2023
Cairn
Katherine Werbaneth et al., « A retrospective cohort study of new-onset refractory status epilepticus (NORSE): clinical features, timing of immunotherapy and outcomes », Epileptic Disorders, ID : 10670/1.p1zmgk
Objective To describe clinical characteristics associated with immunotherapy in patients with new-onset refractory status epilepticus (NORSE) and assess its timing and effect on outcomes at hospital discharge after six and 12 months of follow-up. Our secondary aim was to apply the cryptogenic NORSE (C-NORSE) score to subjects in order to evaluate its utility in identifying C-NORSE in our cohort. Methods This was a retrospective single university hospital cohort study (2004-2021) of adults and children with NORSE. First-line immunotherapy was defined as corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis (PLEX). Early immunotherapy was defined as administration of a first-line agent within seven days of presentation. Results Twenty-one subjects with NORSE were identified between 2004 and 2021, which was cryptogenic in 18 and immune-mediated in three. All patients received immunotherapy. Seventeen patients received early immunotherapy (81%). There was no significant difference between early versus late immunotherapy regarding “good or favorable” outcomes (mRS 0-2) at hospital discharge or during follow-up. For cryptogenic NORSE patients, 7/11 (64%) achieved good outcomes at six months, 9/11 (82%) at 12 months, and 8/10 (80%) at the last follow-up visit at >13 months. For immune-mediated NORSE patients, 3/3 (100%) achieved good outcomes at six months and 2/2 (100%) at the last follow-up visit at >13 months. In our cohort, a C-NORSE score of ≥5 was obtained in 12/18 (67%) of cryptogenic cases and a score