Brainstem tegmental necrosis and olivary hypoplasia: a lethal entity associated with congenital apnea

Journal of Neuropathology and Experimental Neurology
S C Cortez, H C Kinney

Abstract

Previously, we reported an isolated case of a newborn with central apnea at birth, ventilator-dependence, and combined malformative and destructive brainstem lesions (1). We now report 2 additional cases with similar clinicopathologic features. All 3 patients were male (XY karyotype) and required immediate ventilatory support in the delivery room. Perinatal complications included polyhydramnios and breech presentation. Variable cranial nerve palsies and orofacial and limb anomalies were present. The patients dies within minutes of withdrawal of ventilatory support at 2 to 11 weeks after birth. Significant neuropathologic findings were localized to the caudal pons and medulla, and included tegmental necrosis (neuronal loss, gliosis, mineralization) with involvement of respiratory-related nuclei, and anomalies of rhombic lip derivatives (olivary hypoplasia, arcuate nucleus hyperplasia). Three-dimensional computer reconstructions facilitated clinicoanatomic correlations, and underscored the restriction of the lesions to pontine and medullary rhombomeres and rhombic lip. The histopathology of these cases suggests a malformative process occurring at the end of the first trimester (time of rhombic lip migrations), and a superimposed ...Continue Reading

Citations

Jul 16, 2004·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Harvey B Sarnat
Mar 23, 2004·Pediatric Neurology·Martin P MoyaThomas J Cummings
Feb 28, 2003·Medical Hypotheses·Slobodan R SekulićDusan P Vuleta
Apr 17, 2002·American Journal of Respiratory and Critical Care Medicine·Carmel HarringtonColin E Sullivan
Nov 8, 2002·American Journal of Respiratory and Critical Care Medicine·Paul M MaceyRonald M Harper
Aug 31, 2011·Arquivos de neuro-psiquiatria·Fulvio A ScorzaRonald M Harper
Jan 28, 2004·Birth Defects Research. Part A, Clinical and Molecular Teratology·Maria Joaquina Marques-DiasSergio Rosemberg
May 20, 2004·American Journal of Medical Genetics. Part a·Alain VerloesMarie-Noelle Van Thienen
Mar 8, 2005·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Marilyn T MillerChristopher Gillberg
Sep 7, 1999·Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus·M T Miller, K Strömland

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