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Related Concept Videos

Neurulation01:30

Neurulation

Neurulation is the embryological process which forms the precursors of the central nervous system and occurs after gastrulation has established the three primary cell layers of the embryo: ectoderm, mesoderm, and endoderm. In humans, the majority of this system is formed via primary neurulation, in which the central portion of the ectoderm—originally appearing as a flat sheet of cells—folds upwards and inwards, sealing off to form a hollow neural tube. As development proceeds, the anterior...
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Monitoring the Mechanical Evolution of Tissue During Neural Tube Closure of Chick Embryo
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Split medulla in association with multiple closed neural tube defects.

Ai Muroi1, Karen L Fleming, J Gordon McComb

  • 1Division of Neurosurgery, Childrens Hospital Los Angeles, Suite 1006, Los Angeles, CA 90027, USA. muroiai@aol.com

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|February 25, 2010
PubMed
Summary

This case study details a rare split medulla and multiple neural tube defects in a pediatric patient. Early gestational notochord splitting is implicated in these complex congenital abnormalities.

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Area of Science:

  • Neuroscience
  • Developmental Biology
  • Pediatric Neurology

Background:

  • Neural tube defects (NTDs) represent a significant group of congenital malformations.
  • Split medulla, a rare anomaly, involves the incomplete separation of the medulla oblongata.
  • Multiple, complex NTDs require thorough investigation and long-term management.

Observation:

  • A 13-year-old female presented with a history of neck abnormalities and a congenital midline occipital dermal sinus.
  • Advanced imaging revealed cervical vertebral segmentation abnormalities, a craniocervical junction bony spur, split medulla, split cervical spinal cord, and a dermal sinus.
  • Neurological status remained stable over an 11-year follow-up period.

Findings:

  • The patient exhibited a rare combination of split medulla and multiple closed neural tube defects.
  • Imaging confirmed complex cervical spine and spinal cord malformations.
  • The constellation of findings suggests early gestational notochordal splitting.

Implications:

  • Understanding early developmental errors is crucial for diagnosing and managing complex NTDs.
  • Surgical intervention for associated malformations should be guided by neurological progression.
  • This case highlights the importance of comprehensive evaluation in pediatric neurodevelopmental disorders.