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Factors associated with long-term outcomes in people with new onset refractory epilepticus: A single-center cohort
Wei Peng1,2, Lu Lu1,2, Jianqiong Yin1,2
1Department of Neurology, West China Hospital, Sichuan University, Chengdu, China.
Objective:
New onset refractory status epilepticus (NORSE) is a neurologic emergency in people without a history of active epilepsy or other preexisting neurological disorders. There are not many studies reporting on the long-term outcomes of NORSE. We investigate factors associated with outcomes such as mortality and sequelae in NORSE.
Methods:
This retrospective, single-center study involved individuals diagnosed with NORSE in West China hospital who were consecutively admitted between January 2018 and December 2022. The primary outcome was death at follow-up. Secondary outcomes included functional recovery and sequelae among survivors. Lasso-Cox regression was used to evaluate factors for death. A Cox proportional hazard regression model was then established, incorporating several parameters identified through Lasso-Cox regression.
Results:
One hundred one individuals with NORSE were identified and reviewed (median age = 30 years, 45 (45%) were female). The etiology was unknown in 37 (37%) people despite comprehensive diagnostic workups. Among the remaining 64 (64%), the most common etiology of NORSE was autoimmune encephalitis (n = 53, 83%), followed by infectious encephalitis (n = 9, 14%). One person died in-hospital, and 24 (24%) died during follow-up. Factors independently associated with death were the presence of superrefractory status epilepticus (hazard ratio [HR] = 3.99, 95% confidence interval [CI] = 1.09-14.61, p = .036) and initial response to immunotherapy (HR = .11, 95% CI = .02-.62, p = .012). The median follow-up duration among the 76 survivors was 40 months (range = 24-82 months). Among them, functional recovery was achieved in 52 (68%) at the last follow-up, whereas sequelae were seen in 24 people, the most common being active epilepsy (n = 9, 12%).
Significance:
The primary etiology in the NORSE cohort was autoimmune, followed by unknown causes. Response to immunotherapy is highly associated with long-term mortality, even in those with unknown etiology.
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