Aug 2026· Neurological Research and Practice· Vol 8· 0 citations· 26 references
Medicine
TL;DR
Beyond the clinical severity of NORSE, the findings show that cohort composition is highly sensitive to the temporal operationalization of consensus criteria, and standardized application of diagnostic timing may therefore be essential for comparability across NORSE studies.
Abstract
New-onset refractory status epilepticus (NORSE) is a rare, life-threatening condition occurring in individuals without prior epilepsy or acute structural, toxic, or metabolic causes. Its etiology often remains cryptogenic, and inconsistent terminology hampers early recognition and treatment. This study aims to better characterize NORSE to support faster clinical diagnosis and management. We retrospectively identified all adult patients with status epilepticus (SE) admitted to the Department of Neurology, University Hospital Marburg, Germany, between 2011 and 2023. Demographic, etiologic, treatment, and outcome data from patients fulfilling consensus criteria for NORSE were compared with those of non-NORSE patients. We additionally performed a sensitivity analysis applying strict temporal diagnostic criteria (≤ 72 h) to assess the impact of consensus-based classification on cohort definition. Of 779 patients with status epilepticus, 120 fulfilled criteria for NORSE, delineating a distinct clinical subgroup characterized by advanced age and a female predominance (67.5%, p = 0.028). Phenotypically, NORSE was strongly associated with generalized seizure semiology (70.7%, p < 0.001) and a higher burden of nonconvulsive status epilepticus (45.8%, p < 0.001), while the underlying etiology frequently remained cryptogenic (56.7%). This constellation translated into an adverse clinical trajectory, reflected by reduced rates of discharge home, increased in-hospital mortality (19.5%, p < 0.001), and greater reliance on rehabilitative care. Notably, these differences were not paralleled by alterations in metabolic indices such as HbA1c or cholesterol, underscoring the limited discriminatory value of these parameters in NORSE. Application of strict temporal criteria resulted in a smaller, more conservative NORSE cohort, while key clinical features and outcomes remained largely unchanged. NORSE defines a distinct and severe clinical syndrome within status epilepticus, characterized by older age, female predominance and adverse outcomes. Beyond the clinical severity of NORSE, our findings show that cohort composition is highly sensitive to the temporal operationalization of consensus criteria. Standardized application of diagnostic timing may therefore be essential for comparability across NORSE studies.
Real-world insights into RSE in a resource-limited setting are offered, highlighting preventable causes, management complexity, and high care demands, and favorable outcomes are often achievable.
Jocelyn Cruz-Pérez, J. Zepeda-Pérez, E. Z. Camacho-Castillo et al.· Neurocritical Care· 0 citations
Refractory status epilepticus (RSE) is a life-threatening neurological emergency in children. However, real-world evidence on pediatric RSE remains limited. This study characterized treatment patterns, in-hospital outcomes, and predictors of clinical outcomes within a resource-constrained regional referral network. We performed a single-center retrospective cohort study of pediatric patients admitted with RSE between March 2018 and March 2026. Demographic, clinical, treatment, and outcome data were systematically collected. A total of 215 pediatric RSE episodes were included (median age, 8.0 years; interquartile range [IQR], 5.8-11.8 years); 45.1% were female, and 39.5% had a history of epilepsy. All patients received benzodiazepines as first-line therapy, but only 67 episodes received guideline-recommended dosing. Diazepam, levetiracetam, and midazolam infusion were the most commonly used first-, second-, and third-line therapies, respectively. Favorable discharge outcomes were observed in 50.7% of patients and were independently associated with prior epilepsy (odds ratio [OR], 2.379; 95% confidence interval [CI], 1.07-5.27), adequate first-line benzodiazepine dosing (OR, 2.104; 95% CI, 1.02-4.33), and shorter duration of status epilepticus (OR, 1.991; 95% CI, 1.53-2.58). In-hospital mortality was 15.3% and was associated with acute etiologies (OR, 3.265; 95% CI, 1.27-8.40); prior epilepsy was inversely associated with mortality (OR, 0.328; 95% CI: 0.15-0.73). In-hospital mortality in pediatric RSE is associated with acute etiologies, whereas prior epilepsy is associated with lower mortality. Favorable functional outcomes are associated with prompt guideline-concordant first-line treatment and shorter seizure duration. These findings suggest that key prognostic determinants of pediatric RSE are consistent across different resource settings.
PURPOSE
The prognostic significance of nonconvulsive status epilepticus (NCSE) compared with convulsive status epilepticus (CSE) remains uncertain. We compared clinical features, treatment burden and outcomes of both phenotypes in critically ill adults, and assessed independent predictors of mortality and functional outcome.
METHODS
We conducted a retrospective study of 168 adults with status epilepticus admitted to a tertiary intensive care unit. After exclusion of 4 patients with undocumented semiology, the comparative cohort included 164 patients: 83 with CSE and 81 with NCSE. Five patients evolving from CSE to NCSE were classified as NCSE. NCSE was diagnosed using clinical assessment, EEG monitoring and the Salzburg Consensus Criteria. Outcomes were hospital mortality and modified Rankin Scale (mRS) >3 at discharge. Multivariable logistic regression identified independent predictors.
RESULTS
Baseline age, APACHE II score and Glasgow Coma Scale did not differ between groups. Among NCSE patients, 35 fulfilled Salzburg criterion IIA and 36 criterion IIB. A benzodiazepine diagnostic trial was recorded in 27 patients, with EEG improvement in 25. Hospital mortality was similar in CSE and NCSE (34%vs 32%; p = 0.869). NCSE was associated with greater treatment burden, longer time to seizure control, longer ICU stay and worse functional outcome (mRS >3: 57% vs 84%; p < 0.001). Mortality was independently associated with age, anoxic encephalopathy and super-refractory status, but not NCSE. NCSE independently predicted poor functional outcome.
CONCLUSION
NCSE did not independently increase mortality, but was associated with greater therapeutic complexity and worse functional outcome at discharge, supporting early EEG monitoring and etiology-guided treatment.
A. Canabal-Berlanga, Isabel Magaña, C. Alvargonzález et al.· Seizure· 0 citations
PURPOSE
To evaluate clinical determinants of refractoriness and outcome in status epilepticus (SE) in a prospective low- and middle-income country (LMIC) cohort.
METHODS
In this single-centre prospective cohort study, 97 consecutive SE patients were classified as non-refractory status (NRSE, n = 50), refractory (RSE, n = 31), or super-refractory SE (SRSE, n = 16). A single 21 channel video-EEG was done within 24 h of presentation to the hospital. Clinical context, preexisting epilepsy history, etiology, Status Epilepticus Severity Score (STESS) and EEG findings were recorded, and outcome was assessed through in-hospital mortality.
RESULTS
The median age of our cohort was 32 years; most common etiologies were remote symptomatic (68%), acute symptomatic (19%) and progressive symptomatic (5%). Patients with drug-sensitive epilepsy (DSE) showed low refractoriness and mortality (29%, 6%), whereas drug-resistant epilepsy and new-onset status epilepticus (NOSE) were more likely to be refractory (68% and 70% respectively) and had higher mortality (26% and 30% respectively). Overall mortality was 16.5%, increasing stepwise from 4% (NRSE) to 13% (RSE) to 63% (SRSE; p < 0.001). STESS discriminated between non-survivors and survivors (median 3 vs 0; p < 0.001). Among 85 patients with available EEG, periodic discharges (PDs) were associated with 71% mortality (p < 0.001).
CONCLUSION
Status Epilepticus in the setting of DSE carries a favourable prognosis, while DRE and NOSE confer refractoriness. A short-term early EEG performed in the first 24 h is associated with refractoriness and its exact role warrants further studies.
S. Lodha, Babu Rao Challepalle, Shubho Acharya et al.· Epilepsy & Behavior· 0 citations
The present case demonstrates that RSE with cortical involvement on MRI could be anatomically associated with CLN, which is accompanied by massive infiltration of plasma cells and T lymphocytes, which was seen in the overlying subarachnoid membrane and in the adjacent intact appearing parenchyma.
Taka-aki Miyahira, Y. Takei, Junji Ikeda et al.· The Egyptian Journal of Neur...· 0 citations
This study explored the association between etiology of status epilepticus (SE) and treatment responsiveness. Consecutive episodes of nonhypoxic SE in patients ≥14 years old were included. Etiology was classified into acute, remote, progressive, defined electroclinical syndromes, and unknown. Four subcategories of acute etiologies were then considered based on the recent proposal: (1) triggering factors in patients with pre-existing epilepsy; (2) acute insults to central nervous system (CNS; "acute-primary CNS"); (3) CNS pathology secondary to metabolic disturbances, systemic infection, or fever ("acute-secondary CNS"); and (4) drug/alcohol intoxication or withdrawal ("acute-toxic"). Multinomial logistic regression models were fitted using a backward stepwise selection. Age, sex, consciousness before treatment, SE semiology, SE etiology, SE recurrence, level of disability, and year of presentation were independent variables. The etiological categories of acute-primary CNS (p < .001), acute-secondary CNS (p = .002), progressive (p = .010), and unknown (p = .018) SE were associated with increased odds of refractory SE. Acute-primary CNS (p < .001), progressive (p = .009), and unknown (p = .048) SE etiologies were associated with increased odds of superrefractory SE, whereas no association was found with acute-secondary CNS etiology (p = .948). Patients with SE showed different responsiveness to medications according to the underlying cause. Heterogeneity exists within the spectrum of acute symptomatic causes; distinct etiological subcategories may carry different risks of refractoriness and superrefractoriness.
Simona Lattanzi, G. Giovannini, Niccolò Orlandi et al.· Epilepsia· 0 citations
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