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J Neurosurg. 2007 Mar;106(3 Suppl):213-21.

Fetal spina bifida in a mouse model: loss of neural function in utero.

Author information

1
Department of Pediatric Surgery, University Children's Hospital Zurich, Switzerland. stiefel_d@yahoo.co.uk

Abstract

OBJECT:

The devastating neurological deficit associated with myelomeningocele has previously been assumed to be a direct and inevitable consequence of the primary malformation-failure of neural tube closure. An alternative view is that secondary damage to the pathologically exposed spinal cord tissue in utero is responsible for the neurological deficiency. If the latter mechanism were shown to be correct, it would provide an objective rationale for the performance of in utero surgery for myelomeningocele, because coverage of the exposed spinal cord could be expected to alleviate or perhaps prevent neurodegeneration. To examine this question, the authors studied the development of neuronal connections and neurological function of mice during fetal and neonatal stages in a genetic model of exposed lumbosacral spina bifida.

METHODS:

The persistently exposed spinal cord of mouse fetuses carrying both curly tail and loop-tail mutations exhibited essentially normal anatomical and functional hallmarks of development during early gestation (embryonic Days 13.5-16.5), including sensory and motor projections to and from the cord. A significant proportion of fetuses with spina bifida at early gestation exhibited sensorimotor function identical to that seen in age-matched healthy controls. However, at later gestational stages, increasing neurodegeneration within the spina bifida lesion was detected, which was paralleled by a progressive loss of neurological function.

CONCLUSIONS:

These findings provide support for the hypothesis that neurological deficit in human myelomeningocele arises following secondary neural tissue destruction and loss of function during pregnancy.

Comment in

PMID:
17465388
PMCID:
PMC3651953
DOI:
10.3171/ped.2007.106.3.213
[Indexed for MEDLINE]
Free PMC Article

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