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Brainstem tegmental necrosis and olivary hypoplasia: raising awareness of a rare neuropathologic correlate of congenital apnea
Journal article   Peer reviewed

Brainstem tegmental necrosis and olivary hypoplasia: raising awareness of a rare neuropathologic correlate of congenital apnea

Christos D Katsetos, Carol E Anderson, Miguel A Guzman, Judy Mae Pascasio, Jean-Pierre de Chadarévian and Agustin Legido
Seminars in pediatric neurology, v 21(2), pp 177-183
Jun 2014
PMID: 25149959

Abstract

Abnormalities, Multiple - pathology Apnea - congenital Apnea - pathology Brain Diseases - congenital Brain Diseases - pathology Brain Stem - pathology Fatal Outcome Female Humans Infant, Newborn Necrosis
This case study describes an instance of death in an early term female newborn with congenital apnea in the clinical setting of multiple congenital anomalies (retrognathia, posteriorly rotated ears, camptodactyly, and arthrogryposis) and prenatal history of polyhydramnios. Postmortem neuropathologic findings were significant for tegmental necrosis in the caudal pons and medulla characterized by a coalescence of microcalcifications accompanied by neuronal loss, axonal spheroids, gliosis, and a concomitant hypoplasia of the inferior olives. This report raises awareness of the rare lethal entity of brainstem tegmental necrosis and olivary hypoplasia and its nosological relationship to the Möbius syndrome in the context of differential diagnosis of congenital apnea owing to central respiratory dysfunction.

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Web of Science research areas
Clinical Neurology
Pediatrics
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