Malformations of the Nervous System

Handbook of Clinical Neurology (Series Editors: Aminoff, Boller and Swaab)

Edited by

  • Harvey Sarnat, University of Calgary, Faculty of Medicine; Division Chief, Paediatric Neurology, Alberta Children's Hospital, Calgary, Alberta, Canada
  • Paolo Curatolo, Paediatric Neurosciences, Tor Vergata University of Rome, Rome, Italy

This volume deals with brain development malformations of the central nervous system, showcasing a unique approach that furthers research through systematic integration of exciting new developments from fields including molecular genetics, neuroimaging, and neuropathology. By integrating data and research from these disciplines, better conceptualization of the mechanisms of the developmental processes is achieved.

Clinicians will find invaluable insights into complex issues, including midline hypoplasias, disorders of segmentation of the neural tube, and hamartomatous disorders of cellular lineage, amongst others. The clinical manifestations of central nervous system malformations are also discussed, along with new advancements in MRI techniques and analysis, including volumetric morphology, spectroscopy, and functional neuroimaging.

Sections dedicated to management and treatment are also included in an effort to aid clinicians in their goal of providing better care for individuals affected by these types of malformations.

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Book information

  • Published: September 2007
  • Imprint: ELSEVIER
  • ISBN: 978-0-444-51896-5

Table of Contents

SECTION I. Specific malformations using the new integrative classification

Revised classification

Axes and gradients of the neural tube and gradients for a morphological and molecular genetic classification of nervous system malformations.

Midline hypoplasias

Holoprosencephaly. Septo-optic-pituitary dysplasia. Rhombencephalosynapsis. Embryology and malformations of the forebrain commissures.

Disorders of segmentation of the neural tube

Disorders of segmentation of the neural tube: Chiari malformations. Segmentation of the neural tube: agenesis of selective neuromeres. Cerebellar hypoplasias.

Hamartomatous disorders of cellular lineage

Tuberous sclerosis. Hemimegalencephaly syndrome. Disorders of radial neuroblast migration and cerebral cortical architecture. Periventricular nodular heterotopia. Gio12. Subcortical laminar (band) heterotopia. Lissencephaly type I
Lissencephaly type II. Schizencephaly.

Disorders of neural crest induction of non-neural tissues

Neural tube programming and the pathogenesis of craniofacial clefts: the neuromeric. organization of the head and neck: Part I. Neural tube programming and the pathogenesis of craniofacial clefts: the neuromeric
organization of the head and neck: Part II. The oral-facial-digital syndromes.

Other dysgeneses

Congential vascular malformations in childhood. Acquired, induced and secondary malformations of the developing central nervous system.

SECTION II. Comparative manifestations of central nervous system malformations

Epilepsy in patients with cerebral malformations. Neuromuscular disorders associated with cerebral malformations. Neuroendocrine complications of central nervous system malformations. Cerebral dysgeneses associated with chromosomal disorders. Cerebral dysgeneses secondary to metabolic diseases in fetal life.

SECTION III. Diagnostic methods

Imaging malformations of cortical development. Clinical neurophysiology of cortical malformations: magnetoencephalography and electroencephalography. Molecular genetic testing and genetic counselling. Embryology and neuropathological examination of central nervous system malformations.

SECTION IV. Management of central nervous system malformations

Medical treatment in children with central nervous system malformations. Surgical treatment of central nervous system malformations. Neurorehabilitation of children with cerebral palsy. Educational, cognitive, behavioral and language development issues.