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The aim of this book is to take a critical look at what is known about outcome of childhood epilepsies, specifically evidence-based findings, and further clarify the direction of clinical and fundamental research for the future. At the time a diagnosis of epilepsy is made for a child, it is highly desirable to predict seizure control and social outcome several months or even years later. Determination of outcome is, however, complex and in order to confront this challenge, a number of simple questions should be addressed: What is to be predicted? This may be seizure control, remission with or without ongoing AED treatment, intractability, social outcome, quality of life, or a combination of the above. What is the purpose of attempting to predict outcome and who will use the information? How accurate is the prediction?
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Having appeared in the 1930s in Montreal, standardised neuropsychological evaluation has become an essential tool in the clinical diagnosis and evaluation of surgical epileptic patients. Nevertheless, despite great progress over the last 20 to 30 years in the diagnosis and medical treatment of epilepsy, clinical neuropsychology still remains largely associated with surgical epilepsy, particularly surgery of the temporal lobe. Clinical neurology has still not managed to clear a way in the daily practice with patients with all types of epilepsy despite significant advances in cognitive neuroscience and a large number of clinical studies on epilepsy and cognition. How is it that there are only rarely major advances in the field of clinical neuropsychology? It has long been time for this question to be asked, and for an attempt to be made to bring about changes. This was the aim of the Toronto workshop and the result of this book. Every approach was debated, providing important elements to reflect on and allowing a great forum for exchanges. This book includes the communications from the main participants and comments from some others on specific subjects.
Progressive myoclonus epilepsies are a group of rare genetic diseases. The onset generally occurs around puberty in otherwise healthy children. They all involve myoclonus and epilepsy but then differ depending on the different symptoms that are related. The outlook of these diseases is nearly always unfavourable and treatment only focuses on symptoms. Much planning will be needed to improve the quality of life for these children who will gradually become over time more and more severely disabled. Among these diseases, the most notable is Unverricht-Lundborg disease and Lafora disease, among others. However, the genetic mechanism of these diseases is simple and has been perfectly identified o...
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Congenital or early-onset disorders of the nervous system have a profound and lifelong impact on the lives of children and their families. Diseases of the Nervous System in Childhood provides up-to-date information on the full range of these neurological disorders, from fetal and neonatal neurology to adolescence. Movement disorders, epilepsies and seizure disorders, metabolic diseases, auditory and visual disorders, and genetic anomalies are among the many topics covered in this text. Extensive reference lists at the end of each chapter guide the clinician to further relevant reading. This fourth edition retains the patient-focussed, clinical approach of its predecessors. The international team of editors and contributors has honoured the request of the late Jean Aicardi, that his book remain ‘resolutely clinical’, which distinguishes Diseases of the Nervous System in Childhood from other texts in the field. This edition: Is completely revised and updated Includes latest developments in genetic advances Contains new chapters on basal ganglia diseases and psychogenic disorders Has an easy-to-use one volume format with full-colour illustrations
The present book is the fruit of a workshop, designed as a discussion forum, with the participation of experts from all over the world, to extensively review clinical, neurophysiological and fundamental research available data in order to generate new axes for research, clinical practice and care. The first section traces back to the definitions and concepts underlying the terms “generalized seizures and epilepsies”. Section II reviews human and animal data suggesting that the brainstem network plays an important role for tonic seizures generation. The third and fourth sections analyze recent knowledge on cortico-thalamic and basal ganglia networks in absence and myoclonic seizures, both in animal models and in humans. The fifth section compares the phenomenology of “Primary versus Secondary Tonico-clonic seizures”, including animal data, clinical expression in humans and genetics. Section VI goes back to the discussion “Cortical” versus “Centrencephalic” theories. The last two chapters thoroughly review the clinical applications of current knowledge, in terms of pharmacological approach and clinical care.