Handbook of Clinical Neurology (Series Editors: Aminoff, Boller and Swaab) To order this title, and for more information, click here
Edited By Harvey Sarnat, MD, FRCPC, Professor of Paediatrics, Pathology (Neuropathology) and Clinical Neurosciences, 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
Description This is a further volume in the Handbook of Clinical Neurology (Series Editors: Michael J. Aminoff, Department of Neurology,
University of California, San Francisco, USA; Fran ois Boller, Bethesda, USA; Dick F. Swaab, Netherlands Institute for Neuroscience,
Amsterdam, the Netherlands).
The period of intrauterine and neonatal brain development is crucial for everything we will become
and can accomplish in the rest of our life. In that short period of time the outline of the human brain develops into a tremendously
complex organ consisting of 100 billion neurons, each making between 1000 and 100 000 contacts with other particular groups of neurons
by means of some 100 000 km of nerve fibers. Each group of neurons should be born at the right moment, migrate to the site where they differentiate and make their specific contacts in a limited critical period of brain development in order to function later in a normal
way. Building such a complex structure as the brain in such a brief period is certainly the most demanding task for nature. It is in
fact a wonder that it does not often end with catastrophic failures in one of the numerous exactly timed and extremely complex processes;
instead it mostly results in a healthy baby with good potential for the rest of its existence. This volume deals with those children
in whom brain development has resulted in a malformation of the central nervous system. This field has recently gained exciting new insights,
for instance from molecular genetics, which are integrated in this volume. Section I of this volume follows the new integrative classification
and deals with midline hypoplasias, disorders of segmentation of the neural tube, hamartomatous disorders of cellular lineage, disorders
of radial neuroblast migration and cerebral cortical architecture and other dysgeneses. Section II describes the different clinical manifestations
of CNS malformation, followed by sections on diagnostic methods, management and treatment. (Taken from the foreword by the series editors.)
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.
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