Long-Term Outcomes of New Onset Super Refractory Status Epilepticus

Findings from a case series suggest that the majority of patients with new-onset refractory status epilepticus (NORSE) had the more severe new-onset super refractory status epilepticus (NOSRSE); however, most of these patients who survived to hospital discharge had a good or fair outcome over a median 9-month follow-up period, according to study results published in Neurology.

Study researchers sought to better understand patients with NORSE, specifically the most severe presentation of it, NOSRSE. In this study, they retrospectively reviewed medical charts of 252 patients admitted to the Columbia University Irving Medical Center Neurological Intensive Care Unit for refractory status epilepticus (RSE). Patients were admitted between February 2009 and February 2016. Electronic medical record data provided information on patient demographics, comorbidities, diagnostic results, and treatments. The primary outcome included the modified Rankin Score (mRS) at time of hospital discharge. Study researchers defined a good or fair outcome as an mRS of 0 to 3, and a poor outcome as an mRS of 4 to 6.

A total of 27 patients with RSE met the criteria for NORSE, and of these, 26 patients met criteria for NOSRSE. Underlying autoimmune disorders represented the most common comorbidity, having been observed in 23 percent of patients. Most patients (42%) had no pre-existing conditions. An infectious and/or psychiatric prodrome was reported in 96 percent of patients prior to seizure onset. Despite extensive work-up, up to 73 percent (n=19) of patients had cryptogenic etiology. Additional etiologies included autoimmune in 19 percent and infectious in 8 percent of patients. 

7 (27%) patients underwent brain biopsy, autopsy, or both. 3 (12%) patients had diagnostic neuropathology, including 1 with herpes simplex virus encephalitis, 1 with candida encephalitis, and 1 with hemorrhagic necrotizing leukoencephalopathy.

The majority of patients (88%) received broad-spectrum antibiotics for meningoencephalitis, but none of these patients were treated with a full course. A total of 21 patients (81%) received immunotherapy.

For patients with NOSRSE, the median duration of the disease was 17 days. A total of 20 patients survived to hospital discharge, and long-term outcomes data were available for 17 of these patients. A good or fair outcome was reported in 23% (n=6) of patients on discharge. Among those with long-term follow-up data (median, 9 months), 71 percent (n=12) of patients had a good or fair outcome.

Limitations of this study included its small sample size, retrospective nature, the inclusion of patients from a single center, and variability in diagnostic work-up.

Due to these limitations, the study researchers wrote that “a prospective, multi-center study of NOSRSE patients is needed to help build on these findings and better understand this particularly fulminant syndrome.”

Reference

Matthews E, Alkhachroum A, Massad N, et al. New-onset SUPER refractory status epilepticus: A case series of 26 patients. Neurology. Published online September 17, 2020. doi:10.1212/WNL.0000000000010787