Neurop Diatrie

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

A comprehensive review of recent advances in the most severe form of epilepsy, focusing on two areas in which progress has been most rapid: basic mechanisms and treatment. Interest in status epilepticus--the most extreme form of epilepsy, involving continuous seizures--has surged in the last 20 years. Since 1979 there have been over 4,000 publications on the subject, including more than 1,700 in the last five years. No other text provides such a comprehensive review of the recent advances in the field of status epilepticus. The book focuses on the two areas in which progress has been most rapid: basic mechanisms and treatment. There is now a greater understanding of the mechanisms and complications of status epilepticus at the molecular level, which should eventually lead to improved therapy, and treatment strategies today have a greater sense of urgency because of the realization that neuronal apoptosis and necrosis can be triggered very quickly. After an overview of history, classification, and epidemiology, the contributors consider clinical phenomenology, biological markers, pathophysiology, brain damage, epileptogenesis, therapeutic principles, pharmacology, and therapeutic management. Their contributions are equally divided between studies of basic mechanisms in animal models and clinical studies, so that the reader can turn easily from the reductionist experiment that isolates a small component of status to the complex clinical situation in which these principles can translate into therapeutic action. The goal is to provide a scientific rationale for clinical decisions while developing therapeutic attitudes that are firmly grounded in pathophysiology.

Les chemins qu’empruntent les enfants pour apprendre gardent toujours une part de mystère et réservent nombre de surprises, le plus souvent fort agréables. Lorsque des embûches se présentent, lorsque l’enfant peine pour apprendre, les parents, l’entourage familial ou éducatif au sens large ainsi que les enseignants, sont bien souvent pris au dépourvu. L’appel aux professionnels de santé est devenu un recours incontournable, dès que des « difficultés d’apprentissage » sont repérées chez un enfant. Écouter, observer... pour pouvoir aider au mieux, voici le défi auquel sont confrontés les soignants en collaboration avec la famille et l’équipe enseignante. Les auteurs de cet ouvrage – pédopsychiatres, psychologues, orthophonistes – s’appuient sur une longue expérience clinique de consultation hospitalière spécialisée dédiée aux troubles du langage et des apprentissages chez l’enfant (centre référent). L’enjeu d’un bilan pluridisciplinaire réalisé dans ce cadre est de proposer une analyse approfondie des difficultés, et de dégager, à partir des observations recueillies, des points d’appui et des leviers afin d’orienter les actions thérapeutiques et les remédiations dont pourra bénéficier l’enfant en difficulté. Les nombreuses vignettes cliniques décrivant le parcours des enfants au sein de ce centre référent ainsi que la description des outils de travail clinique utilisés, feront partager au lecteur l’expérience quotidienne de l’équipe d’auteurs cliniciens. Largement illustré par des cas cliniques évocateurs, ce livre intéressera tous les acteurs du soin – équipes de proximité (CMP, CMPP, SESSAD... ), équipes hospitalières spécialisées et praticiens libéraux (orthophonistes, psychologues, psychomotriciens, ergothérapeutes, psychopédagogues, pédiatres, pédopsychiatres, neuropédiatres...) – ainsi que les acteurs des champs pédagogique, éducatif et associatif, engagés auprès des enfants, sans oublier les familles.

Jean Aicardi Days, October 1995. These scientific days were organised in tribute to Jean Aicardi, the internationally recognised neuropaediatrician. His friends and colleagues, eminent specialists from all over the world, exchanged their experiences and latest data on infantile epilepsy, with particular emphasis on aspects including migraine and epilepsy, the environmental determinants of cerebral development and cognitive disorders.

This book is devoted to the neuropsychological description of childhood epilepsy, a neurolo- cal condition that constitutes one of the most prevalent forms of chronic and disabling childhood illnesses. Indeed, one child out of 20 experiences one or more seizures before the age of 5, and one in a hundred develops epilepsy as a chronic disorder. Approximately half of these children with epilepsy display academic difficulties and/or behavioral disorders. Moreoever, it is now believed that a sizable proportion of children with learning disability suffer from undiagnosed epilepsy. While a great number of textbooks have been devoted to various medical aspects of chi- hood epilepsy (diagnosis, genetics, etiology, drug and surgical treatment, etc.), there have been no comprehensive accounts of the cognitive consequences of this condition. Advance of medical knowledge has shown that childhood epilepsy should not be considered as a single disorder but encompasses a whole range of different conditions that exhibit specific clinical EEG and outcome characteristics. It is not becoming apparent that these various clinical entities have different cognitive expression that yet need to be specified. The purpose of this book is to provide a complete up-to-date analysis of this multi-faceted pathology.

Idiopathic generalised epilepsies are characterised by strong genetic factors and multiple clinical phenotypes; animal models of untreated epilepsies are relevant to some of the clinical syndromes found in humans. This volume is the first to confront human clinical, animal (experimental) as well as basic and applied genetic data. Main section headings: Current approaches; Familial neonatal and infantile convulsions; Absence seizures and absence epilepsy; Juvenile myoclonic epilepsy and related syndromes; Photosensitivity; Pathophysiology of convulsive seizures; Fundamental and therapeutic aspects.

No detailed description available for "Review Articles, including those from an International Conference held in Bielefeld, F. R. of Germany, June 1-2, 1984".

The second volume of the Handbook does not parallel any volume of the first edition; it is one more sign, or reflection, of the expansion of the field. By emphasizing the experimental approach, it illustrates the tools that have re cently become available for investigating the nervous system. Also, perhaps even more than other volumes, it illustrates the multidisciplinary nature of the field, requiring multidisciplinary methodology. It is now recognized that the availability of methodology is often the rate-limiting determinant of studies and that improvements or innovations in instrumentation can open up new avenues. A new improved method, although opening up new possibilities and being crucial to making advances, is only a tool whose use will determine its use fulness. If we do not recognize its possibilities, its use will be limited; if we do not recognize its limitations, it will mislead us. It is the possibilities and limitations and the results obtained that are illustrated here.