Refractory status epilepticus after non-specific infection in 3 previously healthy children

Objectives: In rare cases refractory status epilepticus (SE) occurs after non-specific infections in previously healthy children and adults.

Materials and Methods: We report 3 normally developed children with prolonged SE of unknown etiology. 2 girls, 1 boy, age at SE 3,6 and 9 years. With the exception of a febrile seizure in one child 3 years before SE, family history and development were normal in all patients.

Results: The clinical course was identical in all patients: all 3 developed a refractory SE three days after an unspecific infection. Patients were initially at least partially responsive.

Therapy: DZP → PHT→ midazolam→ thiopental. All patients became ventilator-dependent for some time. SE lasted weeks and months; subsequent trials with the whole spectrum of antiepileptic drugs were without sufficient effect; there was an aggravation with CBZ; Chloralhydrate and high-dose of phenobarbital had the best effect. The patients were admitted to our clinic for rehabilitation-purposes in persistent vegetative state, severe muscular hypotonia and still having countless seizures.

Diagnostics: Tests for possible infectiological, immunological or metabolic reasons were negative. MRIs were initially normal and showed progressive cortical atrophy from 2 months on. Brain-biopsies in 2 patients revealed non-specific gliosis, but no signs of inflammation.

Conclusion: Most patients with this tragic course will finally have the diagnosis “residual status after SE due to an unspecific viral encephalitis“. There is considerable doubt that this is true. In particular, the results of brain-biopsies in 2 of our cases suggest that the SE itself is most likely responsible for the severe brain damage. A genetic predisposition to such dramatic evolutions might be possible. Because state of the art treatment of SE is not able to avoid such developments and because of some peculiarities in the history of these cases a different management during the initial course could be considered; e.g. avoidance of AEDs potentially aggravating seizures. Prospective multi-centre studies are needed.