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22 - Congenital malformations of the brain

from Section 4 - Specific conditions associated with fetal and neonatal brain injury

Published online by Cambridge University Press:  12 January 2010

By
Jin S. Hahn  and
Ronald J. Lemire
Edited by
David K. Stevenson ,
William E. Benitz ,
Philip Sunshine ,
Susan R. Hintz  and
Maurice L. Druzin
David K. Stevenson
Affiliation:
Stanford University School of Medicine, California
William E. Benitz
Affiliation:
Stanford University School of Medicine, California
Philip Sunshine
Affiliation:
Stanford University School of Medicine, California
Susan R. Hintz
Affiliation:
Stanford University School of Medicine, California
Maurice L. Druzin
Affiliation:
Stanford University School of Medicine, California
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Summary

Introduction

This chapter focuses on some of the more common brain malformations that are encountered early in life. Tremendous advances in neuroimaging with MRI in the past two decades have significantly improved our ability to diagnose brain malformations. In conjunction, there have been rapid advances in neurobiology that have led to better understanding of how the brain develops and what disturbances to the development lead to malformation. Each year more and more genes responsible for malformations are being discovered. Furthermore, modern fetal ultrasonography and more recently fetal MRI have increased the ability to detect a large variety of central nervous system malformations in utero. Prenatal detection and anatomic diagnosis of the malformations will better allow the medical caregivers to provide prognosis and management counseling.

Normal brain development

A brief overview of normal embryonic and fetal brain development will help to clarify the timing and etiology of brain malformations. Normal human brain development occurs in a highly defined spatial and temporal sequence of events in utero (Table 22.1). The temporal sequence consists of several overlapping phases. During the induction phase, signals sent to the ectoderm cause it to develop into neural tissue. The neural plate, a sheet of cells that will ultimately develop into the nervous system, develops by the 17th to 20th day of gestation. Neurulation occurs next, where the neural plate begins to fold into the neural tube, a process that begins by the 21st day.

Type
Chapter
Information
Fetal and Neonatal Brain Injury , pp. 265 - 276
Publisher: Cambridge University Press
Print publication year: 2009

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