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Neuropathologic Changes in Sudden Unexplained Death in Childhood.

Declan McGuone¹, Dominique Leitner², Christopher William^3,4

¹From the Department of Pathology, Yale School of Medicine, New haven, Connecticut.

Journal of Neuropathology and Experimental Neurology

|January 30, 2020

Summary

Sudden unexplained death in childhood (SUDC) in children over one year old is often linked to hippocampal abnormalities. Neuropathologic investigation revealed these alterations, though their exact cause and significance remain unclear.

Keywords:

Dentate gyrus Hippocampal abnormalities Neuropathology Pediatric Seizure Sudden unexplained death in childhood

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Area of Science:

Neuropathology
Pediatric Pathology
Sudden Death Autopsy

Background:

Sudden unexplained death in childhood (SUDC) affects children over one year old.
Causes remain unexplained after comprehensive investigation.
Hippocampal abnormalities are frequently associated with SUDC.

Purpose of the Study:

To conduct a systematic neuropathologic investigation of SUDC cases.
To analyze autopsy data, molecular studies, MRI, and histology.
To assess the frequency and characteristics of hippocampal abnormalities in SUDC.

Main Methods:

Prospective neuropathologic examination of 20 SUDC cases.
Inclusion of autopsy data, molecular testing, ex vivo 3T MRI, and histology.
Blinded neuropathologic review by two experts.

Main Results:

Hippocampal abnormalities, particularly in the dentate gyrus, were the most common finding.
Molecular testing identified genetic mutations in some cases, possibly explaining 3 deaths.
Hippocampal findings occurred similarly in cases with and without molecular explanations, and did not correlate with seizure history.

Conclusions:

Hippocampal alterations are a common neuropathologic finding in SUDC.
The significance and specificity of these hippocampal findings are uncertain.
Further research is needed to determine if hippocampal changes are causative, a consequence of seizures, or incidental.