Pediatric Neurology

Author by : Olivier Dulac
Languange : en
Publisher by : Newnes
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Description : 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


Pediatric Neurology Part Iii

Author by : Anonim
Languange : en
Publisher by : Newnes
Format Available : PDF, ePub, Mobi
Total Read : 65
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Description : 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.


Pediatric Neurology Part I

Author by : Robert H.A. Haslam
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : A thorough but focused history and neurological examination remain the most important initial elements of neurological diagnosis at all ages. Advances over the past two decades in clinical neurophysiology, neuroimaging, genetics, and neuropathological examination of tissue have at times appeared to predominate over traditional history and physical exam, but no laboratory studies can provide the focus and clues to diagnosis that clinical findings offer. History taking and the techniques of neurological examination are skills to be learned by the student, refined by the resident, and practiced and perfected throughout the career of a pediatric neurologist. Examination must be specifically modified to correspond to age and with the expectation of developmental skills achieved at various ages, in addition to the localizing value of particular signs that may apply at all ages. Hypotonia, extensor plantar responses, and lack of visual fixation may be normal in a preterm infant but abnormal at several months of age. “Primitive” reflexes disappear at a certain age, but really are only suppressed or inhibited and may become re-expressed with disinhibition many decades later. Finally, the pediatric neurologist needs to have a firm foundation in normal development, neuroembryology, and changes in the expression of diseases at various stages of maturation of the nervous system.


Pediatric Neurology Part I

Author by : Gabriel M. Ronen
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : The practice of pediatric neurology demands a high level of responsibility at multiple levels. These include listening carefully to people's stories in order to assess each situation, planning and implementing investigations and therapies, individual and family counseling, longitudinal follow-up from fetal life throughout childhood and adolescence, organization of transition to adult care, and societal advocacy. In the 21st century these activities must be carried out in the context of major societal and technological changes which have brought about many new challenges for pediatric neurologists. In this chapter, we address ethical and moral issues that may help guide pediatric neurologists with regard to a number of specific challenges. These include physician–patient relationships that are based on benign paternalism with respect for autonomy and promoting quality of life, practicing evidence-based medicine, and the technological imperative. In addition we discuss the tension between clinical practice and research, relationships between physicians and industry, and the public role of pediatric neurologists to advocate for children with neurological and developmental conditions. We also illustrate some challenges in selected situations such as prenatal counseling (fetal neurology), neonatal encephalopathy, and persistent vegetative state.


Textbook Of Pediatric Neurology

Author by : Gerald J. Golden
Languange : en
Publisher by : Springer
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Description : Neurological signs or symptoms are present in approximately 20% of all chil dren admitted to the hospital. These may be the reason for admission or may be part of preexisting and often unrelated problems. In ambulatory practice, acute neurological disease is not seen as frequently, but issues relating to normal and abnormal development are constantly being faced. For these reasons, familiarity with the progress of normal development and factors interfering with it, as well as knowledge of the major acute and chronic disorders of the nervous and neu romuscular systems, is important for any practitioner, specialist, or generalist who cares for children. The pathophysiology of neurological disorders in childhood is based on the same principles of the organization, structure, and function of the nervous sys tem as apply to adults. Two pitfalls are present for the student, however. First, the abnormalities are superimposed on a changing, developing brain, not a rather static, mature organ. The manifestations of the disease may vary, there fore, in seemingly unpredictable fashion depending on the rate of progression of the disorder and the rate and adequacy of the ongoing developmental changes in the nervous system. The second problem is the large number of unfa miliar conditions, many of which have no counterpart in adult neurology or medicine. These include developmental malformations, disorders specific to the neonatal period, and many hereditary and metabolic diseases.


Pediatric Neurology Part I

Author by : Gabriel M. Ronen
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : In this chapter we argue that modern thinking about chronic neurological and developmental conditions requires that we recognize all aspects of functioning and quality of life in addition to the biomedical dimensions of these disorders. We find the International Classification of Functioning, Disability, and Health (ICF) a useful framework by which to think about and understand the many dimensions of health; and we appreciate its heuristic value as a stimulus to consider a range of outcomes of these individuals. Quality of Life (QOL) and Health-Related Quality of Life (HRQL) cover the additional perceived dimensions that are so important to patients’ personal valuations, aspirations, and satisfaction about health throughout their lifespan, of which we see QOL being the broader construct. In order to measure outcomes at either the individual clinical or research level it is essential to ask clear and specific questions as a prelude to selecting measures that are appropriate in terms of both their content and their measurement properties. We provide some brief guidelines that we hope will be helpful to readers who wish to expand their activities in measuring clinical outcomes


Iap Textbook Of Pediatric Neurology

Author by : Anoop Verma
Languange : en
Publisher by : Jaypee Brothers Medical Publishers
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Description :


Pediatric Neurology Part I

Author by : Jean-Paul Amann
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : This chapter addresses the issue of the balance between protection of children submitted to research and children's access to new treatments, focusing on the field of antiepileptic drugs. Due to the lack of interest pharmaceutical companies have for such an unrewarding market, ethical and legal problems, difficulties recruiting patients, and in Europe insufficient public resources, many infants and children with epilepsy are still exposed to off-label drugs. The incentives and regulations specifically directed toward research in children in the United States and EU provided the first step to change this condition. The challenge is to perform trials determined by the needs of children and to obtain benefits in the same order of magnitude as for adults but with reduced risks. In order to optimize the development of new AEDs in pediatrics, the new European guidelines (2010) recommend the identification of adult clinical trial results that can be extrapolated to the pediatric population (i.e., those in partial epilepsies) and the use of innovative strategies that help limit the number of pediatric patients enrolled in trials (i.e., those with epileptic encephalopathies). A key step will be to develop international networks of pediatric epilepsy centers with the shared purpose of optimizing development and execution of clinical trials.


Pediatric Neurology Part I

Author by : Kate Himmelmann
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description :


Pediatric Neurology Part I

Author by : Ritva Paetau
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Cortical generators of epileptic and certain physiological activity can be localized noninvasively by magnetoencephalography (MEG). MEG detects weak magnetic fields produced by the postsynaptic currents of pyramidal cortical cells in sulcal walls. Unlike EEG, MEG signals are not distorted by edema or bone defects, and unlike fMRI, abnormal hemodynamics do not alter the MEG. The patient's head is centered inside a helmet housing over a hundred magnetic field sensors. Cortical generators of MEG signals are determined with a useful spatial resolution and an excellent time resolution, which enable tracking of brain activity in successive points of, for example, an epileptic network. MEG sources can be co-registered and visualized on magnetic resonance images (MRI). MEG is highly sensitive for the detection of interictal epileptic discharges, and present techniques allow some degree of head movements enabling ictal recordings also. MEG is also useful for localizing the somatosensory, visual, and language areas before tailored surgery in the vicinity of eloquent cortex. In conjunction with other noninvasive modalities MEG provides nonredundant data in one-third of epilepsy surgery patients. Clinical MEG utilization is mainly focused on presurgical localization of the epileptogenic zone and eloquent cortex in epilepsy surgery candidates, including patients with Landau–Kleffner syndrome. However, MEG is also an excellent noninvasive tool to study the source distribution in childhood epilepsy syndromes and epileptic encephalopathies.


Pediatric Neurology

Author by : Olivier Dulac
Languange : en
Publisher by : Newnes
Format Available : PDF, ePub, Mobi
Total Read : 73
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Description : 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


Pediatric Neurology

Author by : Tena Rosser
Languange : en
Publisher by : Lippincott Williams & Wilkins
Format Available : PDF, ePub, Mobi
Total Read : 31
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Description : Pediatric Neurology for the Oral Boards: A Case-Based Review is the first pediatric neurology review book written specifically for neurology residents preparing for the oral boards. The book presents sixty cases with discussions structured according to the neurology oral boards format: localization of neurologic findings; differential diagnosis and most likely diagnosis; diagnostic workup; and patient management. The cases will help readers lay a foundation of knowledge in pediatric neurology and develop an organized approach to clinical decision-making. An introduction explains in detail what to expect on the examination and gives helpful hints on preparing for and taking the exam.


Acute Pediatric Neurology

Author by : Thomas Sejersen
Languange : en
Publisher by : Springer Science & Business Media
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Description : This book provides recommendations for evaluation and therapy in the area of acute pediatric neurology; these are presented didactically with frequent use of illustrations and algorithms. Chapters in the first part of the book discuss presenting symptoms of acute neurological conditions. The second part of the book covers major areas of acute pediatric neurology and each of these chapters has three key elements: description of presenting symptoms; recommended assessments; and recommended interventions. Acute Pediatric Neurology provides an accessible, clinically focused guide to assist physicians in the emergency ward or intensive care unit in decisions on diagnosis and therapeutic interventions in all major acute pediatric neurological diseases.


Frontiers In Pediatric Neurology

Author by : TM Ananda Kesavan
Languange : en
Publisher by : JP Medical Ltd
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Description : This book is a complete guide to paediatric neurology for clinicians. Beginning with an overview of potential pitfalls in neurological examination of children, the following chapters discuss the diagnosis and management of numerous neurological disorders that may be encountered in daily paediatric practice. Topics covered include cerebral palsy, Guillan-Barré Syndrome, febrile seizures, muscle disorders, cerebral edema, epilepsy, neurogenic bladder dysfunction and much more. Each section is presented with an emphasis on the importance of accurate clinical examination, and covers all the latest developments and management strategies. The comprehensive text is highly illustrated with clinical images and diagrams to enhance learning. Key Points Comprehensive guide to paediatric neurology Covers diagnosis and management of numerous disorders with emphasis on importance of accurate clinical examination Includes discussion on potential pitfalls in neurological examination of children Highly illustrated with clinical photographs and diagrams


Pediatric Neurology Part I

Author by : Peter Uldall
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : The disclosure of diagnosis for a child with cerebral palsy (CP) is a highly stressful experience to the parents. The experience can be alleviated by clarity, empathy, and an emphasis on the child's resources and abilities. Despite chronic stress many families function well and manage to strengthen the child and family's resources through spousal and family support, maintenance of hope for further development, and active care taking. The caregiver burden can be divided into an objective burden (socio-structural constraints) and a subjective burden (emotional distress). The subjective burden of care seems less important, as illustrated by the quote: “We are tired, not sad.” Quality of life is similar in 8- to 12-year-old European children with CP and controls, whereas participation in daily life was lower for children with CP. Participation varies significantly among countries implying that some countries can improve in this area. In a study from Denmark only 29% of adults with CP were employed (versus 88% of controls), 25% were cohabitating, and 20% had children. These long-term achievements could be predicted from development quotient, CP type, and motor impairment at age 5. The goal of habilitation is integration into society, which is not achieved for the majority.


Swaiman S Pediatric Neurology E Book

Author by : Kenneth F. Swaiman
Languange : en
Publisher by : Elsevier Health Sciences
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Description : 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.


Pediatric Neurology Part I

Author by : Marie Vidailhet
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Symptomatic treatment of cerebral palsy (CP) is difficult, with variable beneficial effect. The choice of therapy is guided by the main clinical features (spasticity, dystonia/choreoathetosis), by the experience of experts, and by the results of open-label trials and a few controlled studies. Treatments of spasticity are not discussed in depth here. From open-label trials and a few controlled studies in dystonia/choreoathetosis CP, it appears that treatment should be started at a low dose and increased slowly, and that more beneficial effects are obtained on upper extremity function, face and jaw dystonia and drooling, and in children. L-Baclofen or antiepileptic drugs are rarely effective and poorly tolerated whereas benzodiazepines may be moderately helpful. Local injections of botulinum toxin help to reduce pain and limit the amplitude of some movements (violent neck movements with high risk of symptomatic radiculomyelopathy). In a rare subtype of dystonia-choreathetosis CP with little spasticity and MRI lesions, bilateral pallidal stimulation (GPi) has shown mild to moderate improvement of dystonia (in open-label small series and in one controlled study) with no cognitive or mood adverse effects. Optimal placement of the leads was a major (but not exclusive) factor for good outcome but results cannot be predicted on an individual basis and larger studies are needed.


Clinical Approach To Pediatric Neurology

Author by : Jaya Shankar Kaushik
Languange : en
Publisher by : Jaypee Brothers Medical Publishers
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Description : This book is a practical guide to the diagnosis and treatment of paediatric neurological disorders for trainee and practising paediatricians. Divided into four sections, the text begins with discussion on neurological evaluation including anatomy and imaging. The second section covers diagnostic techniques for different neurological disorders including seizures, neuromuscular weakness, autism and ADHD, and movement disorders. Section three presents a selection of clinical cases similar to those candidates may encounter in postgraduate examinations. The final section discusses therapeutic methods for a variety of neurological disorders. The comprehensive text is further enhanced by clinical photographs, tables, and flowcharts. Key points Comprehensive guide to diagnosis and management of paediatric neurological disorders Covers numerous conditions including seizures, movement disorders, and autism and ADHD Provides a selection of clinical cases for trainees preparing for examinations Highly illustrated with photographs, tables, and flowcharts


Pediatric Neurology Part I

Author by : Brian G.R. Neville
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Pediatric neurology comprises a very large of number of conditions exhibiting symptoms and signs in several functional domains arising from damage and dysfunction to the developing nervous system. The diagnostic process involves ensuring that data from all possible domains are sought including those that are unaffected. The subsequent analysis involves fitting these data into patterns of classical natural history and rigorous investigation of the aspects that do not appear to fit. There may be a pattern of illness that is immediately recognized or something that is a fairly close fit. However, the aim is to develop a pathogenic sequence for the condition particularly so that conditions that have been lumped together for convenience are separated into distinct disease entities. The major presentations of pediatric neurology of fixed central motor impairments (the cerebral palsies), the epilepsies, and the progressive degenerative diseases are in the process of being split into such pathogenic sequences so that definitive treatments and possible primary prevention can be added to aims of simple diagnostic recognition. Much of this is at an early stage and pediatric neurology is still a young and fast developing specialty.


Pediatric Neurology Part I

Author by : Harvey B. Sarnat
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : 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.


Pediatric Neurology Part I

Author by : Renzo Guerrini
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Epileptic myoclonus can be defined as an elementary electroclinical manifestation of epilepsy involving descending neurons, whose spatial (spread) or temporal (self-sustained repetition) amplification can trigger overt epileptic activity and can be classified as cortical (positive and negative), secondarily generalized, thalamo-cortical, and reticular. Cortical epileptic myoclonus represents a fragment of partial or symptomatic generalized epilepsy; thalamo-cortical epileptic myoclonus is a fragment of idiopathic generalized epilepsy. Reflex reticular myoclonus represents the clinical counterpart of fragments of hypersynchronous epileptic activity of neurons in the brainstem reticular formation. Epileptic myoclonus, in the setting of an epilepsy syndrome, can be only one component of a seizure, the only seizure manifestations, one of the multiple seizure types or a more stable condition that is manifested in a nonparoxysmal fashion and mimics a movement disorder. This complex correlation is more obvious in patients with epilepsia partialis continua in which cortical myoclonus and overt focal motor seizures usually start in the same somatic (and cortical) region. In patients with cortical tremor this correlation is less obvious and requires neurophysiological studies to be demonstrated.


Pediatric Neurology Part I

Author by : André Palmini
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Malformations of cortical development (MCD) are now well established as a most prevalent and relevant etiology of medically refractory epilepsies in children and adolescents. Focal cortical dysplasia (FCD) and hemimegalencephalies (HMG) occupy a special status because their focality (or in the case of HMG, their unihemispheric distibution) makes them amenable to surgical treatment to attempt seizure control. Since interictal epileptiform discharges and frequent seizures can lead to abnormal development because of brain plasticity during early childhood, the aim of surgical treatment is not only seizure control but also the redirection of development toward more physiological paths. In this review, we propose an “imaging-semiological organization” including (1) patients whose dysplastic lesion surrounds the fronto-rolandic cortex with increased signal and a transmantle sign, (2) multilobar hemispheric lesions, predominating in the anterior or posterior quadrants with large areas of abnormal gyration, increased cortical thickness, and gray–white blurring, (3) anterior temporal dysplasias usually featuring volume reduction combined with blurring of the underlying white matter in the temporal pole, and (4) a very relevant group of patients with refractory seizures, normal or roughly normal intellect, and normal MRI, later shown to harbor microscopic “nidus” of dysplastic cells. Classification takes into account the cortical disorganization, the presence of aberrant cellular elements, and the association with other lesion types.


Pediatric Neurology Part I

Author by : Georg Dorfmüller
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Whereas there is no specific neurosurgical technique in pediatric epilepsy, the frequency of each type of surgery is very different from epilepsy surgery applied in adults, and reflects the underlying etiologies, which are much more diverse in children, with malformations of cortical development and tumors as the prevailing etiologies. Extensive resective or disconnective procedures for extratemporal epilepsy are more frequently performed in infants and younger children, whereas temporo-mesial resection is by far the most common surgical treatment for adults with epilepsy. More recently, less invasive techniques in children with an extensive epileptogenic zone, such as multilobar disconnection, hemispherotomy and other functional hemispherectomy variants, have been introduced in order to reduce duration of surgery, perioperative morbidity and length of hospital stay. Likewise, minimally invasive techniques are utilized, such as the endoscopic disconnection of hypothalamic hamartomas for gelastic epilepsy. This development has been encouraged with the introduction of image-guided navigation systems for the preoperative planning and during surgery. Historically, epilepsy surgery for children has been established much later than for adults. Apart from the particular aspects in perioperative management of younger infants, surgery-related morbidity as well as seizure outcome is in general similar to those in adults, depending rather on each type of surgery.


Handbook Of Pediatric Neurology

Author by : Katherine Sims
Languange : en
Publisher by : Lippincott Williams & Wilkins
Format Available : PDF, ePub, Mobi
Total Read : 55
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Description : Lippincott Williams & Wilkins is proud to introduce the Handbook of Pediatric Neurology. Pediatric neurology, an entirely separate specialty from adult neurology is heavily weighted toward the study of metabolic and genetic disorders. This pocket book, based on the critically acclaimed Sabatines Pocket Medicine, and created as a spinoff of the classic Greers Pocket Neurology, will be of primary interest for neurologists and pediatricians during their peds neuro rotation. Handbook of Pediatric Neurology provides a practical, comprehensive guide to the management of hospital- and clinic-based pediatric neurological workup, diagnosis, and management. There is crossover into the adult neurology arena due to the science contained within pediatric neurology. As advances in the science of metabolic/genetic disorders occur, adult neurologists are becoming aware of their need to know.The editors believe that topics addressed in Pocket Pediatric Neurology have broad and important interest beyond pediatric neurologists, including pediatricians, adult neurologists and internal medicine physicians.


Pediatric Neurology Part I

Author by : David W. Dunn
Languange : en
Publisher by : Elsevier Inc. Chapters
Format Available : PDF, ePub, Mobi
Total Read : 23
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Description : Primary attention-deficit/hyperactivity disorder (ADHD) is a familial neurodevelopmental disorder involving the frontostriatal pathways and probably connections with the cerebellum and parietal lobe. ADHD is a polygenetic disorder involving predominantly catecholaminergic receptors and transporters. Though more common in school age children, ADHD may persist into adolescence and adulthood. Comorbidity with other disruptive behavior disorders, anxiety disorders, mood disorders, and learning disability is common. Secondary problems with attention are associated with neurological disorders that involve damage to the frontal lobes, drugs and toxins, and some genetic syndromes. Questionnaires are helpful in the diagnosis and follow-up of ADHD, and neuropsychological assessment can be useful for assessing cognitive function. Behavioral therapies are usually combined with medication for treatment of ADHD. Problems with attention are more responsive to treatment with stimulants, but nonstimulant medications such as atomoxetine, and possibly other drugs, are also effective and may be particularly useful in children and adolescents with comorbidity. The response to pharmacotherapy is better in patients with primary ADHD compared to those with secondary problems with attention.


Pediatric Neurology Part I

Author by : I. Jambaqué
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Childhood epilepsy may cause cognitive disorders and the intellectual quotient is indeed not normally distributed in epileptic children, a fair proportion of whom show an IQ in the deficient range. Some epileptic syndromes happen during vulnerability periods of brain maturation and interfere with the development of specific cognitive functions. This is the case for the Landau–Kleffner syndrome, which generally appears during speech development and affects language. Similarly, West syndrome – or infantile spasms – is an epileptogenic encephalopathy appearing during the first years of life and induces a major delay in social and oculo-motor development. Specific impairments can also be identified in partial childhood epilepsies in relation with seizure focus localization. For instance, left temporal and frontal epilepsies are frequently associated with verbal impairments. Moreover, episodic memory disorders have been described in children suffering from temporal lobe epilepsy whereas executive deficits (planning, self-control, problem solving) have been reported in frontal lobe epilepsy. In most cases, including its mildest forms, childhood epilepsy induces attention deficits, which may affect academic achievement. These observations militate in favor of individual neuropsychological assessments as well as early interventions in order to provide the child with an optimal individualized treatment program.


Pediatric Neurology Part I

Author by : Nadia Bahi-buisson
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Epilepsies associated with inborn errors of metabolism (IEM) represent a major challenge. Seizures rarely dominate the clinical presentation, which is more frequently associated with other neurological symptoms, such as hypotonia and/or cognitive disturbances. Although epilepsy in IEM can be classified in various ways according to pathogenesis, age of onset, or electroclinical presentation, the most pragmatic approach is determined by whether they are accessible to specific treatment or not. The main potentially treatable causes comprise vitamin B6 (pyridoxine deficiency), biotine, and GLUT1 deficiency (GLUT1DS) syndromes. Folinic acid-dependent seizures are allelic with pyridoxine dependency. Incompletely treatable IEMs include pyridoxal phosphate, serine, and creatine deficiencies. The main IEMs that present with epilepsy but offer no specific treatment are nonketotic hyperglycinemia, mitochondrial disorders, sulfite oxidase deficiency, ceroid-lipofuscinosis, Menkes disease, and peroxisomal disorders.


Pediatric Neurology Part I

Author by : Laurent Mottron
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : The autistic spectrum currently encompasses common precocious behaviourally identified constellations of social and communication atypicalities associated with restricted interests and repetitive behavior, together with uneven ability profiles. It is associated with multiple but heterogeneous genetic, functional, and structural variations whose established links with an autistic behavioral phenotype are as yet minimal. Strong evidence of high heritability contrasts with limited determination of genes and modes of transmission involved. Adaptation and outcomes vary widely according to opportunities, accommodation, and co-occurring conditions. With current diagnostic practices, multiple genetic conditions overlap with the autistic spectrum, with potential for confusion arising from phenocopies. Recent advances question the often presumed association between autism and intellectual disability and/or epilepsy. Autism is currently understood as a final common phenotypical pathway resulting from an indefinite number of genetic variations, possibly involving the same information processing pathways, and producing a variant in the way humans perceive, memorize, manipulate, and attribute emotional value to available information. Findings plausibly converge on more optional, rather than typically mandatory, hierarchies of information processing as fundamental to autism. Adaptation of education and employment according to individual strengths and needs, as well as attention to co-occurring conditions as necessary, remains today the best way to assist autistic individuals.


Pediatric Neurology Part I

Author by : Dominique M. Ijff
Languange : en
Publisher by : Elsevier Inc. Chapters
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Description : Although the causes of cognitive impairment in patients with epilepsy have not been completely elucidated, three factors are clearly involved: the underlying etiology of epilepsy, the effects of seizures or the epileptiform EEG discharges themselves, and the central nervous system effects of antiepileptic drugs (AEDs). All commonly used AEDs have some effect on cognitive function, and the effect may be substantial when crucial functions are involved, such as learning in children. With phenobarbital, there is a high risk for serious cognitive effects impacting attention and memory. Phenytoin may affect mental speed, mainly in higher dosing and polytherapy. Moderate monotherapy doses do not seem to induce much effect. Valproate does not seem to impair cognition if sufficiently controlled for hyperammonemia. For carbamazepine, there are conflicting reports, which may be due to selection bias or dosing. For oxcarbazepine, there is no evidence for any detrimental change compared to valproate but mild improvements on attentional tests. For topiramate, there is clear evidence for topiramate-induced cognitive impairment (attention, memory, and language function) in adults and children. Although data is sketchy, levetiracetam does not seem to have a negative impact on cognition. For lamotrigine, there is evidence of a cognitive-enhancing effect on attention. No evidence for cognitive side-effects has been found for vigabatrin. Ethosuximide is not associated with cognitive impairment although the evidence is sketchy. For gabapentin, tiagabine, zonisamide, and rufinamide no studies in children are available.


Pediatric Neurology Part I

Author by : Friedrich G. Woermann
Languange : en
Publisher by : Elsevier Inc. Chapters
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Total Read : 38
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Description : Malformations of cortical development, especially focal cortical dysplasia in infants and children, and hippocampal sclerosis in adolescents with epilepsy are frequent lesions, but they are overlooked on standard MRI. In infants, errors in the interpretation of MRI in epilepsy can be attributed to MRI signal changes due to ongoing myelination. Poor technique, perceptual misses, incomplete knowledge and poor judgment are, however, other likely sources of errors when reading MRIs. This review covers MRI search strategies, i.e., how to conduct MRI examinations in epilepsy and what to expect in the structural MRI of an infant or child with focal epilepsy. Exploiting increased sensitivity, false positive results can be avoided in the light of a clinical hypothesis, possibly isolating a localized brain area by seizure semiology, EEG, and sometimes PET prior to MR reading.