Hippocampal Formation Maldevelopment and Sudden Unexpected Death across the Pediatric Age Spectrum. Issue 10 (October 2016)

• Record Type: Journal Article
• Authors: Kinney, Hannah C.; Poduri, Annapurna H.; Cryan, Jane B.; Haynes, Robin L.; Teot, Lisa; Sleeper, Lynn A.; Holm, Ingrid A.; Berry, Gerald T.; Prabhu, Sanjay P.; Warfield, Simon K.; Brownstein, Catherine; Abram, Harry S.; Kruer, Michael; Kemp, Walter L.; Hargitai, Beata; Gastrang, Joanne; Mena, Othon J.; Haas, Elisabeth A.; Dastjerdi, Roya; Armstrong, Dawna D.; Goldstein, Richard D.
• Is Part Of: Journal of neuropathology and experimental neurology. Volume 75:Issue 10(2016)
• Publication Date: 2016-10
• Subjects: Dentate gyrus -- Febrile seizures -- Granule cell dispersion -- Sudden unexplained death in childhood (SUDC) -- Sudden infant death syndrome (SIDS) -- Sudden unexplained death in epilepsy (SUDEP) -- Sudden unexpected death in pediatrics with hippocampal formation maldevelopment (SUDP-HFM); Neurology -- Diseases -- Periodicals; Neurology -- Diseases -- Physiopathology -- Periodicals; 616.8047
• DOI: 10.1093/jnen/nlw075
• Languages: English
• ISSNs: 0022-3069

Abstract

Abstract : Sudden infant death syndrome (SIDS) and sudden unexplained death in childhood (SUDC) are defined as sudden death in a child remaining unexplained despite autopsy and death scene investigation. They are distinguished from each other by age criteria, i.e. with SIDS under 1 year and SUDC over 1 year. Our separate studies of SIDS and SUDC provide evidence of shared hippocampal abnormalities, specifically focal dentate bilamination, a lesion classically associated with temporal lobe epilepsy, across the 2 groups. In this study, we characterized the clinicopathologic features in a retrospective case series of 32 children with sudden death and hippocampal formation (HF) maldevelopment. The greatest frequency of deaths was between 3 weeks and 3 years (81%, 26/32). Dentate anomalies were found across the pediatric age spectrum, supporting a common vulnerability that defies the 1-year age cutoff between SIDS and SUDC. Twelve cases (38%) had seizures, including 7 only with febrile seizures. Subicular anomalies were found in cases over 1 year of age and were associated with increased risk of febrile seizures. Sudden death associated with HF maldevelopment reflects a complex interaction of intrinsic and extrinsic factors that lead to death at different pediatric ages, and may be analogous to sudden unexplained death in epilepsy.