Abstract [eng] |
Febrile infection-related epilepsy syndrome is a very rare epilepsy with poor outcomes, which most often strikes previously healthy 4 - 9 year old children. The syndrome manifests with prolonged superrefractory status epilepticus after an unspecified febrile infection. The acute phase can last up to a few months, frequently without response to the treatment with antiepileptic drugs. Currently most often status epilepticus is suppressed only with anesthetics, therefore efforts are being made to elucidate the so far unknown etiology of the syndrome in the hope of applying effective treatment. Recent data show promising results with a ketogenic diet and anakinra, a recombinant human interleukin 1 receptor antagonist. The acute phase is followed by chronic, characterized by treatment-resistant epilepsy and neurological disorders without marked clinical improvement. This paper reviews the literature on this syndrome and presents a 4-year-old patient with a typical course of the syndrome, who had seizures resistant to antiepileptic drugs and underwent a corpus callosotomy (an incision of the corpus callosum) in the acute phase in order to control the seizures. Callosotomy has long been used as a palliative treatment for drug-resistant epilepsy, but its application to febrile infection-related epilepsy has not been described in the literature. |