Abstracts

Rationale: Migrating partial seizures in infancy (MPSI) is a newly recognized, age-specific epileptic syndrome, first described in 1995. To date, 33 cases have been reported. Affected infants present in the first 6 months of age with nearly continuous multifocal partial seizures arising independently and sequentially from both hemispheres, and evolve into intractable seizure towards status epilepticus (SE) and subsequent neurological deterioration with loss of both cognitive and motor abilities and progressive decline of head circumference percentile. Etiology remains unknown. Conventional AEDs, steroids, ACTH, pyridoxine, biotin, folinic acid, and ketogenic diet have been proven ineffective. Zonisamide and topiramate have been tried without success. Vigabatrin and carbamazepine may worsen the seizures. Potassium bromide and levetiracetam appeared to be effective in reducing seizure frequency in two cases and one case respectively. We report the efficacy of intravenous levetiracetam (ivLEV) in stopping the refractory SE in two new patients diagnosed with MPSI. Methods: The first patient had seizure onset at 4 months of age and quickly progressed into SE with multifocal migrating seizures associated with focal motor signs and profound apnea, as well as generalized tonic-clonic seizures. He was admitted in the PICU at 8 months of age and treated with intravenous phenobarbital, phenytoin, valproate, clonazepam, diazepam, lorazepam, as well as oral carbamazepine, vigabatrin, zonisamide, topiramate, levetiracetam, potassium bromide, steroids, ACTH, pyridoxine, biotin, pyridoxal-5-phosphate, folinic acid, and ketogenic diet with no response. Thiopental was introduced to the point of burst-suppression on EEG. He persisted in a refractory SE for 10 months with recurrence of SE at every attempt of thiopental sodium withdrawal. The second child presented with multifocal motor seizures at one month, and showed a progressive increase in the frequency of seizures that became almost continuous. At 4 months of age she was admitted in the PICU because of SE and treated with intravenous thiopental sodium and midazolam, as well as levetiracetam and potassium bromide via NG tube. SE was resistant to phenytoin, carbamazepine, vigabatrin, diazepam, lorazepam, and pyridoxine. In both patients, comprehensive etiology workup was noncontributory. Intravenous Levetiracetam (IvLEV) was administered at the dose of 60 mg/kg/day. Results: SE resolved in both patients within 12 hours, thiopental was discontinued. No side effects such as arrhythmia, changes in blood count, liver enzymes and serum creatinine, or skin reaction were noted. IvLEV was maintained during several weeks and then switch to oral formulation at the same dose. Conclusions: Until the recent release of ivLEV, phenytoin, phenobarbital and valproic acid were the only parenteral compounds available to treat status epilepticus. All three were ineffective in our cases. We suggest that ivLEV may represent the first valid option for treating MPSI, an epileptic syndrome refractory to both conventional and newer AEDs.