Humanity Lost: The Cost of Cortical Maldevelopment. Is There Light Ahead?

Miyan, Jaleel; Sobkowiak, Carole; Draper, Clare E.

doi:10.1055/s-2001-19740

Cited by 12 publications

(6 citation statements)

References 45 publications

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“…In 2001, Miyan et al [69] asked a key question: "Humanity lost: the cost of cortical maldevelopment in hydrocephalus. Is there light ahead?"…”

Section: At Late Gestational Stages Vz Disruption In the Telencephalmentioning

confidence: 99%

Neural Stem Cells and Fetal-Onset Hydrocephalus

Rodríguez¹,

Guerra²

2017

Pediatr Neurosurg

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Fetal-onset hydrocephalus is not only a disorder of cerebrospinal fluid (CSF) dynamics, but also a brain disorder. How can we explain the inborn and, so far, irreparable neurological impairment in children born with hydrocephalus? We hypothesize that a cell junction pathology of neural stem cells (NSC) leads to two inseparable phenomena: hydrocephalus and abnormal neurogenesis. All neurons, glial cells, and ependymal cells of the mammalian central nervous system originate from the NSC forming the ventricular zone (VZ) and the neural progenitor cells (NPC) forming the subventricular zone. Several genetic mutations and certain foreign signals all convey into a final common pathway leading to cell junction pathology of NSC and VZ disruption. VZ disruption follows a temporal and spatial pattern; it leads to aqueduct obliteration and hydrocephalus in the cerebral aqueduct, while it results in abnormal neurogenesis in the telencephalon. The disrupted NSC and NPC are released into the CSF and may transform into neurospheres displaying a junctional pathology similar to that of NSC of the disrupted VZ. These cells can then be utilized to investigate molecular alterations underlying the disease and open an avenue into possible NSC therapy.

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“…In 2001, Miyan et al [69] asked a key question: "Humanity lost: the cost of cortical maldevelopment in hydrocephalus. Is there light ahead?"…”

Section: At Late Gestational Stages Vz Disruption In the Telencephalmentioning

confidence: 99%

Neural Stem Cells and Fetal-Onset Hydrocephalus

Rodríguez¹,

Guerra²

2017

Pediatr Neurosurg

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“…Additionally, other proteins (such as vimentin) appear over-expressed in Chiari II malformation and aqueduct stenosis [ 32 , 33 ]. These altered concentrations of signaling molecules may influence cerebral development [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Spina bifida aperta (SBA) is associated with cerebral pathology, such as hydrocephalus, Chiari II malformation, heterotopias and cortical abnormalities [ 1 - 3 ]. In SBA, ventriculomegaly of the posterior horns (i.e.…”

Section: Introductionmentioning

confidence: 99%

Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

Wit

Dunnen

Sollie

et al. 2008

Fluids Barriers CNS

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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.

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“…Revisions of shunt were associated with poor achievement particularly when the revisions were needed after the age of 2. The cranial sutures have usually fused by the age of 2 after which the skull is less expansile rendering the brain more susceptible to pressure [7]. Table 5 shows that most of those who had revisions after the age of 2 had had symptoms or signs of raised intracranial pressure.…”

Section: Discussionmentioning

confidence: 99%

Untitled

Hunt

Oakeshott

2004

Cerebrospinal Fluid Res

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Background and Methods : From 1963: From to 1971 babies with open spina bifida were treated non-selectively from birth. In 2002 we reviewed all the survivors by postal questionnaire and telephone call. The aims were to find out how many were living independently in the community or were in open employment or drove a car. In addition to these achievements we recorded health, medication and admissions to hospital and asked how much daily help they needed.

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Humanity Lost: The Cost of Cortical Maldevelopment. Is There Light Ahead?

Cited by 12 publications

References 45 publications

Neural Stem Cells and Fetal-Onset Hydrocephalus

Neural Stem Cells and Fetal-Onset Hydrocephalus

Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

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