Alternating hemiplegia of childhood (AHC) is a very rare disease characterized by recurrent attacks of loss of muscular tone resulting in hypomobility of one side of the body. The etiology of the disease due to ATP1A3 gene mutations in the majority of patients. Few familial cases have been described. AHC has an onset in the first few months of life. Hemiplegic episodes are often accompanied by other paroxysmal manifestations, such as lateral eyes and head deviation toward the hemiplegic side and a very peculiar monocular nystagmus. As the attack progresses, hemiplegia can shift to the other side of the body. Sometimes the attack can provoke bilateral paralysis, and these patients may have severe clinical impairment, with difficulty in swallowing and breathing. Hemiplegic attacks may be triggered by different stimuli, like bath in warm water, motor activity, or emotion. The frequency of attacks is high, usually several in a month or in a week. The duration is variable from a few minutes to several hours or even days. Sleep can stop the attack. Movement disorders such as dystonia and abnormal movements are frequent. Cognitive delay of variable degree is a common feature. Epilepsy has been reported in 50% of the cases, but seizure onset is usually during the third or fourth year of life. Many drugs have been used in AHC with very few results. Flunarizine has the most supportive anecdotal evidence regarding efficacy.
A reference book on diagnosis, consequences and management of neonatal and infantile seizures There is a very high incidence of seizures during the first two years of life. This may reflect multiple etiologies depending on the circumstances under which seizures occur. They may have a benign cause but for others they may lead to more devastating consequences. This book provides new insights on how it is best to approach seizures and epilepsy in the first two years of life, to systematically create a blueprint upon which diagnostic and treatment decisions can be based. Ongoing efforts are to understand: - How seizures may occur in the developing brain? - What are their consequences? - Which biomarkers are being developed? - What are the effective treatments to promptly stop ongoing seizures and alter the course of epileptic encephalopathies? The data are highly reflecting the state of the art and also individualize for the particular milieu of the patient in taking into account both nature (i.e. genetics), and nurture (i.e. events that may interfere with normal development) and result in seizures and epilepsy.
In this chapter we include a series of epilepsies with onset in pediatric age characterized by focal seizures, idiopathic etiology, normal psychomotor development, and a benign course related to the spontaneous remission of seizures without sequelae. These entities are age-dependent and seizures tend to disappear spontaneously. For these reasons often the drug treatment is not necessary. On the basis of genetic assessment idiopathic focal epilepsies can be divided into two groups: nonautosomal dominant and autosomal dominant. In the group of nonautosomal entities we include benign epilepsy with centro-temporal spikes, Panayiotopoulos syndrome, idiopathic childhood occipital epilepsy described by Gastaut, and benign idiopathic midline spikes epilepsy. Seizures are rare, sometimes prolonged, as autonomic status in Panayiotopoulos syndrome. A common feature is the presence of peculiar EEG interictal paroxysmal abnormalities. In the group with an autosomal dominant mode of inheritance we include benign familial infantile seizures and benign familial neonatal–infantile seizures. These entities are characterized by partial seizures in cluster, self-limited in a brief period during the first months of life. There are no typical interictal EEG abnormalities. In some families a mutation in SCN2A, the gene coding for the 2α subunit of the voltage-gated sodium channel, has been described.
A reference book on diagnosis, consequences and management of neonatal and infantile seizures There is a very high incidence of seizures during the first two years of life. This may reflect multiple etiologies depending on the circumstances under which seizures occur. They may have a benign cause but for others they may lead to more devastating consequences. This book provides new insights on how it is best to approach seizures and epilepsy in the first two years of life, to systematically create a blueprint upon which diagnostic and treatment decisions can be based. Ongoing efforts are to understand: - How seizures may occur in the developing brain? - What are their consequences? - Which biomarkers are being developed? - What are the effective treatments to promptly stop ongoing seizures and alter the course of epileptic encephalopathies? The data are highly reflecting the state of the art and also individualize for the particular milieu of the patient in taking into account both nature (i.e. genetics), and nurture (i.e. events that may interfere with normal development) and result in seizures and epilepsy.
In this chapter we include a series of epilepsies with onset in pediatric age characterized by focal seizures, idiopathic etiology, normal psychomotor development, and a benign course related to the spontaneous remission of seizures without sequelae. These entities are age-dependent and seizures tend to disappear spontaneously. For these reasons often the drug treatment is not necessary. On the basis of genetic assessment idiopathic focal epilepsies can be divided into two groups: nonautosomal dominant and autosomal dominant. In the group of nonautosomal entities we include benign epilepsy with centro-temporal spikes, Panayiotopoulos syndrome, idiopathic childhood occipital epilepsy described by Gastaut, and benign idiopathic midline spikes epilepsy. Seizures are rare, sometimes prolonged, as autonomic status in Panayiotopoulos syndrome. A common feature is the presence of peculiar EEG interictal paroxysmal abnormalities. In the group with an autosomal dominant mode of inheritance we include benign familial infantile seizures and benign familial neonatal–infantile seizures. These entities are characterized by partial seizures in cluster, self-limited in a brief period during the first months of life. There are no typical interictal EEG abnormalities. In some families a mutation in SCN2A, the gene coding for the 2α subunit of the voltage-gated sodium channel, has been described.
Alternating hemiplegia of childhood (AHC) is a very rare disease characterized by recurrent attacks of loss of muscular tone resulting in hypomobility of one side of the body. The etiology of the disease due to ATP1A3 gene mutations in the majority of patients. Few familial cases have been described. AHC has an onset in the first few months of life. Hemiplegic episodes are often accompanied by other paroxysmal manifestations, such as lateral eyes and head deviation toward the hemiplegic side and a very peculiar monocular nystagmus. As the attack progresses, hemiplegia can shift to the other side of the body. Sometimes the attack can provoke bilateral paralysis, and these patients may have severe clinical impairment, with difficulty in swallowing and breathing. Hemiplegic attacks may be triggered by different stimuli, like bath in warm water, motor activity, or emotion. The frequency of attacks is high, usually several in a month or in a week. The duration is variable from a few minutes to several hours or even days. Sleep can stop the attack. Movement disorders such as dystonia and abnormal movements are frequent. Cognitive delay of variable degree is a common feature. Epilepsy has been reported in 50% of the cases, but seizure onset is usually during the third or fourth year of life. Many drugs have been used in AHC with very few results. Flunarizine has the most supportive anecdotal evidence regarding efficacy.
This book introduces a general approach for schematization of mechanical systems with rigid and deformable bodies. It proposes a systems approach to reproduce the interaction of the mechanical system with different force fields such as those due to the action of fluids or contact forces between bodies, i.e., with forces dependent on the system states, introducing the concepts of the stability of motion. In the first part of the text mechanical systems with one or more degrees of freedom with large motion and subsequently perturbed in the neighborhood of the steady state position are analyzed. Both discrete and continuous systems (modal approach, finite elements) are analyzed. The second part is devoted to the study of mechanical systems subject to force fields, the rotor dynamics, techniques of experimental identification of the parameters and random excitations. The book will be especially valuable for students of engineering courses in Mechanical Systems, Aerospace, Automation and Energy but will also be useful for professionals. The book is made accessible to the widest possible audience by numerous, solved examples and diagrams that apply the principles to real engineering applications.
Federico Chabod (1901-1960) was one of Italy's best-known historians, noted for his study of Italian history in a European context. This is the first English translation of his most important book. Although he carried out his extensive archival research for this work from 1936 until 1943, the fall of fascism and Chabod's active participation in the Resistance delayed its completion. When it was published in 1951, it was immediately hailed as a masterpiece. Chabod intended to write a new kind of diplomatic history-- one in which political history is seen as part of a larger historical whole. He does not present a detailed chronological account of Italian foreign policy during the period studied, but rather the "moral and material" underpinnings of that policy. In fact, he crafts a highly developed portrait of an age, with the real subjects being the Italian state and society, the ruling class and political culture. This work offers readers a superb picture of post-Risorgimento Italy and an outstanding example of Chabod's historiographical method. Originally published in 1996. The Princeton Legacy Library uses the latest print-on-demand technology to again make available previously out-of-print books from the distinguished backlist of Princeton University Press. These editions preserve the original texts of these important books while presenting them in durable paperback and hardcover editions. The goal of the Princeton Legacy Library is to vastly increase access to the rich scholarly heritage found in the thousands of books published by Princeton University Press since its founding in 1905.
Acknowledged as the premier Italian guide, this revised handbook includes updated ratings of restaurants--more than 500--and hotels. It also features dozens of locations of related interest, including best places for ice cream, pastry, pizza, and coffee.
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