Fetal brain damage in congenital hydrocephalus
- PDF / 391,137 Bytes
- 8 Pages / 595.276 x 790.866 pts Page_size
- 4 Downloads / 190 Views
FOCUS SESSION
Fetal brain damage in congenital hydrocephalus Maria Florencia Varela 1
&
Marcos M. Miyabe 1
&
Marc Oria 1,2
Received: 9 April 2020 / Accepted: 28 April 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Background Congenital hydrocephalus (HCP) is a developmental brain disorder characterized by the abnormal accumulation of cerebrospinal fluid within the ventricles. It is caused by genetic and acquired factors that start during early embryogenesis with disruption of the neurogerminal areas. As might be expected, early-onset hydrocephalus alters the process of brain development leading to irreparable neurological deficit. A primary alteration of the ependyma/neural stem cells (affecting vesicle trafficking and abnormal cell junctions) leads to its loss or denudation and translocation of neural progenitor cells (NPCs) and neural stem cells (NSCs) into the cerebrospinal fluid (CSF). Under these abnormal conditions, morphological and functional processes, underlying the concept of astroglial reaction, are initiated in an attempt to recover homeostasis in the periventricular zone. This astroglial reaction includes astrocyte hypertrophy, hyperplasia, and development of a new layer with reorganized functional features that resemble the ependyma. Despite decades of research, there is a lack of information concerning the biological basis of the brain abnormalities that are associated with HCP. Discussion The present review of current literature discusses the neuropathological changes during gestation following the onset of congenital hydrocephalus and the unanswered questions into the pathophysiology of the disease. A better understanding of those missing points might help create novel therapeutic strategies that can reverse or even prevent the ultimate neurological impairment that affects this population and improve long-term clinical outcome. Keywords Congenital hydrocephalus . Brain damage . Neurodevelopment . Fetal therapy
Abbreviations CNS Central nervous system CSF Cerebrospinal fluid EEA1 Early endosomal antigen 1 ETV Endoscopic third ventriculostomy GFAP Glial fibrillary acidic protein HCP Hydrocephalus HTx Hydrocephalus Texas L1CAM L1 cell adhesion molecule NSC Neural stem cells NPC Neural progenitor cells PCCH Perspective Classification of Congenital Hydrocephalus
* Marc Oria [email protected] 1
Center for Fetal and Placental Research, Cincinnati Children’s Hospital Medical Center (CCHMC), Cincinnati, OH, USA
2
Department of Pediatrics, College of Medicine, University of Cincinnati, Cincinnati, OH, USA
RGM SVZ TNFα TNFαR1 VEGF VZ
Repulsive guidance molecule Subventricular zone Tumor necrosis factor alpha Tumor necrosis factor alpha receptor 1 Vascular endothelial growth factor Ventricular zone
Introduction Congenital hydrocephalus (HCP) is a complex developmental disorder characterized by abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles [15, 93]. It has an incidence of between 68 and 316 per 100,000 births, with the greatest incidence i
Data Loading...
