Pediatric Neuropathology

At last, a much-needed route map to pediatric neuropathology. Up to now a comprehensive volume covering of pediatric neuropathology simply did not exist. This atlas is thus a unique, all-encompassing reference providing material on the fundamentals of brain disorders of the nervous system that can affect fetuses, infants and young children, as well as the essentials of diagnosis in developmental brain pathology and neuroimaging. Emphasising the characteristic morphology and a concise summary of clinical features, pathogenesis, and genetics, the generously illustrated atlas presents more than 100 disorders. This compilation is a valuable resource for pathologists, pediatric neurologists and neurosurgeons, geneticists, neonatologists, radiologists, and pediatricians.

The preoperative study of patients who are candidates for epilepsy surgery often classifies their epileptic foci as “lesional” or “non-lesional” based upon evidence from neuroimaging. Many lesions not detected by MRI are found by microscopic examination of the resected tissue. Advances have been made in neuropathological techniques to study resected brain tissue and to specify the types of focal cortical dysgeneses and other lesions by extending microscopic findings by applying immunocytochemical markers that identify specific types and distributions of neurons and glial cells that denote tissue architecture. There may be etiological differences between focal and extensive cortical dysplasias involving many gyri or entire lobes of cerebral cortex. Of additional importance in pediatric brain resections is that these modern techniques also denote cellular maturation and can identify abnormal cells with mixed lineage. α-B-crystallin can serve as a metabolic tissue marker of epileptic activity, regardless of the presence or absence of a “structural” lesion by MRI or by conventional histopathology. Satellitosis may contribute to epileptogenic neurons and later to death of those neurons. The classification of malformations of the brain is a process requiring continuous updates that include genetics, neuroimaging, and neuropathology as new data emerge, but should not be exclusive to one region of the brain, such as cerebral cortex or cerebellum. Standardization in neuropathological terminology enhances scientific communication. The ILAE recently published a useful consensus classification of focal cortical dysplasias that is flexible to enable future revisions and changes as new data become available.

The child is neither an adult miniature nor an immature human being: at each age, it expresses specific abilities that optimize adaptation to its environment and development of new acquisitions. Diseases in children cover all specialties encountered in adulthood, and neurology involves a particularly large area, ranging from the brain to the striated muscle, the generation and functioning of which require half the genes of the whole genome and a majority of mitochondrial ones. Human being nervous system is sensitive to prenatal aggression, is particularly immature at birth and development may be affected by a whole range of age-dependent disorders distinct from those that occur in adults. Even diseases more often encountered in adulthood than childhood may have specific expression in the developing nervous system. The course of chronic neurological diseases beginning before adolescence remains distinct from that of adult pathology – not only from the cognitive but also motor perspective, right into adulthood, and a whole area is developing for adult neurologists to care for these children with persisting neurological diseases when they become adults. Just as pediatric neurology evolved as an identified specialty as the volume and complexity of data became too much for the general pediatician or the adult neurologist to master, the discipline has now continued to evolve into so many subspecialties, such as epilepsy, neuromuscular disease, stroke, malformations, neonatal neurology, metabolic diseases, etc., that the general pediatric neurologist no longer can reasonably possess in-depth expertise in all areas, particularly in dealing with complex cases. Subspecialty expertise thus is provided to some trainees through fellowship programmes following a general pediatric neurology residency and many of these fellowships include training in research. Since the infectious context, the genetic background and medical practice vary throughout the world, this diversity needs to be represented in a pediatric neurology textbook. Taken together, and although brain malformations (H. Sarnat & P. Curatolo, 2007) and oncology (W. Grisold & R. Soffietti) are covered in detail in other volumes of the same series and therefore only briefly addressed here, these considerations justify the number of volumes, and the number of authors who contributed from all over the world. Experts in the different subspecialties also contributed to design the general framework and contents of the book. Special emphasis is given to the developmental aspect, and normal development is reminded whenever needed – brain, muscle and the immune system. The course of chronic diseases into adulthood and ethical issues specific to the developing nervous system are also addressed. A volume in the Handbook of Clinical Neurology series, which has an unparalleled reputation as the world's most comprehensive source of information in neurology International list of contributors including the leading workers in the field Describes the advances which have occurred in clinical neurology and the neurosciences, their impact on the understanding of neurological disorders and on patient care

Since 1975, Dr. Kenneth Swaiman’s classic text has been the reference of choice for authoritative guidance in pediatric neurology, and the 6th Edition continues this tradition of excellence with thorough revisions that bring you fully up to date with all that’s new in the field. Five new sections, 62 new chapters, 4 new editors, and a reconfigured format make this a comprehensive and clearly-written resource for the experienced clinician as well as the physician-in-training. Nearly 3,000 line drawings, photographs, tables, and boxes highlight the text, clarify key concepts, and make it easy to find information quickly. New content includes 12 new epilepsy chapters, 5 new cerebrovascular chapters, and 13 new neurooncology chapters, as well as new chapters on neuroimmunology and neuromuscular disorders, as well as chapters focused on clinical care (e.g., Counseling Families, Practice Guidelines, Transitional Care, Personalized Medicine, Special Educational Law, Outcome Measurements, Neurorehabilitation, Impact of Computer Resources, and Training Issues). Additional new chapters cover topics related to the developmental connectome, stem cell transplantation, and cellular and animal models of neurological disease. Greatly expanded sections to increase your knowledge of perinatal acquired and congenital disorders, neurodevelopmental disabilities, pediatric epilepsy, and nonepileptiform paroxysmal disorders and disorders of sleep. Coverage of new, emerging, or controversial topics includes developmental encephalopathies, non-verbal learning disorders, and the pharmacological and future genetic treatment of neurodevelopmental disabilities.

Prion diseases are rare in children. Three types are known: kuru, variant Creutzfeldt–Jakob disease (CJD), and iatrogenic CJD. All three affect children and young adults, and are transmitted by infectious contamination. Kuru was the result of ritual funeral practices similar to cannibalism; variant CJD affects young people who have eaten meat from cows with mad cow disease (mostly in the UK); and iatrogenic CJD is secondary to graft of human tissues performed in the 1980s (dura mater, pituitary extracted growth hormone). The disease appears after 4–30 years of incubation. The initial symptomatology is frequently neurological (cerebellar ataxia, oculomotor disturbance, peripheral nerve pain, pyramidal syndrome) followed by dementia. There is no biological test available that can give a definite diagnosis of prion disease apart from neuropathology, although prion accumulation in vCJD can be demonstrated in pharyngeal tonsil by immunohistochemical techniques. This devastating disease results inevitably in death. No specific treatment is available.

Child Neurology: Its Origins, Founders, Evolution and Growth, Second Edition updates the first biographical study of important contributors to the field of child neurology, consisting of over 250 biographical sketches written by over 100 physicians specializing in neurology, child neurology, pediatrics and obstetrics. Organized chronologically into six chapters, beginning before 1800 and continuing to the present, Child Neurology traces the emergence of child neurology as a separate specialty from its roots in pediatrics and neurology. With a definitive historical introduction by the editor, Dr. Stephen Ashwal. This new edition will feature a new section on The Dynamic Growth and Expansion of Child Neurology: The Late Twentieth Century (1960 to 2000+) and features about 138 new biographical sketches of leaders in the field during this recent time frame. Child Neurology: Its Origins, Founders, Evolution and Growth, Second Edition will be published on behalf of the Child Neurology Society, a professional society that strives to foster recognition and support for children with neurological disorders and to promote and exchange national and international scientific research, education, and training in the field of neurology. Identifies top contributors to child neurology research from the 1800s to today Includes 238 biographical sketches of contributors and their scientific research Contains 138 new biographies on contributors from the late 20th and early 21st centuries Authored by physicians and published by the Child Neurology Society

Neurodevelopmental disorders result from an inordinate number of genetic and environmental causes during the embryological and fetal periods of life. In the clinical setting, deciphering precise etiological diagnoses is often difficult. Newer screening technologies allow a gradual shift from traditional nature-versus-nurture debates toward the focused analysis of gene-by-environment interactions (G X E). Further understanding of developmental adaptation and plasticity requires consideration of epigenetic processes such as maternal nutritional status, environmental toxins, maternal illnesses, as well as genetic determinants, alone or in combination. Appreciation of specific G X E mechanisms of neurodevelopmental pathogenesis should lead to better risk-modifying or preventive strategies. We provide a brief overview of clinical and experimental observations that link prenatal-onset toxic exposures, metabolic disturbances, and maternal illnesses to certain neurodevelopmental disorders.