Journal of child neurology
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Fosphenytoin, a prodrug of phenytoin, is rapidly and completely converted to phenytoin in adults after intravenous or intramuscular administration and is significantly better tolerated than parenteral phenytoin. Fosphenytoin is highly plasma-protein bound and, when present in sufficient concentration, will displace phenytoin from plasma proteins. The clinical utility is that fosphenytoin may be used to achieve therapeutic phenytoin concentrations more rapidly than intravenous phenytoin infused at its maximum recommended rate. ⋯ Adverse events associated with fosphenytoin generally were related to the central nervous system and were similar to those associated with phenytoin, except for a higher incidence of transient pruritus with fosphenytoin. Intravenous fosphenytoin has significant advantages over intravenous phenytoin: It requires a shorter infusion time and fewer intravenous disruptions, causes less pain and burning at the infusion site and minimal consequences in case of intravenous infiltration, allows longer maintenance of intravenous sites, and has better intravenous fluid compatibility and stability. In contrast to intramuscular phenytoin, intramuscular fosphenytoin is well tolerated in both large loading doses and maintenance doses.
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The pharmacologic interventions for treatment of acute repetitive seizures and those for treatment of status epilepticus are similar. The choice of treatment should be based on the drug's onset of action, spectrum of anticonvulsant activity, route and ease of administration, elimination half-life, therapeutic margin of safety, and redistribution from the central nervous system. ⋯ Short-acting benzodiazepines, including diazepam, lorazepam, clonazepam, and midazolam, can decrease the frequency of emergency department visits if given at the appropriate times. The recently approved intravenous formulation of valproate may be of use in children receiving oral valproate who develop breakthrough seizures caused by subtherapeutic plasma levels that are secondary to missed doses or an inability to tolerate oral valproate.
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Generalized tonic-clonic seizure activity in infants and children frequently leads to an emergency department visit, often after emergency medical service personnel, such as paramedics, provide initial evaluation and treatment. Important subsets of patients who present to the emergency department include those with non-seizure-mediated movements, those with nongeneralized seizure activity, those with complications of anticonvulsant therapy, and those with status epilepticus. ⋯ Of the children with seizures who are seen in the emergency department, those with febrile convulsions or exacerbations of underlying seizure disorders predominate, while those with new-onset epilepsy or other seizure disorders account for a smaller proportion. Current issues in the emergency department management of seizures in children include: (1) modifying interventions to stabilize patients and simultaneously minimize the physiologic deterioration accompanying generalized seizures; (2) selection, initiation, administration, and refinement of anticonvulsant therapy; (3) minimizing complications of prolonged seizures and their treatment; (4) rapid recognition and treatment of life-threatening illnesses that underlie seizure presentations; (5) selection of appropriate diagnostic measures; and (6) use of electroencephalography in selected patients.
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A questionnaire was circulated to 1126 members of the Child Neurology Society to assess their opinions regarding the current medical-legal system and to determine the involvement of members of the Child Neurology Society as expert witnesses. Two-hundred eighty-five questionnaires were completed, corresponding to a response rate of 25.3%. Of the respondents, 52% were child neurologists in university-based, academic practices, whereas 32% were in private practice. ⋯ Two thirds of the respondents indicated that there should be a mechanism for peer review and more than half believed that the Child Neurology Society should monitor the expert witness activities of its members. These results indicate that although child neurologists frequently participate as medical experts, the respondents have an unfavorable opinion of the current medical liability system. Based on the results of the questionnaire and the need for reform in the current system, monitoring of the expert witness activities of child neurologists should be considered.
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Rett syndrome (RTT) has been described in its classic form only in females. Although the majority of cases are sporadic, familial cases give valuable insight into the genetic basis and phenotypic variability of the disorder. ⋯ We identified two boys in families with recurrent Rett syndrome who had encephalopathies with neonatal onset and who may represent the phenotype of males harboring Rett syndrome mutations. The difference in severity of disease in these males and their female relatives supports the location of Rett syndrome locus on the X-chromosome.