• James J Gugger, Khalil Husari, John C Probasco, and Mackenzie C Cervenka.
• Johns Hopkins Hospital, Johns Hopkins University School of Medicine, Department of Neurology, 600 N. Wolfe St, Baltimore, MD, 21287-7247, USA. Electronic address: james.gugger@uphs.upenn.edu.
• Seizure. 2020 Jan 1; 74: 41-48.

PurposeTo describe the clinical, laboratory, neuroimaging, electroencephalographic features, etiology, treatment, as well as short-term and long-term outcomes of adults with new-onset refractory status epilepticus (NORSE).MethodA retrospective, single institution cohort study (2010-2018) of consecutive adult patients with NORSE.ResultsAmong 20 patients with NORSE, nine (45 %) had prodromal febrile illness, 12 (60 %) had evidence of inflammation on CSF profile. Six patients (30 %) met criteria for definite autoimmune encephalitis (AE) while 8 patients (40 %) had probable AE. Eleven out of 13 (85 %) patients had an abnormal FDG-PET scan with the most common finding being regional hypermetabolism. Fourteen patients (70 %) received immunotherapy and ten (50 %) received the ketogenic diet (KD). Fifteen patients (75 %) progressed to super-refractory status epilepticus (SRSE) and seven patients (35 %) died in the hospital or within six months of discharge. Among the surviving patients, eight (40 %) had a good outcome (i.e., modified Rankin Scale score 0-2); 12 (80 %) received a diagnosis of epilepsy of which nine (75 %) developed drug-resistant epilepsy.ConclusionsNew-onset refractory status epilepticus is a syndrome associated with multiple complications, high mortality, and subsequent intractable epilepsy. There are multiple causes, some of which are autoimmune encephalitides; however, in this series the majority of patients had no clear etiology identified after extensive evaluation. Prospective studies are needed to determine optimal evaluation and treatment.Copyright © 2019 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

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