New-onset refractory status epilepticus: A retrospective cohort study

Seizure. 2020 Jan;74:41-48. doi: 10.1016/j.seizure.2019.12.002. Epub 2019 Dec 3.


Purpose: To 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).

Method: A retrospective, single institution cohort study (2010-2018) of consecutive adult patients with NORSE.

Results: Among 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.

Conclusions: New-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.

Keywords: Autoimmune encephalitis; FDG-PET; Ketogenic diet; New-onset refractory status epilepticus; Status epilepticus.

MeSH terms

  • Adult
  • Aged
  • Cohort Studies
  • Drug Resistant Epilepsy / diagnostic imaging*
  • Drug Resistant Epilepsy / metabolism*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Positron-Emission Tomography
  • Retrospective Studies
  • Status Epilepticus / diagnostic imaging*
  • Status Epilepticus / metabolism*