Pathogenesis of cerebral malformations in human fetuses with meningomyelocele
| Autor: | Linda C. Meiners, Esteban M. Rodríguez, Wilfred F. A. den Dunnen, Rosa I. Muñoz, Olga A de Wit, Oebele F. Brouwer, Deborah A Sival, KM Sollie |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2008 |
| Předmět: |
medicine.medical_specialty
Fetus Pathology congenital hereditary and neonatal diseases and abnormalities Neurology Ependymal Cell business.industry Research Germinal matrix medicine.disease lcsh:RC346-429 Hydrocephalus nervous system diseases Pathogenesis Neuroepithelial cell Cellular and Molecular Neuroscience Developmental Neuroscience Cerebral aqueduct embryonic structures medicine business lcsh:Neurology. Diseases of the nervous system |
| Zdroj: | Cerebrospinal Fluid Research, Vol 5, Iss 1, p 4 (2008) Cerebrospinal Fluid Research, 5:4 Cerebrospinal Fluid Research |
| ISSN: | 1743-8454 |
| Popis: | Background Fetal spina bifida aperta (SBA) is characterized by a spinal meningomyelocele (MMC) and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation) may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events. The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. Methods Seven fetuses and 1 neonate (16–40 week gestational age, GA) with MMC and 6 fetuses with normal cerebral development (22–41 week GA) were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US). After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. Results At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21–34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37–40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. Conclusion In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation and/or hydrocephalus. Since denuded areas cannot re-establish cell function, neuro-developmental consequences could induce permanent cerebral pathology. |
| Databáze: | OpenAIRE |
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