Pediatric neurology
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Pediatric neurology · Oct 2011
Lovastatin as treatment for neurocognitive deficits in neurofibromatosis type 1: phase I study.
In a neurofibromatosis type 1 murine model, treatment with lovastatin reversed cognitive disabilities. We report on a phase I study examining the safety and tolerability of lovastatin in children with neurofibromatosis type 1. Twenty-four children with neurofibromatosis type 1 underwent a dose-escalation protocol for 3 months to identify the maximum tolerated dose and potential toxicity. ⋯ Additional analyses, using reliable change indices, indicated improvements exceeding those of test-retest or practice effects in some participants. These observations may be analogous to the improvements observed in a neurofibromatosis type 1 murine model treated with lovastatin, although further study and replication are required. The safety and preliminary cognitive results support the need for a larger phase II trial in this population.
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Ictal asystole is a cardiac phenomenon associated with epileptic seizures, and may play a role in sudden unexplained death in epilepsy. We present a 17-year-old boy with chronic intractable epilepsy and a vagus nerve stimulator who developed ictal asystole many years after the onset of epilepsy. ⋯ A cardiac pacemaker and vagus nerve stimulator can be safely used simultaneously after careful testing during placement. The onset of asystolic events many years after the onset of epilepsy suggests that repeated seizures may exert long-term effects on cardiac function.