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De novo cryptogenic refractory multifocal febrile status epilepticus in the young adult: a review of six cases.

BACKGROUND
Status epilepticus (SE) is a common neurological emergency. In around 10 percent, the etiology of SE is not clear, i.e. cryptogenic SE.


AIM
To describe six young adult patients with de novo cryptogenic multifocal febrile SE with poor outcome.


METHODS
Retrospective chart review.


RESULTS
We describe six patients who presented with de novo SE. All were young adults with an unremarkable previous medical history. In all except one, the family history for epilepsy was negative. All had a febrile illness in the week preceding SE. Investigations were unable to reveal the underlying etiology. SE proved refractory to conventional antiepileptic drug treatment (AED) and anesthesia was required to control seizures. Seizures during SE or ensuing epilepsy were multifocal. Brain pathology in four patients was normal in two and showed changes secondary to seizures in two. Outcome was poor in all cases: one patient died during the course of SE, another patient remained with severe mental and physical disability as well as refractory epileptic seizures, the other four developed refractory multifocal epilepsy. One of these patients died a sudden unexpected death in epilepsy (SUDEP) and one drowned during a seizure.


CONCLUSIONS
Six patients were reviewed who presented with de novo cryptogenic multifocal refractory febrile SE. The possible etiologies are discussed. Based on the similarities in these cases, a distinct refractory SE syndrome might be assumed. Further prospective studies will, however, be required to validate this hypothesis as well as to elucidate its etiology and optimize treatment.