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Summary Objective In 2015, the International League Against Epilepsy (ILAE) proposed a new definition of status epilepticus (SE): 5 minutes of ongoing seizure activity to diagnose convulsive SE (CSE, ie, bilateral tonic–clonic SE) and 10 minutes for focal SE and absence SE, rather than the earlier criterion of 30 minutes. Based on semiology, several types of SE with prominent motor phenomena at any time (including CSE) were distinguished from those without (ie, nonconvulsive SE, NCSE). We present the first population‐based incidence study applying the new 2015 ILAE definition and classification of SE and report the impact of the evolution of semiology and level of consciousness (LOC) on outcome. Methods We conducted a retrospective population‐based incidence study of all adult patients with SE residing in the city of Salzburg between January 2011 and December 2015. Patients with hypoxic encephalopathy were excluded. SE was defined and classified according to the ILAE 2015. Results We identified 221 patients with a median age of 69 years (range 20‐99 years). The age‐ and sex‐adjusted incidence of a first episode of SE, NCSE, and SE with prominent motor phenomena (including CSE) was 36.1 (95% confidence interval [CI] 26.2‐48.5), 12.1 (95% CI 6.8‐20.0), and 24.0 (95% CI 16.0‐34.5; including CSE 15.8 [95% CI 9.4‐24.8]) per 100 000 adults per year, respectively. None of the patients whose SE ended with or consisted of only bilateral tonic–clonic activity died. In all other clinical presentations, case fatality was lower in awake patients (8.2%) compared with patients with impaired consciousness (33%). Significance This first population‐based study using the ILAE 2015 definition and classification of SE found an increase of incidence of 10% compared to previous definitions. We also provide epidemiologic evidence that different patterns of status evolution and LOCs have strong prognostic implications.

Background Early recognition and treatment of autoimmune encephalitis (AE) has become an essential issue in clinical practice. However, little is known about patients with deteriorating conditions and the need for intensive care treatment. Here, we aimed to characterize underlying aetiologies, clinical symptoms, reasons for intensive care admission, and mortality of critically ill patients with AE. Methods We conducted a retrospective chart review of all patients with “definite” or “probable” diagnoses of AE treated at our neurological intensive care unit between 2002 and 2015. We collected and analyzed clinical, paraclinical, laboratory findings and assessed the mortality at last follow-up based on patient records. Results Twenty-seven patients [median age 55 years (range 25–87), male = 16] were included. Thirteen (48%) had “definite” AE. The most common reasons for admission were status epilepticus (7/27, 26%) and delirium (4/27, 15%). One-year survival was 82%, all five deceased were male, and 3 (60%) of them had “probable” disease. The non-survivors (median follow-up 1 year) were more likely to have underlying cancer and higher need for respiratory support compared to the survivors (p

Status epilepticus (SE) is one of the most common neurological emergencies with a high incidence in the elderly. Major determinants of prognosis are patients' age, duration of SE and underlying etiology. We aimed identifying differences in clinical presentation of SE, etiologies and outcome between patients (pts.) sixty years or older (≥60) and younger than sixty (60) years (yrs).We retrospectively analyzed 120 patients (48 women) with SE admitted to the Neurological Intensive Care Unit (NICU), Department of Neurology, Paracelsus Medical University Salzburg, Austria between 1/2011 and 01/2013.Median age was 69 years (range 14-90) (63% ≥60yrs). Generalized tonic clonic SE was the most common SE type, whereas non convulsive SE with and without coma tended to occur more frequently in the elderly (33% ≥60 yrs. vs. 20%60 yrs, Chi2=3.511, p=0.061). Preexisting history of epilepsy was more common in the younger age group (64% vs 41% p=0.014). An acute symptomatic cause of SE was identified in 25% (31/120), with cerebrovascular diseases being more frequent in the elderly (47% vs. 11%; p0.01). Duration of SE did not differ between the age groups (p=0.63). Mortality was higher in elderly patients (31% vs. 7%, p=0.028, Chi Square=5.18) and moderate disability in younger patients (42% vs 17%; p=0.005, Chi Square=7.83). After Bonferroni correction only the higher rate of cerebrovascular etiologies in the elderly was statistically significant.In the elder population, SE occurs more often in patients without preexisting epilepsy and is most frequently caused by cerebrovascular diseases. NCSE tends to be more frequent in the elderly and diagnosis is complicated by subtle clinical presentation. Even though comorbidities represent treatment limitations, in our sample no differences in choice of AED as well as dosage were observed between the age groups, reflecting a trend toward AEDs with more favorable adverse event profile in all patients. SE in older patients is associated with poorer outcome and higher mortality.

Objective The aim was to describe the safety and efficacy of (S)-ketamine [(S)-KET] in a series of patients with refractory and super-refractory status epilepticus (RSE and SRSE) in a specialized neurological intensive care unit (NICU). Methods We retrospectively analyzed the data of patients with RSE and SRSE treated with (S)-KET in the NICU, Salzburg, Austria, from 2011 to 2015. Data collection included demographic features, clinical presentation, diagnosis, electroencephalogram (EEG) data, anticonvulsant treatment, timing, and duration of treatment with (S)-KET. Outcomes were seizure control and death. Results A total of 42 patients (14 women) with RSE and SRSE were treated with (S)-KET. The median duration of status epilepticus (SE) was 10 days [first quartile (Q1) 5.0, Q3 21.0]; the median latency from SE onset to the first administration of (S)-KET was 3 days (Q1 2.0, Q3 6.8). Prior to (S)-KET administration, patients had received a median of two (Q1 2.0, Q3 3.0) anesthetics and three (Q1 2.0, Q3 4.0) antiepileptic drugs. Forty percent of patients (17/42) received propofol: 65 % prior to (S)-KET; 35 % at the same time with (S)-KET. Seven patients received a median bolus of (S)-KET of 200 mg (Q1 200, Q3 250) followed by a continuous infusion, while 35 started with a continuous infusion (maximum rate median 2.55 mg/kg/h; Q1 2.09, Q3 3.22). In 64 % of patients (27/42), (S)-KET was the last drug before SE cessation; in five patients, it was given with propofol at the same time. Median duration of administration was 4 days (Q1 2.0, Q3 6.8). Overall (S)-KET treatment was well tolerated, adverse effects were not observed, and overall mortality was 45.2 %. Conclusions Treatment of SRSE in adult patients with (S)-KET led to resolution of status in 64 %. No adverse events were found, indicating a favorable safety profile.

Introduction In refractory status epilepticus (SE), because of subcellular maladaptive changes, GABAergic drugs are no longer effective, and the excitatory neurotransmitter glutamate (Glu) plays a major role in seizure perpetuation. Perampanel (PER, licensed since 09/2012) is the first orally active noncompetitive AMPA receptor antagonist for adjunctive treatment of refractory focal epilepsy. Methods We analyzed treatment response, seizure outcome, and adverse effects of add-on treatment with perampanel in patients with refractory status epilepticus in the Neurological Intensive Care Unit (NICU), Salzburg, Austria between 09/2012 and 11/2014 by retrospective chart review. Results Twelve patients (75% women) with refractory status epilepticus were treated with PER administered per nasogastric tube between 09/2012 and 11/2014. Median age was 75 years [range: 60–91]. The most frequent SE type was nonconvulsive SE (NCSE) with (5/12, 42%) and without coma (6/12, 50%). In seven patients (58%), SE arose de novo, with an acute symptomatic cause in five patients (42%). Cerebrovascular diseases (4/12, 33%) and cerebral tumors (4/12, 33%) were the most common etiologies. Perampanel was given after a median number of four antiepileptic drugs [range: 2–7] and a median time of 1.5 days [range: 0.8–18.3]. In one patient (8%), clinical improvement was observed within 24 h and EEG improvement within 60 h after administration of PER, while in another patient (8%), clinical and EEG improvement was observed more than 48 h after administration. Median initial dose was 4 mg [range: 2–12; SD: 4.11], titrated up to a median of 12 mg [range: 4–12] in steps of 2 to 4 mg per day. No adverse effects were reported regarding cardiorespiratory changes or laboratory parameters. Outcomes after SE were moderate disability in five patients (42%), death in three patients (25%), and persistent vegetative state in two patients (17%). Conclusion Though glutamate plays a major role in seizure perpetuation, the noncompetitive AMPA receptor antagonist PER could only ameliorate seizure activity in a few patients with refractory SE. The long duration of SE before the administration of PER via nasogastric tube, as well as relatively low doses of PER, might be responsible for the modest result. Perampanel was well tolerated, and no adverse events were reported. This article is part of a Special Issue entitled Status Epilepticus.

Brivaracetam (BRV) is a high-affinity synaptic vesicle glycoprotein 2A ligand that is structurally related to levetiracetam (LEV). Compared to LEV, its affinity to the ligand is >10%-30% higher. Due to its more lipophilic characteristics, it might have a quicker penetration across the blood-brain barrier and potentially also a stronger anticonvulsant effect. Thus, we aimed to explore its usefulness in the treatment of status epilepticus (SE). We retrospectively assessed treatment response and adverse events in adjunctive treatment with intravenous BRV in patients with SE from January 2016 to July 2017 at our institution. Seven patients aged median 68 years (range = 29-79) were treated with intravenous BRV. Three patients had SE with coma and four without. SE arose de novo in two patients; etiology was remote symptomatic in four patients and progressive symptomatic in one patient. The most frequent etiology was remote vascular in two patients. BRV was administered after median four antiepileptic drugs (range = 2-11). Time of treatment initiation ranged from 0.5 hours to 105 days (median = 10.5 hours). Immediate clinical and electrophysiological improvement was observed in two patients (29%). Median loading dose was 100 mg intravenously over 15 minutes (range = 50-200 mg), titrated up to a median dose of 100 mg/d (range = 100-300). Median Glasgow Outcome Scale score was 3 (range = 3-5), with an improvement in 86% of patients compared to admission. We observed no adverse events regarding cardiorespiratory function. BRV might have potential as a novel antiepileptic drug in early stages of SE. Its potential may lie its ability to cross the blood-brain barrier more quickly than LEV and its favorable safety profile. Prospective studies for the use of BRV in SE are required.

Der englische Begriff „motor neuron disease“ (MND) beschreibt eine Gruppe von Erkrankungen, die wahrscheinlich durch einen degenerativ bedingten Verlust der motorischen Nervenfasern charakterisiert ist. Dabei konnen isoliert sowohl das 1. als auch das 2. Motoneuron (MN) oder auch beide gemeinsam betroffen sein. Letzteres ist am haufigsten und der typische Befund bei amyotropher Lateralsklerose (ALS). Die ALS ist eine seltene Erkrankung. Weltweit sind etwa 150.000 ALS-Falle bekannt. Die Aufklarung uber Diagnose und Spontanverlauf sollte sich nach den Informationswunschen und -bedurfnissen der Patienten richten und mit der notwendigen Zuruckhaltung auch den Wunsch der Patienten nach einer prognostischen Aussage mitberucksichtigen. Des Weiteren sollte auch an eine schriftliche Willenserklarung in Form einer Patientenverfugung gedacht werden.

Background Prevention and early recognition of critical illness in patients with autoimmune encephalitis (AE) is essential to achieve better outcome. Aim of the study To evaluate risk factors for intensive care unit (ICU) admission and its prognostic impact in patients with AE. Patients and methods A reclassification of patients hospitalized between 2011 and 2016 revealed 17 “definite” and 15 “probable” AE cases. Thirteen patients (41%) developed critical illness and required ICU admission. The underlying conditions were intractable seizures or status epilepticus (54%), altered mental state (39%), and respiratory failure (8%). Results ICU admission was associated with longer time from first symptoms to hospitalization (p = 0.046). Regression analysis revealed that anemia on hospital admission and definite diagnosis of AE was associated with a higher risk of acquiring critical illness. At last follow-up after a median of 31 months (range 2.5–52.4), seven patients had died (23%) and 63% had a good outcome [modified Rankin Scale (mRS) 0–3]. Anemia was associated with poor prognosis (p = 0.021), whereas development of critical illness did not impact mortality and functional outcome. Conclusion We confirmed the need for ICU care in a subgroup of patients and the prevailing objective is improved seizure control, and definite diagnosis of AE and anemia were identified as risk factors for development of critical illness. However, prognosis was not affected by ICU admission.

Status epilepticus (SE) is a neurological emergency with high mortality and often a poor functional outcome amongst survivors. So far, only status epilepticus severity score (STESS) is available to predict individual outcomes. STESS is based on weighted sum scores of age, type of seizure, level of consciousness and history of previous seizures. Weighting factors were based on a priori assumptions. We tested in an explorative, hypothesis generating approach, whether epidemiological data (i.e. mortality rates) can be combined to form a score (Epidemiology-based Mortality score in SE—EMSE), and further, which combination of aetiology, age, comorbidity, EEG, duration and level of consciousness yields highest test performance. Positive and negative predictive value, and correctly classified were compared to STESS (with different cut-off levels: STESS-3, STESS-4). Score points for each parameter, e.g. age, were derived from previously published specific mortality rates. For each combination of parameters, the lowest sum-score of deceased individuals was taken as cut-off. Ninety-two consecutive non-hypoxic patients (age range 20–90 years), with various forms of SE admitted to a tertiary care neurological intensive care unit were investigated retrospectively. EMSE using a combination of aetiology, age, comorbidity and EEG (NPV = 100 %, PPV = 68.8 %, correctly classified 89.1 %) was superior to STESS-3 and STESS-4 (p = 0.0022 or lower). EMSE explained individual mortality in almost 90 % of cases, and performed significantly better than previous scores. This explorative study needs external prospective corroboration. EMSE may be a valuable tool for risk stratification in interventional studies in the future.

The aim of our study was to assess whether cerebral artery clots undergo time-dependent morphological and compositional changes in acute ischemic stroke. We performed a retrospective chart review of patients admitted within 5 h from symptom onset to three European stroke centers and evaluated non-contrast-enhanced CT (NECT) for hyperdense artery signs (HAS) in 2565 scans. The occlusion site, density of HAS expressed in Hounsfield units (HU), area of HAS, and relative density (rHU) (HU clot/HU non-affected artery) were studied and related to time from symptom onset, clinical severity, stroke etiology, and laboratory parameters. A HAS was present in the middle cerebral artery (MCA) in 185 (7.2%) and further explored. The mean time from symptom onset to CT was 100 min (range 17-300). We found a time- dependent loss of density in the occluded M1 segment within the first 5 h (N = 118, 95% CI [-15, -2], p = 0.01). Further, the thrombus area in the M2 segment decreased with time (cubic trend N = 67, 95% CI [-63, -8], p = 0.02). Overall, and especially in the M2 segment, a lower clot area was associated with higher fibrinogen (-21.7%, 95% CI [-34.8, -5.8], p = 0.009). In conclusion, our results disclosed time-dependent changes of intracranial thrombi with regard to occlusion site, density and area.

Objective: Nontraumatic spinal cord injuries (NTSCIs) form a heterogeneous group of diseases, which may evolve into a life-threatening condition. We sought to characterize spectrum, causes of admission and predictors of death in patients with NTSCI treated at the neurological intensive care unit (NICU). Methods: We performed a retrospective observational analysis of NTSCI cases treated at a tertiary care center between 2001 and 2013. Among the 3937 NICU admissions were 93 patients with NTSCI (2.4%). Using multivariate logistic regression analysis, we examined predictors of mortality including demographics, etiology, reasons for admission and GCS/SAPS (Glasgow Coma Scale/Simplified Acute Physiology Score) scores. Results: Infectious and inflammatory/autoimmune causes made up 50% of the NTSCI cases. The most common reasons for NICU admission were rapidly progressing paresis (49.5%) and abundance of respiratory insufficiency (26.9%). The mortality rate was 22.6% and 2.5-fold higher than in the cohort of all other patients treated at the NICU. Respiratory insufficiency as the reason for NICU admission [odds ratio (OR) 4.97, 95% confidence interval (CI) 1.38–17.9; p < 0.01], high initial SAPS scores (OR 1.04; 95% CI 1.003–1.08; p = 0.04), and the development of acute kidney injury throughout the stay (OR 7.25, 1.9–27.5; p = 0.004) were independent risk factors for NICU death. Conclusions: Patients with NTSCI account for a subset of patients admitted to the NICU and are at risk for adverse outcome. A better understanding of predisposing conditions and further knowledge of management of critically ill patients with NTSCI is mandatory.

BACKGROUND: Over the course of multiple sclerosis (MS) several conditions may arise that require critical care. We aimed to study the reasons for admission and outcome in patients with MS admitted to a neuro-intensive care unit (NICU). METHODS: We retrospectively searched the electronic charts of a 9-bedded NICU in a tertiary hospital for patients with a diagnosis of multiple sclerosis (MS) from 1993-2015, and matched them to NICU controls without MS based on age and gender. Conditional logistic regression was used to compare admission causes, Charlson's Comorbidity Index, indicators of disease severity, and survival between MS and non-MS patients. RESULTS: We identified 61 MS patients and 181 non-MS controls. Respiratory dysfunction was the most frequent reason for NICU admission among MS patients (34.4%), having infectious context as a rule. In a matched analysis, after adjusting for co-morbidities and immunosuppressive medications, patients with MS were more likely to be admitted to the NICU because of respiratory dysfunction (OR = 7.86, 95% CI 3.02-20.42, p

Background Status epilepticus (SE) is a frequent neurological emergency complicated by high mortality and often poor functional outcome in survivors. The aim of this study was to review available clinical scores to predict outcome. Methods Literature review. PubMed Search terms were “score”, “outcome”, and “status epilepticus” (April 9th 2015). Publications with abstracts available in English, no other language restrictions, or any restrictions concerning investigated patients were included. Results Two scores were identified: “Status Epilepticus Severity Score — STESS” and “Epidemiology based Mortality score in SE — EMSE”. A comprehensive comparison of test parameters concerning performance, options, and limitations was performed. Epidemiology based Mortality score in SE allows detailed individualization of risk factors and is significantly superior to STESS in a retrospective explorative study. In particular, EMSE is very good at detection of good and bad outcome, whereas STESS detecting bad outcome is limited by a ceiling effect and uncertainty of correct cutoff value. Epidemiology based Mortality score in SE can be adapted to different regions in the world and to advances in medicine, as new data emerge. In addition, we designed a reporting standard for status epilepticus to enhance acquisition and communication of outcome relevant data. A data acquisition sheet used from patient admission in emergency room, from the EEG lab to intensive care unit, is provided for optimized data collection. Conclusion Status Epilepticus Severity Score is easy to perform and predicts bad outcome, but has a low predictive value for good outcomes. Epidemiology based Mortality score in SE is superior to STESS in predicting good or bad outcome but needs marginally more time to perform. Epidemiology based Mortality score in SE may prove very useful for risk stratification in interventional studies and is recommended for individual outcome prediction. Prospective validation in different cohorts is needed for EMSE, whereas STESS needs further validation in cohorts with a wider range of etiologies. This article is part of a Special Issue entitled “Status Epilepticus”.

WCN 2013 No: 2147 Topic: 1 — Epilepsy Status epilepticus in the elderly on a neurological intensive care unit A. Rohracher, D.P. Huber, J. Dobesberger, J. Hofler, H. Novak, A. Thomschewski, A. Zerbs, A. Akhundova, E. Trinka. Department of Neurology, Paracelsus Medical University, Salzburg, Austria; Department of Neurology, Medical University Azerbaijan, Baku, Azerbaijan Background: Status epilepticus (SE) is a common neurological emergency in the elderly and is associated with high morbidity and mortality. Objective: We aimed to compare clinical factors and outcome of SE in patients ≥60 and b60 years. Patients and methods: We analyzed 137 patients with SE admitted to our NICU between 1/2011 and 12/2012 retrospectively, grouped them according to age (≥60 and b60) and compared clinical factors, etiology and outcome between the groups. Results: Median age was 68 years (range 14–90), 62% were ≥60 yrs (85/137), and 38% (52/137) were b60 yrs. Tonic–clonic SE was the most frequent type in both groups (48% ≥ 60 vs. 58% b 60), comatose NCSE occurred more frequently in older patients (25% ≥ 60 vs. 6% b 60) (p = 0.466). SE lasted b30 min in 36% (49/137), 30–60 min in 26% (35/137) and N7 d in 4% (5/137) and did not differ between the groups (p = 0.97). 52% (71/137) had preexisting seizures (41% ≥ 60 vs.69% b 60) (p = 0.15). An acute symptomatic cause of SE was identified in 26% (36/137) with cerebrovascular diseases being more frequent in the elderly group (43% ≥ 60 vs. 10% b 60) (p b 0.01). Mortality was significantly higher in patients ≥60 (22% ≥ 60 vs. 6% b 60) (p = 0.01), moderate disability in younger patients (17% ≥ 60 vs. 42% b 60) (p b 0.01). Conclusion: In the elderly population SE occurs more often in patients without preexisting epilepsy and is most frequently caused by cerebrovascular diseases. Non-convulsive SE with coma occurs more frequently in patients ≥60. SE in older patients is associated with poorer outcome and higher mortality. doi:10.1016/j.jns.2013.07.169 Abstract — WCN 2013 No: 2488 Topic: 1 — Epilepsy Evaluation of cognitive deficit in patients with epilepsy measured with MMSE (mini mental state examination) WCN 2013 No: 2488 Topic: 1 — Epilepsy Evaluation of cognitive deficit in patients with epilepsy measured with MMSE (mini mental state examination) E. Suljic, A. Ilda, N. Subasic, I. Kapetanovic, A. Mehicevic. Neurology Clinic, University Clinical Center of Sarajevo, Bosnia and Herzegovina; Ophthalmology Clinic, University Clinical Center Sarajevo, Sarajevo, Bosnia and Herzegovina Background: Cognitive deficit represents damage of intellectual abilities that manifest themselves in the form of disorientation in time and space, impaired attention and memory, inability to recognize, judgment and reasoning, and impairment of complex intellectual abilities such as analyzing and using information. Objective: To determine the prevalence and major parameters of cognitive deficits in patients with epilepsy, depending on the type of anti epileptic therapy compared to the control group. Patients and methods: Study enrolled 100 subjects of both sexes, the age structure of 18–65 years old who were divided into two groups: group with 60 patients suffering from epilepsy and a control group of 40 healthy subjects. Results: Of the total number of patients with epilepsy, 55% had cognitive deficits, where the deficit of attention and concentration was present in about 57% treated with valproate, 53% treated with carbamazepine and 42% treated with phenobarbital. Phenobarbital affected the understanding of spoken words by 16.6%, and the difficulties in tracing the figures were most pronounced in patients taking carbamazepine (76.47%) and lamotrigine (47%). Conclusion: The results showed significant incidence of cognitive deficits in patients with epilepsy compared to the control group. Patients with epilepsy had the most damaged domains of attention and concentration on the MMSE. Phenobarbital, carbamazepine and valproate are anticonvulsants that have led to the dominance of attention and concentration, while patients on carbamazepine and lamotrigine had major difficulties in tracing figures. doi:10.1016/j.jns.2013.07.170 Abstract — WCN 2013 No: 2505 Topic: 1 — Epilepsy The situation of the EEG service in a developing country/Egypt WCN 2013 No: 2505 Topic: 1 — Epilepsy The situation of the EEG service in a developing country/Egypt K.A.M.T. Ahmed. Neurology Department, Ain Shams University