Journal of child neurology
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Review Case Reports
Extrapontine myelinolysis with involvement of the hippocampus in three children with severe hypernatremia.
Central pontine myelinolysis is a disorder of unknown etiology linked to overly aggressive correction of hyponatremia. In addition to the typical location of demyelination with preservation of neurons and axon cylinders in the basis pontis, similar lesions have been described in extrapontine locations. Central pontine myelinolysis and extrapontine myelinolysis usually occur together, and are identified at autopsy rather than in life because symptoms of extrapontine myelinolysis are often masked in the critically ill patient. ⋯ Peak sodium values in each child were 195, 168, and 177 mmol/L, respectively; each received aggressive treatment for hypernatremia. We believe this to be the first report of extrapontine myelinolysis in children, the first report of extrapontine myelinolysis without central pontine myelinolysis in children, and the first report in children of hippocampal formation involvement. The pathogenesis of the central and extrapontine myelinolysis complex in children is more complicated than previously believed, and might differ significantly from that of adults.
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Status epilepticus is more common among children than young adults. Children might be less likely to die and might be resistant to permanent neurologic damage due to status epilepticus, but significant sequelae also have been demonstrated. Aggressive intervention and rapid termination of seizures contribute significantly to better prognosis and reduced mortality from status epilepticus. ⋯ Traditionally, refractory status epilepticus is treated with barbiturate coma or general anesthetics, both of which require invasive cardiorespiratory and hemodynamic monitoring and are associated with significant complications. Midazolam is a water-soluble benzodiazepine with a fast onset of action, a short half-life, and inactive metabolites that has been very effective in terminating seizures refractory to diazepam, lorazepam, phenytoin, and phenobarbital in pediatric patients. Midazolam is a valuable treatment option for refractory status epilepticus, especially in pediatric patients.
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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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Trauma in the United States is the leading cause of death and disability in the pediatric population. Differences of age and development affect recovery and outcome following head injury. ⋯ Treatment and management should be tailored to each case in order to effect a positive outcome with respect to brain functioning. Aggressive intervention for prevention of primary and secondary injury must be continued and understanding of the impact of these injuries should provide for a brighter future for these patients.