Abstracts

Rationale: Refractory status epilepticus (RSE) is a life-threatening condition, which is characterized by failure to respond to first and second line anticonvulsant therapy. Methods: This is a descriptive, retrospective review of 7 previously normal adult patients (age between 28 and 45 years) with NORSE admitted to Hamad Medical Corporation MICU in Qatar from 2012 to 2015. Results: Seven patients with NORSE syndrome were identified, where a cause was not established despite an exhaustive search with an average duration of 27 days (range 14-32). Characterizing features were male gender, young age, previous good health, cerebrospinal fluid pleocytosis (in 4), antecedent febrile illness (in 5), extraordinarily prolonged status epilepticus, failure of extensive investigations to reveal an underlying cause, catastrophic outcome as well as temporal lobe(in 3) and leptomeningeal enhancement (in 1) on brain magnetic resonance imaging. Treatment modalities included at least 3antiepileptic drugs (in all patients), anesthetics (in all patients), intravenous immunoglobulin (IVIG, 5 patients), steroids (4 patients), plasmopheresis (1 patient). Three patients improved completely without neurological deficit and two of them survived with severe disability. Two patients who were not given early immunotherapy, died from complications associated with prolonged ICU stay. None of the survivors received long term immunotherapy. Conclusions: The cause of NORSE syndrome may often be difficult to find. NORSE carries a poor prognosis and early recognition and treatment may improve outcomes. In this case series, we have shown that immunotherapy seems to be a helpful treatment option when conventional therapy fails. Funding: none