Treating status epilepticus in adults. / Behandling av status epilepticus hos voksne.

This clinical review examines the treatment of status epilepticus, a condition in which epileptic seizures are prolonged and pose a significant risk of brain damage and death. International guidelines recommend the use of benzodiazepines as first-line treatment, and these should be administered promptly and in appropriate doses. Second-line treatment involves the use of high-dose anti-seizure medications to stop and prevent seizures. If seizure activity persists, general anaesthesia should be administered as soon as possible. All neurological hospital departments should have established and rehearsed protocols for treating status epilepticus.

A man in his twenties with weakness and numbness in his legs. / En mann i 20-årene med svakhet og nummenhet i beina.

Neuropathy can have many causes, some less well known than others. In this article, we present the case of a young man with progressive neurological deficit over several months. The cause was found to be an increasing social problem.

Prediction of Long-term Survival After Status Epilepticus Using the ACD Score.

Importance: Early prediction of long-term mortality in status epilepticus is important given the high fatality rate in the years after diagnosis. Objective: To improve prognostication of long-term mortality after status epilepticus diagnosis. Design, Settings, and Participants: This retrospective, multicenter, multinational cohort study analyzed adult patients who were diagnosed with and treated for status epilepticus at university hospitals in Odense, Denmark, between January 1, 2008, and December 31, 2017, as well as in Oslo, Norway; Marburg, Germany; and Frankfurt, Germany. They were aged 18 years or older and had first-time, nonanoxic status epilepticus. A new scoring system, called the ACD score, for predicting 2-year (long-term) mortality after hospital discharge for status epilepticus was developed in the Danish cohort and validated in the German and Norwegian cohorts. The ACD score represents age at onset, level of consciousness at admission, and duration of status epilepticus. Data analysis was performed between September 1, 2019, and March 31, 2020. Exposures: Long-term follow-up using data from national and local civil registries in Denmark, Norway, and Germany. Main Outcomes and Measures: The predefined end point was 2-year survival for all patients and for a subgroup of patients with status epilepticus causes that were not damaging or were less damaging to the brain. Neurological deficits before and after onset, demographic characteristics, etiological categories of status epilepticus, comorbidities, survival, time points, treatments, and prognostic scores for different measures were assessed. Results: A total of 261 patients (mean [SD] age, 67.2 [14.8] years; 132 women [50.6%]) were included, of whom 145 patients (mean [SD] age, 66.3 [15.0] years; 78 women [53.8%]) had status epilepticus causes that were not damaging or were less damaging to the brain. The validation cohort comprised patients from Norway (n = 139) and Germany (n = 906). At hospital discharge, 29.8% of patients (n = 64 of 215) had new moderate to severe neurological deficits compared with baseline. New neurological deficits were a major predictor of 2-year survival after hospital discharge (odds ratio, 5.1; 95% CI, 2.2-11.8); this association was independent of etiological category. Nonconvulsive status epilepticus in coma and duration of status epilepticus were associated with development of new neurological deficits, and a simple 3-factor score (ACD score) combining these 2 risk factors with age at onset was developed to estimate survival after status epilepticus diagnosis. The ACD score had a linear correlation with 2-year survival (Pearson r2 = 0.848), especially in the subset of patients with a low likelihood of brain damage. Conclusions and Relevance: This study found that age, long duration, and nonconvulsive type of status epilepticus in coma were associated with the development of new neurological deficits, which were predictors of long-term mortality. Accounting for risk factors for new neurological deficits using the ACD score is a reliable method of prediction of long-term outcome in patients with status epilepticus causes that were not damaging or were less damaging to the brain.

Influence of valproate-induced hyperammonemia on treatment decision in an adult status epilepticus cohort.

INTRODUCTION: Status epilepticus (SE) is a neurological emergency in which immediate intervention is required to prevent permanent brain damage and death. Intravenous (IV) valproic acid (VPA) is often used for the treatment of SE. However, IV VPA frequently increases the blood ammonia level. In this study, we explore the impact of IV VPA-induced hyperammonemia (HA) on treatment management of SE and discuss the challenges related to this particular condition. METHODS: We used data from medical records of 31 adult patients (≥18 years) treated with IV VPA for SE at Oslo University Hospital between January 2006 and October 2019. Clinical and blood sample data and information about the influence of HA on treatment were collected. Correlations between ammonia levels and other continuous or categorical variables were tested using the Pearson's correlation coefficient. The Kruskal-Wallis H-test was used to analyze associations between different variables and treatment decisions. RESULTS: Thirty of 31 patients had increased ammonia level during IV VPA treatment. In 16/30 patients, VPA was discontinued, and in 6/30 patients, the dose was reduced. We found a difference in the median peak ammonia level among the groups where VPA was discontinued (99 µmol/l), reduced (71 µmol/l), and continued (55.5 µmol/l) (P = 0.008). Also clinical status, measured by West Haven Criteria, varied among the groups where VPA was discontinued (3.5), reduced (2.5), and continued (2.0) (P = 0.01). Treatment decisions at peak ammonia were not associated with the level of liver enzymes and bilirubin. CONCLUSION: Hyperammonemia had a substantial impact on further management. To date, no recommendations exist on how to manage VPA-induced HA in SE. We call for systematic prospective studies and evidence-based guidelines.

Predictive performances of STESS and EMSE in a Norwegian adult status epilepticus cohort.

PURPOSE: "Status Epilepticus Severity Score" (STESS) and "Epidemiology-based Mortality Score in Status Epilepticus" (EMSE) are two clinical scoring systems aiming to predict mortality in status epilepticus (SE). The objective of this study was to compare their predictive performances in a cohort of 151 SE-patients from Oslo University Hospital in the period 2001-2017. METHOD: Variables used to calculate STESS (age, previous seizures, worst SE-semiology, level of consciousness) and two different versions of EMSE, EMSE-EAC (etiology, age, comorbidities) and EMSE-EACE (etiology, age, comorbidities, EEG-pattern), as well as outcome were collected retrospectively. Receiver Operating Characteristic (ROC)-analyses, determination of best cut-off values, sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative predictive value (NPV) were performed. In addition, Precision-Recall curves (PRC) were produced, plotting PPV as a function of Se. RESULTS: Thirteen patients (9%) died during their hospital stay. STESS did not accurately predict mortality, with a ROC-curve showing an area under the curve (AUC) of 0.625(95%CI = 0.472-0.783), p = 0.15. EMSE-EAC performed better with an AUC of 0.714(95%CI = 0.552-0.873), p = 0.01 and a best cut-off value of 37. Se was 69.2%, Sp 72.1%, PPV 19% and NPV 96.2%. EMSE-EACE performed best with an AUC of 0.855(95%CI = 0.736-0.976), p < 0.0005 and a best cut-off value of 79. Se was 77.8%, Sp 87.8%, PPV 36.8% and NPV 97.7%. The PRC showed areas under the PRC of 0.23 for EMSE-EAC and 0.46 for EMSE-EACE. CONCLUSIONS: EMSE-EAC and EMSE-EACE performed better than STESS and may be useful in identifying the patients at risk of death in SE. PRC may give a more relevant visual representation of predictive utility than ROC-curves in situations of imbalanced datasets.

Factors associated with refractoriness and outcome in an adult status epilepticus cohort.

PURPOSE: Several multinational prospective registers have shown that a significant proportion of status epilepticus (SE) patients are not treated in line with international guidelines. The aim of this study was to assess quality of treatment and to identify factors associated with refractoriness and outcome in a cohort of adult SE patients in Norway. METHODS: 151 patients treated in Oslo University Hospital from 2001 to 2017 were included. One SE-episode was selected per patient and variables related to the patient, SE-episode, treatment and outcome entered into a database. Status Epilepticus Severity Score (STESS) and Epidemiology-based Mortality Score in SE (EMSE) were calculated for each episode. RESULTS: 68% (n = 102) of SE-episodes were responsive, 20% (n = 30) refractory and 12% (n = 19) superrefractory. Mortality was 9%, with a significant difference between responsive episodes (1%) and refractory (superrefractory included) episodes (24%), p < 0.001. 86% of patients received a benzodiazepine as 1st antiepileptic drug. Multivariate analysis showed that non-convulsive SE in coma was significantly associated with refractoriness (p = 0.04), while focal non-convulsive SE without coma was associated with responsiveness (p = 0.03). Younger age was associated with superrefractoriness (p = 0.02). Regarding outcome, EMSE-EtiologyAgeComorbiditiesEEG (EACE)≥64 (p = 0.02) and use of vasopressors (p = 0.03) were associated with a worsening of the modified Rankin scale at discharge. STESS was only associated with outcome in univariate analysis. CONCLUSION: In this cohort in which international guidelines for treatment of SE were well followed, semiology of the SE was found to be the most important determinant of refractoriness, and the new clinical scoring system EMSE-EACE was robustly associated with outcome.