Description

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Among physicians responsible for the care of human infants born with a malformed nervous system, there is a compelling need for an accessible synthesis and summary of modern embryological concepts of dysgenesis. This book integrates the morphological, clinical, neurological, genetic, neurophysiological and imaging aspects of cerebral malformation in the context of a lucid embryological explanation. Rather than presenting a catalog of anatomical abnormalities, the text focuses on pathogenesis and clinical aspects. Anatomical appearances are described by imaging in life, while neuropathological features are derived from findings at autopsy. Embryological explanations are identified as documented observation or speculation. Among the topics covered are encephaloceles as a disorder of the primitive streak and node; cerebral hypoplasia as a precocious differentiation of the ependyma before all mitotic generations of the neuroepithelial cells are completed; holosprosencephaly as a disorder of the lamina terminalis; agenesis of the corpus callosum as a failure of programmed glial cell death in the callosal plate in preparation for axonal growth cone passage; subcortical heterotopia as an arrest of late neuroblast migrations due to retraction of radial glial guide fibers from the pial surface of the brain. The literature is exhaustively reviewed and supplemented by the author's own experience. The book is richly illustrated throughout.