position. We conclude that a potential new entity may account for the majority of SUDC in toddlers, defined by sleep-related death in the prone position, individual/family history of febrile seizures, and hippocampal and temporal lobe anomalies. The mechanism of death appears analogous to sudden death in (temporal lobe) epilepsy, with a putative unwitnessed seizure during sleep leading to airway occlusion and death. This study mandates further research into the potential link between simple febrile seizures and death.

Original languageEnglish (US)
Pages (from-to)455-463
Number of pages9
JournalPediatric and Developmental Pathology
Volume12
Issue number6
DOIs
StatePublished - Dec 1 2009

Keywords

  • Ammon's horn sclerosis
  • Microdysgenesis
  • Sudden infant death syndrome (SIDS)
  • Sudden unexpected death in epilepsy (SUDEP)
  • Sudden unexplained death in childhood (SUDC)
  • Temporal lobe epilepsy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

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    Kinney, H. C., Chadwick, A. E., Crandall, L. A., Grafe, M., Armstrong, D. L., Kupsky, W. J., Trachtenberg, F. L., & Krous, H. F. (2009). Sudden death, febrile seizures, and hippocampal and temporal lobe maldevelopment in toddlers: A new entity. Pediatric and Developmental Pathology, 12(6), 455-463. https://doi.org/10.2350/08-09-0542.1