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J Neuropathol Exp Neurol. 2016 Oct;75(10):981-997. Epub 2016 Sep 9.

Hippocampal Formation Maldevelopment and Sudden Unexpected Death across the Pediatric Age Spectrum.

Author information

1
From the Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (HCK, RLH, LT, RD); Epilepsy Genetics Program, Department of Neurology, F.M. Kirby Neurobiology Center, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (AHP); Division of Neuropathology, Beaumont Hospital, Dublin, Ireland (JBC); Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (LAS); Department of Genetics and Genomic Medicine, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (IAH, GTB, CB); Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (SPP, SKW); Division of Child Neurology, Nemours Children's Specialty Care, Jacksonville, Florida (HAS); Barrow Neurological Institute, Phoenix Children's Hospital, Department of Child Health, University of Arizona College of Medicine Phoenix Children's Hospital, Phoenix, Arizona (MK); Department of Pathology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, North Dakota (WLK); Department of Cellular Pathology Birmingham Women's Hospital, Birmingham, UK (BH); Division of Mental Health and Wellbeing, University of Warwick, and Coventry and Warwickshire Partnership NHS Trust, Coventry, UK (JG); Office of the Medical Examiner, County of San Diego, California (OJM); Department of Pathology, Rady Children's Hospital, San Diego, California (EAH); Department of Pathology, Baylor College of Medicine, Retired Professor of Pathology, Houston, Texas (DDA); Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Department of Medicine, Boston Children's Hospital, and Harvard Medical School, Boston, Massachusetts (RDG) Hannah.Kinney@childrens.harvard.edu.
2
From the Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (HCK, RLH, LT, RD); Epilepsy Genetics Program, Department of Neurology, F.M. Kirby Neurobiology Center, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (AHP); Division of Neuropathology, Beaumont Hospital, Dublin, Ireland (JBC); Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (LAS); Department of Genetics and Genomic Medicine, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (IAH, GTB, CB); Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts (SPP, SKW); Division of Child Neurology, Nemours Children's Specialty Care, Jacksonville, Florida (HAS); Barrow Neurological Institute, Phoenix Children's Hospital, Department of Child Health, University of Arizona College of Medicine Phoenix Children's Hospital, Phoenix, Arizona (MK); Department of Pathology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, North Dakota (WLK); Department of Cellular Pathology Birmingham Women's Hospital, Birmingham, UK (BH); Division of Mental Health and Wellbeing, University of Warwick, and Coventry and Warwickshire Partnership NHS Trust, Coventry, UK (JG); Office of the Medical Examiner, County of San Diego, California (OJM); Department of Pathology, Rady Children's Hospital, San Diego, California (EAH); Department of Pathology, Baylor College of Medicine, Retired Professor of Pathology, Houston, Texas (DDA); Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Department of Medicine, Boston Children's Hospital, and Harvard Medical School, Boston, Massachusetts (RDG).

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.

KEYWORDS:

Dentate gyrus; Febrile seizures; Granule cell dispersion; Sudden infant death syndrome (SIDS); Sudden unexpected death in pediatrics with hippocampal formation maldevelopment (SUDP-HFM); Sudden unexplained death in childhood (SUDC); Sudden unexplained death in epilepsy (SUDEP)

PMID:
27612489
DOI:
10.1093/jnen/nlw075

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