Pediatric Neurology Part I

Pediatric Neurology Part I

Author: Charlotte Dravet

Publisher: Elsevier Inc. Chapters

ISBN: 9780128077702

Category: Medical

Page: 892

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Severe myoclonic epilepsy in infancy (SMEI) is a rare disease, characterized by febrile and afebrile, generalized and unilateral, clonic or tonic–clonic seizures that occur in the first year of life in an otherwise apparently normal infant. They are later associated with myoclonus, atypical absences, and partial seizures. Developmental delay becomes apparent within the second year of life and is followed by definite cognitive impairment and personality disorders of variable intensity. In the borderline form, children do not present with myoclonic symptoms but have the same general picture. SMEI is a channelopathy and the genetic studies have shown a mutation in the SCN1A gene in 70 to 80% of the patients, including the borderline forms. At present, there are no well-established correlations between genotype and phenotype. The electroencephalograms, often normal at the onset, display both generalized and focal anomalies, without a specific electroencephalographic pattern. As a rule, neuroimaging is normal. All seizure types are resistant to antiepileptic drugs and status epilepticus is frequent. Some drugs have been shown to aggravate the seizures and must be avoided. Two recent drugs have been proved to partially control the convulsive seizures and the status epilepticus. Therefore, it is crucial to diagnose this epilepsy soon after its onset in order to prescribe the most appropriate treatment.
Epileptic Seizures and Syndromes
Language: en
Pages: 698
Authors: Peter Wolf
Categories: Medical
Type: BOOK - Published: 1994 - Publisher: John Libbey Eurotext

This book reflects debates and results which have developed since the introduction of an international system of classifications in epileptology. The creation of such a system was initiated, in the sixties, mainly for practical reasons: growing international exchange had revealed that divergence of terminology in epilepsy had become important enough
Epilepsy Syndromes E-Book
Language: en
Pages: 232
Authors: Mary Ann Werz, Ignacio L. Pita
Categories: Epilepsy
Type: BOOK - Published: 2017-07-12 - Publisher: Saunders

Drs. Mary Ann Werz and Ignacio L. Pita present Epilepsy Syndromes, a unique book with companion DVD that guides you through the difficult diagnostic challenges associated with this disorder. It is organized by epilepsy syndromes for easy reference, and the DVD shows patient videos running concurrently with their EEG tracings.
Pediatric Neurology Part I
Language: en
Pages: 892
Authors: Charlotte Dravet, Hirokazu Oguni
Categories: Medical
Type: BOOK - Published: 2013-04-24 - Publisher: Elsevier Inc. Chapters

Severe myoclonic epilepsy in infancy (SMEI) is a rare disease, characterized by febrile and afebrile, generalized and unilateral, clonic or tonic–clonic seizures that occur in the first year of life in an otherwise apparently normal infant. They are later associated with myoclonus, atypical absences, and partial seizures. Developmental delay becomes
A Clinical Guide to Epileptic Syndromes and their Treatment
Language: en
Pages: 578
Authors: C. P. Panayiotopoulos
Categories: Medical
Type: BOOK - Published: 2010-05-10 - Publisher: Springer Science & Business Media

Affecting 4 percent of children and 1-2 percent of the general population, epilepsy is one of the most common neurological disorders. The 1st edition of this guide proved to be the only one of its kind, covering many important aspects of diagnosis and treatment. Due to the continued advances being
The Epilepsies
Language: en
Pages: 541
Authors: Chrysostomos P. Panayiotopoulos
Categories: Medical
Type: BOOK - Published: 2005 - Publisher: Bladon Medical Pub

The Epilepsies: Seizures, Syndromes and Management is the latest work from one of the world's leading experts and offers an exhaustive account of the classification and management of epileptic disorders. In thirteen chapters, Dr Panayiotopoulos gives clear and didactic guidance on the diagnosis, treatment and ongoing management of the full