Hippocampal malformation associated with sudden death in early childhood: a neuropathologic study: Part 2 of the investigations of The San Diego SUDC Research Project

Forensic Science, Medicine, and Pathology
Marco M HeftiHannah C Kinney

Abstract

Sudden unexplained death in childhood (SUDC), while rare, accounts for an important fraction of unexpected deaths in children >1 year of age. Previously we reported an association between febrile seizures, hippocampal maldevelopment, and sudden, unexpected deaths in young children (1-6 years), termed "hippocampal maldevelopment associated with sudden death (HMASD)." Here, we characterize in greater detail the hippocampal pathology in a large cohort of cases (n = 42) of this entity, and attempt to define possible new entities responsible for sudden, unexplained death in young children without HMASD/febrile seizure phenotypes. We performed comparative analysis on cases, which we classified in a cohort of 89 sudden and unexpected deaths as HMASD, explained deaths, SUDC with febrile seizure phenotype (SUDC-FS) but without hippocampal pathology, and SUDC (without hippocampal pathology or febrile seizure phenotype). The frequency of each subgroup was: HMASD 48% (40/83); SUDC 27% (22/83); SUDC-FS 18% (15/83); explained 7% (6/83). HMASD was characterized clinically by sudden, sleep-related death, term birth, and discovery in the prone position. Key morphologic features of HMASD were focal granule cell bilamination of the dentate gyrus ...Continue Reading

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Citations

Mar 28, 2016·Forensic Science, Medicine, and Pathology·Michael J AckermanDeborah A Robinson
Mar 30, 2016·Forensic Science, Medicine, and Pathology·Paul A Rutecki, Thomas P Sutula
Mar 30, 2016·Forensic Science, Medicine, and Pathology·Marco M HeftiHenry F Krous
Mar 31, 2016·Forensic Science, Medicine, and Pathology·Colin Smith
Apr 21, 2016·Forensic Science, Medicine, and Pathology·Jeffrey Noebels
Sep 11, 2016·Journal of Neuropathology and Experimental Neurology·Hannah C KinneyRichard D Goldstein
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Feb 25, 2021·The Application of Clinical Genetics·Christine KeywanIngrid A Holm
May 28, 2016·Pediatrics·Richard D GoldsteinMarian Willinger

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