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Background: A third of people with juvenile myoclonic epilepsy (JME) are drug-resistant. Three-quarters have a seizure relapse when attempting to withdraw anti-seizure medication (ASM) after achieving seizure-freedom. It is currently impossible to predict who is likely to become drug-resistant and safely withdraw treatment. We aimed to identify predictors of drug resistance and seizure recurrence to allow for individualised prediction of treatment outcomes in people with JME. Methods: We performed an individual participant data (IPD) meta-analysis based on a systematic search in EMBASE and PubMed – last updated on March 11, 2021 – including prospective and retrospective observational studies reporting on treatment outcomes of people diagnosed with JME and available seizure outcome data after a minimum one-year follow-up. We invited authors to share standardised IPD to identify predictors of drug resistance using multivariable logistic regression. We excluded pseudo-resistant individuals. A subset who attempted to withdraw ASM was included in a multivariable proportional hazards analysis on seizure recurrence after ASM withdrawal. The study was registered at the Open Science Framework (OSF; https://osf.io/b9zjc/). Findings: Our search yielded 1641 articles; 53 were eligible, of which the authors of 24 studies agreed to collaborate by sharing IPD. Using data from 2518 people with JME, we found nine independent predictors of drug resistance: three seizure types, psychiatric comorbidities, catamenial epilepsy, epileptiform focality, ethnicity, history of CAE, family history of epilepsy, status epilepticus, and febrile seizures. Internal-external cross-validation of our multivariable model showed an area under the receiver operating characteristic curve of 0·70 (95%CI 0·68–0·72). Recurrence of seizures after ASM withdrawal (n = 368) was predicted by an earlier age at the start of withdrawal, shorter seizure-free interval and more currently used ASMs, resulting in an average internal-external cross-validation concordance-statistic of 0·70 (95%CI 0·68–0·73). Interpretation: We were able to predict and validate clinically relevant personalised treatment outcomes for people with JME. Individualised predictions are accessible as nomograms and web-based tools. Funding: MING fonds.

Juvenile myoclonic epilepsy (JME) is the most common idiopathic generalized epilepsy. Disease onset is typically in puberty and poor social adjustment and behavioral disturbances, which resemble frontal lobe dysfunction, are often observed. In recent advanced brain imaging studies on JME patients, emotional and behavioral problems have been associated to subtle structural and functional alterations mainly in frontal cortex and thalamus. There is emerging evidence that patients with abnormal emotion processing and regulation, such as those with bipolar disorder, show disrupted connectivity between limbic structures and frontal cortices. There are no neuroimaging or neuropsychological studies related to emotion processing in patients with JME with a focus on limbic structures. We aimed to address the problem of emotional disturbances and social adjustment in JME patients from multiple aspects through thorough functional and structural assessment, which would potentially enable elaboration of a unifying concept explaining neurobiological background of disturbances in emotional processing and social adjustment in JME patients. Results of this study may potentially enable the development of psychological and pharmacological interventional strategies for managing behavioral disturbances in patients with JME.

Several emergencies were admitted less frequently to the hospital during the coronavirus disease 2019 (COVID‐19) pandemic. To investigate whether this also occurred with status epilepticus (SE) we compared admissions due to first SE from March to April 2020 (“Time of COVID,” TOC) with January to February 2020 (“pre‐COVID,” preCOV). We also compared admission numbers in TOC and preCOV with the respective 2‐month periods in 2018 and 2019 in a retrospective cohort analysis. Two investigators independently searched the hospital patient database for various forms of SE. There was no significant change in the 2‐month incidences of first SE in the city of Salzburg from preCOV of 6.1 (95% confidence interval [CI] 2.9‐12.3) to TOC of 6.9/100 000 adults (95% CI 3.4‐13.3). Admission numbers did not differ significantly from previous years. Estimated adjusted incidence was in line with a recent 5‐year epidemiological study in Salzburg. However, a trend toward less‐frequent nonconvulsive SE (NCSE) and loss of female predominance were indirect hints of underdiagnosing SE. In contrast to other medical conditions, SE most often presents clinically with impaired consciousness, which may promote admission to emergency departments even in times of lock‐down. Further research of medical support of women and patients with NCSE during pandemic‐related restrictions is warranted.

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.

Objective: The European Union–funded E-PILEPSY network (now continuing within the European Reference Network for rare and complex epilepsies [EpiCARE]) aims to harmonize and optimize presurgical diagnostic procedures by creating and implementing evidence-based guidelines across Europe. The present study evaluates the current evidence on the diagnostic accuracy of long-term video-electroencephalographic monitoring (LTM) in identifying the epileptogenic zone in epilepsy surgery candidates. Methods: MEDLINE, Embase, CENTRAL, and ClinicalTrials.gov were searched for relevant articles. First, we used random-effects meta-analytical models to calculate pooled estimates of sensitivity and specificity with respect to postsurgical seizure freedom. In a second phase, we analyzed individual patient data in an exploratory fashion, assessing diagnostic accuracy within lesional and nonlesional temporal lobe epilepsy (TLE) and extratemporal lobe epilepsy (ETLE) patients. We also evaluated seizure freedom rate in the presence of “localizing” or “nonlocalizing” LTM within each group. The quality of evidence was assessed using the QUADAS-2 tool and the GRADE approach. Results: Ninety-four studies were eligible. Forty-four were included in sensitivity meta-analysis and 34 in specificity meta-analysis. Pooled sensitivity was 0.70 (95% confidence interval [CI] = 0.60-0.80) and specificity was 0.40 (95% CI = 0.27-0.54). Subgroup analysis was based on individual data of 534 patients (41% men). In lesional TLE patients, sensitivity was 0.85 (95% CI = 0.81-0.89) and specificity was −0.19 (95% CI = 0.13-0.28). In lesional ETLE patients, a sensitivity of 0.47 (95% CI = 0.36-0.58) and specificity of 0.35 (95% CI = 0.21-0.53) were observed. In lesional TLE, if LTM was localizing and concordant with resection site, the seizure freedom rate was 247 of 333 (74%), whereas in lesional ETLE it was 34 of 56 (61%). The quality of evidence was assigned as “very low.”. Significance: Long-term video-electroencephalographic monitoring is associated with moderate sensitivity and low specificity in identification of the epileptogenic zone. Sensitivity is remarkably higher in lesional TLE compared to lesional ETLE. Substantial heterogeneity across the studies indicates the need for improved design and quality of reporting.

Status epilepticus (SE) is among the most common neurological emergencies. In refractory SE, due to subcellular maladaptive changes, γ-aminobutyric acidergic drugs are no longer effective, and there is an increase in synaptic N-methyl-d-aspartate (NMDA) receptors. Ketamine (KET) is a noncompetitive N-methyl-d-aspartate receptor antagonist that could be of value in the treatment of refractory SE. The aim was to analyze all published studies on KET in the treatment of SE. A systematic search (PubMed) of the literature identified 25 studies (from December 1996 to March 2017); with the exception of two prospective studies, all studies were retrospective in nature and published as a full paper. To date, PubMed lists a total number of 244 episodes of SE treated with KET (13 case reports and five case series in adults; four case reports and three case series in children); the overall success rate was 74% (153/207) in adults and 73% (27/37) in children. Adverse events were rare. Current evidence on the use of KET in acute seizures and SE is mostly restricted to retrospective case reports and case series, except for two prospective case series without control (class IV). Further prospective studies to inform clinicians are necessary.

Purpose The aim of this qualitative study was to determine which perception of personal comfort patients name in the context of their hospitalization in an Austrian Epilepsy Monitoring Unit (EMU). Methods Problem-centred interviews with twelve inpatients were conducted. Data analyses were done according to Mayring's qualitative content analyses following the technique of structuring-deductive category assignment. Results Patients experienced different kinds of comfort along with their hospitalization in the EMU. Comfort-decreasing factors were bed rest , boredom , and waiting for possible seizures . As comfort-increasing factors, hope for enhanced seizure control, support by family and staff, and intelligible information about the necessity of restrictive conditions were identified. Conclusions The study results should assist health care professionals, enabling them to design comfort enhancing interventions for patients undergoing video-electroencephalography (EEG) investigations in an EMU. Some of these seem to be simple and obtainable without high financial or technical effort. Others are more complex and have to be further assessed for their feasibility.

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 Wolf Dietrich of Raitenau (WD) ruled the archiepiscopal Salzburg from March 2nd 1587 to December 17th 1611. He was condemned by his successor Archbishop Markus Sittikus of Hohenems to spend his last years imprisoned at the Fortress Hohensalzburg, where he died on January 16th 1617. This historical note describes the causes of his death. Materials and methods The original Latin handwriting, including the detailed medical history and the autopsy of the Archbishop's body performed by his personal physician, was analyzed in conjunction with historical handwritings provided by St. Peter's Abbey, Salzburg handwriting assigned to Markus Sittikus. Results Wolf Dietrich of Raitenau had his first well-documented left hemispheric stroke in winter 1604/05. He had palsy of his right arm, was unable to write, and, therefore, used a stamp instead of his signature until October 1605. After another stroke, right hemispheric in origin with persisting palsy of his left arm [“leva corporis pars iam pridem simili ex apoplectico assultu in paralysin resoluta”], he developed symptomatic epilepsy with recurring seizures [“epileptico insultu quo etiam alias correptus est”]. On January 15th 1617, he suffered from a secondarily generalized convulsive status epilepticus [“toto corpore convellitur epileptico insultu”] with stertorous breathing and distortion of his face [“spuma stertore insigni faciei perversione”] and was unconscious for 8 h. He recovered from coma and showed dysphagia, buccofacial apraxia [“abolitam diglutiendi facultatem”], reversible speech disturbance [“accisa etiam verba loqui”], and left-sided hemiplegia [“leva corporis pars… immobilis prorsus est reddita”]. The following day, he had speech disturbances, and he died at noon. His autopsy showed large but intact liver [“hepar magnum sanum”] and heart [“cor magnum in quo lapsus nullus”]. There was intrapulmonal mucus [“pituita imbutus”], and part of the lungs adhered to its pleura. He had five kidney stones and a partly cirrhotic spleen. The cause of his death was assumed to be intracerebral [“causa mortis in capite requienda fuisset”]. Discussion The terminal suffering of Wolf Dietrich of Raitenau is the first witnessed case report on a fatal status epilepticus in Salzburg. Most likely, he suffered from vascular epilepsy due to a right hemispheric stroke, leading to status epilepticus with left-sided Todd's palsy and speech disturbances. An acute symptomatic etiology of this disease cannot be ruled out, as for religious reasons, the Archbishop's brain was not autopsied. Conclusion Meticulous medical reporting including autopsy was already available in Salzburg in 1617, and the symptomatic etiology of epilepsy was diagnosed correctly. This article is part of a Special Issue entitled “Status Epilepticus”.

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.

Sowohl Drogenentzug, als auch Intoxikationen stellen eine haufige Ursache fur akut symptomatische Anfalle dar. Die Pathomechanismen beruhen sowohl auf direkten, durch die Droge selbst bedingten, als auch auf indirekten Faktoren (Schadelhirntrauma, metabolische Storungen, intrazerebrale Blutungen oder Ischamien, zerebrale als auch systemische Infektionen). Bei jedem Patienten mit einem erstmaligen epileptischen Anfall muss daher eine umfassende Anamnese und Abklarung einschlieslich MRI, Labor, EKG und EEG erfolgen.

SUMMARY Background. Early identification of potential epilepsy surgery candidates is essential to the treatment process. Aim. To evaluate the clinical applicability of the ILAE definition of drug resistant epilepsy and its potential in identifying surgical candidates earlier compared to three established “older” definitions of drug resistant epilepsy. Material and Methods. Retrospective analysis of 174 patients who underwent epilepsy surgery between 1998 and 2009. Clinical factors and course of disease were extracted from patients' charts. Drug resistant epilepsy was classified according to four definitions and the time until fulfillment of criteria compared. Results. Mean time to fulfillment of criteria of drug resistant epilepsy ranged from 11.8 (standard deviation (SD) 9.8) to 15.6 years (SD 11.3). Time to drug resistance was significantly longer applying the only definition, requiring failure of three antiepileptic drugs (AEDs) (Canada definition), whereas time to fulfillment of all other definitions did not differ. Fifty percent of all patients experienced a seizure free period of ≥1 year prior to being classified as drug resistant, 13% entered another 1-year remission after fulfilling any criteria for drug resistance. Conclusion. We conclude that the ILAE definition identifies drug resistant epilepsy, with similar latency like two of three formerly used definitions. It is an easy applicable tool to minimize the delay of referral to a specialized center. Intermittent remissions delay assessment of drug resistance for all definitions and 13% of patients enter a remission despite established drug resistance.

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.

Summary Objective Safety in epilepsy monitoring units (EMUs) has become an increasing concern because adverse events occur in up to 10% of patients undergoing long‐term video EEG in EMUs. The aim of this study was to assess the effectiveness of a specific safety protocol in an EMU. Methods We retrospectively assessed the adverse event rates in a group without (group 1, 84‐month period, Innsbruck, Austria) and a group with (group 2, 33‐month period, Salzburg, Austria) personalized safety measures utilizing a standardized protocol for long‐term epilepsy monitoring in high‐risk patients. Differences in adverse event rates during and after long‐term video EEG between the two groups were calculated and compared. Results In group 1, 44/507 (9%, 95% confidence interval [CI] 6.5–11.5%) patients experienced 53 adverse events: 20/507 (4%, 95% CI 2.6–6.0%) patients had psychiatric events, 15/507 (3%, 95% CI 1.8–4.8%) patients sustained a total of 19 injuries during seizures, and 10/507 (2%, 95% CI 1.1–3.6%) patients had 13 episodes of status epilepticus; one adverse event was treatment‐related (valproic acid–induced encephalopathy; 1/507, 0.2%, 95% CI 0.0–1.1%). By using the new safety protocol in group 2, the adverse event rate was only 5% (95% CI 3.4–7.6%; 30 adverse events in 26/491; 45% reduction; p = 0.036), in contrast. These events included 13 psychiatric complications in 13/491 (2%, 95% CI 1.6–4.5%, p = 0.252) patients, 12 seizure‐related injuries in 9/491 (2%, 95% CI 1.0–3.4%, p = 0.250) patients, and 5 episodes of status epilepticus in 4/491 (1%, 95% CI 0.3–2.1%, p = 0.120) patients. Significance Implementation of personalized safety measures in high‐risk patients resulted in a clinically relevant reduction of adverse events in the EMU. Safety protocols are a valid tool to reduce the occurrence of adverse events in EMUs.

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.

Introduction The effectiveness of valproate (VPA) in the treatment of focal and generalized epilepsies is well established. The drug has a wide spectrum of action, good tolerability, and has been available as an injectable formulation since 1993. Despite the lack of class A evidence, it has been used extensively in various forms of status epilepticus (SE). Aim Our aim was to present a systematic review of data from randomized and non-randomized controlled trials to evaluate the efficacy and safety of intravenous VPA for the treatment of SE. Methods Data sources included MEDLINE, back tracing of references in pertinent studies, and contact with the manufacturer of VPA (Sanofi-Aventis). Results Overall, the search strategy yielded 433 results (425 MEDLINE, seven congress abstracts, one unpublished study); after excluding duplicate publications and case reports, 30 studies were identified (the earliest was published in 1993, the most recent in 2012); ten were controlled (six randomized controlled trials, four non-randomized controlled studies), and 20 uncontrolled trials (eight prospective observational studies, 12 retrospective case series). The cumulative literature describes the experiences of 860 patients with various forms of SE treated with intravenous VPA. The overall response rate to abrogate SE was 70.9 % (601/848; 95 % confidence interval [CI] 67.8–73.9). Response rates to intravenous VPA were better in children than in adults and did not differ between the SE types. The most commonly reported effective doses were between 15 and 45 mg/kg in bolus (6 mg/kg/min) followed by 1–3 mg/kg/h infusion. Safety studies of intravenous VPA administration in patients with SE showed a low incidence of adverse events overall (

Highlights • We assessed midbrain–hindbrain in a large series of cortical malformation patients. • Midbrain–hindbrain malformations are commonly linked to cortical malformations. • Midbrain–hindbrain malformations are associated with severe clinical phenotype., Summary Midbrain–hindbrain malformations (MHM) may coexist with malformations of cortical development (MCD). This study represents a first attempt to investigate the spectrum of MHM in a large series of patients with MCD and epilepsy. We aimed to explore specific associations between MCD and MHM and to compare two groups of patients: MCD with MHM (wMHM) and MCD without MHM (w/oMHM) with regard to clinical and imaging features. Two hundred and twenty patients (116 women/104 men, median age 28 years, interquartile range 20–44 years at the time of assessment) with MCD and epilepsy were identified at the Departments of Neurology and Pediatrics, Innsbruck Medical University, Austria. All underwent high-resolution MRIs (1.5-T) between 01.01.2002 and 31.12.2011. Midbrain–hindbrain structures were visually assessed by three independent raters. MHM were seen in 17% (38/220) of patients. The rate of patients wMHM and w/oMHM differed significantly (p = 0.004) in three categories of MCD (category I – to abnormal neuronal proliferation; category II – to abnormal neuronal migration; and category III – due to abnormal neuronal late migration/organization): MCD due to abnormal neuronal migration (31%) and organization (23%) were more commonly associated with MHM compared to those with MCD due to abnormal neuronal proliferation (9%). Extensive bilateral MCD were seen more often in patients wMHM compared to those w/oMHM (63% vs. 36%; p = 0.004). In wMHM group compared to w/oMHM group there were higher rates of callosal dysgenesis (26% vs. 4%; p

Status epilepticus is among the most common neurologic emergencies, with a mortality rate of up to 20%. The most important therapeutic goal is fast, effective, and well-tolerated cessation of status epilepticus. Intravenous phenytoin/fosphenytoin, phenobarbital, or valproate is the current standard treatment after failure of benzodiazepines. Lacosamide as a new antiepileptic drug has been available as an intravenous solution since 2009. To date, PubMed lists 19 studies (10 single case reports and 9 case series), reporting a total of 136 episodes of refractory status epilepticus (50% nonconvulsive status epilepticus, 31% focal status epilepticus, and 19% convulsive status epilepticus) treated with lacosamide. The most often used bolus dose was 200-400 mg over 3-5 min. The overall success rate was 56% (76/136). Adverse events (AEs) were reported in 25% (34/136) of patients: mild sedation in 25 cases, 1 patient with possible angioedema, 2 with allergic skin reaction, 4 with hypotension, and 1 with pruritus. One patient developed a third-degree atrioventricular (AV) block and paroxysmal asystole. Overall, the rate of AEs was low. Current evidence on the use of intravenous lacosamide in acute seizures and status epilepticus is restricted to retrospective case reports and case series (class IV). Further prospective studies to inform clinicians are necessary.

The aim of this qualitative study was to determine which perception of personal comfort patients name in the context of their hospitalization in an Austrian Epilepsy Monitoring Unit (EMU).Problem-centred interviews with twelve inpatients were conducted. Data analyses were done according to Mayring's qualitative content analyses following the technique of structuring-deductive category assignment.Patients experienced different kinds of comfort along with their hospitalization in the EMU. Comfort-decreasing factors were bed rest, boredom, and waiting for possible seizures. As comfort-increasing factors, hope for enhanced seizure control, support by family and staff, and intelligible information about the necessity of restrictive conditions were identified.The study results should assist health care professionals, enabling them to design comfort enhancing interventions for patients undergoing video-electroencephalography (EEG) investigations in an EMU. Some of these seem to be simple and obtainable without high financial or technical effort. Others are more complex and have to be further assessed for their feasibility.

Objectives There are clinical situations where it might be appropriate to switch patients from immediate-release oxcarbazepine (OXC) to eslicarbazepine acetate (ESL). We investigated the effects of transitioning patients overnight from OXC to ESL. Materials and Methods A retrospective, single-center study was conducted in which patients with drug-resistant focal epilepsy on a stable dose of immediate-release OXC for at least 4 weeks were switched overnight to ESL. Patients were switched because they experienced persistent seizures with OXC but were unable to tolerate increased OXC dosing due to adverse events. Tolerability was assessed using the Adverse Events Profile (AEP), quality of life was assessed using the Quality of Life in Epilepsy Inventory 10 (QOLIE-10), and alertness was assessed as reaction time using a subtest of the Test Battery for Attention Performance version 2.3. Assessments were performed immediately prior to and 5 days after switching from OXC to ESL (days 0 and 5, respectively). Results The analysis included 21 patients (12 women, 9 men; mean age 36 years). After switching from OXC to ESL, there were significant improvements in mean scores for AEP (P

The non-competitive α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) - receptor antagonist perampanel (PER) was approved in 2015 for treatment of primary generalized tonic-clonic seizures (pGTCS). The aim of this narrative review is to summarize available data on pharmacological properties, efficacy and tolerability of PER in pGTCs.Data sources included MEDLINE, EMBASE, Google Scholar and ClinicalTrials.gov, conference proceedings of the ILAE congresses and the most recent conference proceedings of the American Epilepsy Society (2013 to 2015).A placebo-controlled clinical phase III study including 164 patients (≥ 12 years) with pGTCS in idiopathic generalized epilepsies (IGE) demonstrated efficacy of PER in reducing pGTCS with good tolerability profile, and without aggravating absence seizures or myoclonic seizures. Dizziness, the main adverse event (AE), can be avoided by bedtime administration. Psychiatric AEs ranging from mild depression to aggression and suicidal attempts should be especially monitored in patients with a history of psychiatric disorders. Co-administration of enzyme inducing antiepileptic drugs (AEDs) might decrease PER plasma levels and make dose adjustment necessary. A reduced efficacy of progesterone-containing oral contraceptives should be considered when administering PER to young women. There is lack of evidence on PER treatment in pregnancy. Although no teratogenic effects were observed in animal models, PER is not recommended for women of childbearing age without contraception.

Purpose: The European Union-funded E-PILEPSY network aims to improve awareness of, and accessibility to, epilepsy surgery across Europe. In this study we assessed current clinical practices in epilepsy monitoring units (EMUs) in the participating centers. Method: A 60-item web-based survey was distributed to 25 centers (27 EMUs) of the E-PILEPSY network across 22 European countries. The questionnaire was designed to evaluate the characteristics of EMUs, including organizational aspects, admission, and observation of patients, procedures performed, safety issues, cost, and reimbursement. Results: Complete responses were received from all (100%) EMUs surveyed. Continuous observation of patients was performed in 22 (81%) EMUs during regular working hours, and in 17 EMUs (63%) outside of regular working hours. Fifteen (56%) EMUs requested a signed informed consent before admission. All EMUs performed tapering/withdrawal of antiepileptic drugs, 14 (52%) prior to admission to an EMU. Specific protocols on antiepileptic drugs (AED) tapering were available in four (15%) EMUs. Standardized Operating Procedures (SOP) for the treatment of seizure clusters and status epilepticus were available in 16 (59%). Safety measures implemented by EMUs were: alarm seizure buttons in 21 (78%), restricted patient's ambulation in 19 (70%), guard rails in 16 (59%), and specially designated bathrooms in 7 (26%). Average costs for one inpatient day in EMU ranged between 100 and 2200 Euros. Conclusion: This study shows a considerable diversity in the organization and practice patterns across European epilepsy monitoring units. The collected data may contribute to the development and implementation of evidence-based recommended practices in LTM services across Europe. (C) 2016 The Authors. Published by Elsevier Ltd. on behalf of British Epilepsy Association.

BACKGROUND: Several EEG criteria have been proposed for diagnosis of non-convulsive status epilepticus (NCSE), but none have been clinically validated. We aimed to assess the diagnostic accuracy of the EEG criteria proposed by a panel of experts at the fourth London-Innsbruck Colloquium on Status Epilepticus in Salzburg, 2013 (henceforth called the Salzburg criteria).METHODS: We did a retrospective, diagnostic accuracy study using EEG recordings from patients admitted for neurological symptoms or signs to three centres in two countries (Danish Epilepsy Centre, Dianalund, Denmark; Aarhus University Hospital, Aarhus, Denmark; and Paracelsus Medical University, Salzburg, Austria). Participants were included from the Danish centres if they were aged 4 months or older, and from the Austrian centre if aged 18 years or older. Participants were sorted into two groups: consecutive patients under clinical suspicion of having NCSE (the clinical validation group) or consecutive patients with abnormal EEG findings but no clinical suspicion of NCSE (the control group). Two raters blinded to all other patient data retrospectively analysed the EEG recordings and, using the Salzburg criteria, categorised patients as in NCSE or not in NCSE. By comparing with a reference standard inferred from all clinical and para-clinical data, therapeutic response, and the final outcome, we calculated sensitivity, specificity, overall diagnostic accuracy, positive and negative predictive values, and inter-rater agreement for the Salzburg criteria. The reference standard was inferred by two raters who were blinded to the scorings of the Salzburg criteria.FINDINGS: We retrospectively reviewed EEG data from 220 patients. EEGs in the clinical validation group were recorded in 120 patients between Jan 1, and Feb 28, 2014 (Austria), and Aug 1, 2014, and Jan 31, 2015 (Denmark). EEGs in the control group were recorded in 100 patients between Jan 13 and Jan 22, 2014 (Austria) and Jan 12 and Jan 26, 2015 (Denmark). According to the reference standard, 43 (36%) of the 120 patients in the validation group had NCSE. In the validation cohort sensitivity was 97·7% (95% CI 87·9-99·6) and specificity was 89·6% (80·8-94·6); overall accuracy was 92·5% (88·3-97·5). Positive predictive value was 84·0% (95% CI 74·1-91·5) and negative predictive value was 98·6% (94·4-100). Three people in the control group (n=100) fulfilled the Salzburg criteria and were therefore false positives (specificity 97·0%, 95% CI 91·5-99·0; sensitivity not calculable). Inter-rater agreement was high for both the Salzburg criteria (k=0·87) and for the reference standard (k=0·95). Therapeutic changes occurred significantly more often in the group of patients fulfilling Salzburg criteria (42 [84%] of 50 patients) than in those who did not (11 [16%] of 70; pINTERPRETATION: The Salzburg criteria for diagnosis of NCSE have high diagnostic accuracy and excellent inter-rater agreement, making them suitable for implementation in clinical practice.FUNDING: None.

Recent neuroimaging studies have reported structural and functional brain abnormalities in patients with juvenile myoclonic epilepsy (JME), which may also involve cortical and subcortical networks that are important for decision making. This study is the first attempt to examine decision making in JME. Twenty-two patients with JME (median age 26.00, range 18-50) and 33 healthy controls (median age 26.00, range 18-57) participated in the study. For the JME group, the median age at seizure onset was 14.00 years (range 1-20); the median epilepsy duration was 11.50 years (range 3-45). Eleven patients (50 %) had pharmacoresistant seizures. All participants completed the Iowa Gambling Task (IGT), a widely used standard task of decision making. In this task, contingencies are not explained and feedback on previous decisions has to be used in order to learn to choose the advantageous alternatives. In the IGT, patients with JME showed difficulty in learning to choose advantageously compared to healthy controls. Difficulty was enhanced for the patients with pharmacoresistant seizures. A correlation analysis revealed an association between decision-making performance of patients with JME and executive functions. Results indicate that patients with JME have difficulty in making advantageous decisions and that persistence of seizures might be a critical factor for cognitive functioning. Findings of this study add a new aspect to the neuropsychological profile of JME. Difficulty in decision making may impair functioning of patients with JME in everyday life and affect their adherence to treatment plans.

SUMMARY Status epilepticus (SE) is the most extreme form of epilepsy. It describes a prolonged seizure that may occur in patients with previous epilepsy or in acute disorders of the central nervous system. It is one of the most common neurologic emergencies, with an incidence of up to 41 per 100,000 per year and an estimated mortality is 20%. The three major determinants of prognosis are the duration of SE, patient age, and the underlying cause. Common and easily recognized causes of SE include cerebrovascular disorders, brain trauma, infections, and low antiepileptic drug levels in patients with epilepsy. Less common causes present a clinical and diagnostic challenge, but are major determinants of prognosis. Among them, inflammatory causes and inborn errors of metabolism have gained wide interest; recent insights into these causes have contributed to a better understanding of the pathophysiology of SE and its appropriate treatment. This review focuses on the different etiologies of SE and emphasizes the importance of prompt recognition and treatment of the underlying causes.

Status epilepticus (SE) and seizure clusters (SC) represent neurologic emergencies with a case fatality rate up to 34%, depending on cause and comorbidity. As SE becomes more refractory to treatment over time, appropriate medication is important. This study aimed to investigate efficacy and tolerability of intravenous (IV) lacosamide (LCM) in treatment of SC and SE. Data of patients with SE or SC who were treated with IV LCM between December 2009 and February 2011 in two Austrian centers were analyzed retrospectively. Clinical information was extracted from patients' charts. Forty-eight patients (26f/22m) aged median 62 years (range 17-95 years) were identified. Thirty-five percent of patients (17 of 48) had SC and 65% (31 of 48) had SE. SE was nonconvulsive in 10 (32%), convulsive in 11 (36%), and focal in 10 (32%) patients. SE was acute symptomatic in six (20%) and remote symptomatic in 11 (35%) patients. Fourteen (45%) had preexisting epilepsy. Median initial bolus dose was 200 mg (range 200-400 mg) in patients with SE and 200 mg in patients with SC. Maximum infusion rate was 60 mg/min. Cessation was observed in 42 patients (88%). Success rate in patients with SE receiving LCM as first or second drug was 100% (8 of 8), as third drug 81% (11 of 15), and as fourth or later drug 75% (6 of 8). There were no side effects observed except for pruritus and skin rash in two patients. These data support use of IV LCM as a potential alternative to standard antiepileptic drugs for acute treatment of seizure emergency situations, although randomized controlled studies are needed.

BACKGROUND: Salzburg Consensus Criteria for diagnosis of Non-Convulsive Status Epilepticus (SCNC) were proposed at the 4th London-Innsbruck Colloquium on status epilepticus in Salzburg (2013).METHODS: We retrospectively analyzed the EEGs of 50 consecutive nonhypoxic patients with diagnoses of nonconvulsive status epilepticus (NCSE) at discharge and 50 consecutive controls with abnormal EEGs in a large university hospital in Austria. We implemented the American Clinical Neurophysiology Society's Standardized Critical Care EEG Terminology, 2012 version (ACNS criteria) to increase the test performance of SCNC. In patients without preexisting epileptic encephalopathy, the following criteria were applied: (1) more than 25 epileptiform discharges (ED) per 10-second epoch, i.e., >2.5/s and (2) patients with EDs ≤ 2.5/s or rhythmic delta/theta activity (RDT) exceeding 0.5/s AND at least one of the additional criteria: (2a) clinical and EEG improvements from antiepileptic drugs (AEDs), (2b) subtle clinical phenomena, or (2c) typical spatiotemporal evolution. In case of fluctuation without evolution or EEG improvement without clinical improvement, "possible NCSE" was diagnosed. For identification of RDT, the following criteria were compared: (test condition A) continuous delta-theta activity without further rules, (B) ACNS criterion for rhythmic delta activity (RDA), and (C) ACNS criteria for RDA and fluctuation.RESULTS: False positive rate in controls dropped from 28% (condition A) to 2% (B) (p = 0.00039) and finally to 0% (C) (p = 0.000042). Application of test condition C in the group with NCSE gives one false negative (2%). Various EEG patterns were found in patients with NCSE: (1) 8.2%, (2a) 2%, (2b) 12.2%, and (2c) 32.7%. Possible NCSE was diagnosed based on fluctuations in 57.1% and EEG improvement without clinical improvement in 14.2%.CONCLUSION: The modified SCNC with refined definitions including the ACNS terminology leads to clinically relevant and statistically significant reduction of false positive diagnoses of NCSE and to minimal loss in sensitivity. This article is part of a Special Issue entitled "Status Epilepticus".

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”.

Introduction Identifying seizures with prolonged duration during video-electroencephalographic (EEG) monitoring is of importance to inform clinicians when to start emergency treatment of seizures to prevent status epilepticus. The aims of this study were to assess the clinical and EEG seizure duration (SD) in consecutive patients with epilepsy who underwent prolonged video-EEG monitoring and to identify a seizure type-dependent time point to start emergency treatment based on the likelihood that seizures will not stop spontaneously. Furthermore, we sought to determine predictors of SD and explored the relationship between antiepileptic drug (AED) serum levels and SD. Material and methods We retrospectively analyzed 1796 seizures in 200 patients undergoing video-EEG monitoring between January 2006 and March 2008. Results Focal simple seizures lasted significantly shorter (clinical SD: 28 s, EEG SD: 42 s) compared with focal complex seizures (clinical SD: 64 s, EEG SD: 62 s), and both seizure types lasted significantly shorter compared with secondarily generalized tonic–clonic seizures (GTCSs; clinical SD: 90 s, EEG SD: 96 s). There was no difference between the duration of the convulsive phase of primary GTCSs (defined as nonfocal) and that of secondarily GTCSs (each 65 s). Cumulative clinical SD (99%) was 7 min in focal complex seizures and 11 min in focal simple seizures. Mixed linear regression model demonstrated that history of status epilepticus ( P = 0.034), temporal lobe seizure onset ( P = 0.040), and MRI lesions ( P = 0.013) were significantly associated with logarithmic EEG SD in focal epilepsies recorded with scalp electrodes. We found significant negative correlations between the AED serum level and the EEG SD in patients treated with monotherapy: carbamazepine ( P P = 0.001), oxcarbazepine ( P = 0.001), and valproic acid ( P = 0.038) but not with lamotrigine monotherapy and EEG SD. Discussion Based on the results of this study, we propose 2 min of convulsive seizure activity (irrespective of focal or generalized onset) as a prolonged seizure, which could serve as a time point to consider treatment to prevent status epilepticus. In focal complex seizures, we suggest an upper limit of 7 min, and in focal simple seizures 11 min, as definition of prolonged seizures. History of status epilepticus, temporal seizure onset, and lesional MRI findings are factors associated with significantly longer SD. Negative correlations of carbamazepine, levetiracetam, oxcarbazepine, and valproic acid serum levels and SD suggest a prolonging effect on seizures during withdrawal of these AEDs during video-EEG monitoring sessions. This article is part of a Special Issue entitled “Status Epilepticus”.

Objective Selective serotonin reuptake inhibitors (SSRIs) are often used in the treatment of depressive disorders in patients with epilepsy. Pro- and anti-convulsive effects of SSRIs are discussed controversially. The aim of this study was to investigate a possible impact of SSRIs-treatment on duration of EEG and clinical features in epilepsy patients. Methods We studied video-EEG data from 162 patients with focal epilepsies between January 2006 and March 2008 using a case–control study design. Eleven patients with 19 complex focal seizures (CFSs) and 16 secondary generalized tonic-clonic seizures (sGTCSs) treated with SSRIs (SSRIs+) were matched to 13 patients without SSRIs-treatment (SSRIs−). We compared duration of ictal EEG in CFSs and sGTCSs, duration of convulsions in sGTCSs and duration of postictal EEG suppression after sGTCSs in SSRIs+ and SSRIs− patients. Results Ictal EEG duration of both, CFSs and sGTCSs, was significantly longer in SSRIs+ patients than in SSRIs− patients ( p = 0.004 and p = 0.015, respectively). No significant difference was found between convulsive phase duration of sGTCSs as well as duration of postictal EEG suppression after sGTCSs in both groups. Conclusion Seizures last significantly longer in patients with epilepsy and SSRIs as co-medication. A causative role of SSRIs in ictal activity has to be explored in prospective studies.

Status epilepticus (SE) is the most extreme form of epilepsy. It describes a prolonged seizure that may occur in patients with previous epilepsy or in acute disorders of the central nervous system. It is one of the most common neurologic emergencies, with an incidence of up to 41 per 100,000 per year and an estimated mortality is 20%. The three major determinants of prognosis are the duration of SE, patient age, and the underlying cause. Common and easily recognized causes of SE include cerebrovascular disorders, brain trauma, infections, and low antiepileptic drug levels in patients with epilepsy. Less common causes present a clinical and diagnostic challenge, but are major determinants of prognosis. Among them, inflammatory causes and inborn errors of metabolism have gained wide interest; recent insights into these causes have contributed to a better understanding of the pathophysiology of SE and its appropriate treatment. This review focuses on the different etiologies of SE and emphasizes the importance of prompt recognition and treatment of the underlying causes.

Status epilepticus (SE) and seizure clusters (SC) represent neurologic emergencies with a case fatality rate up to 34%, depending on cause and comorbidity. As SE becomes more refractory to treatment over time, appropriate medication is important. This study aimed to investigate efficacy and tolerability of intravenous (IV) lacosamide (LCM) in treatment of SC and SE. Data of patients with SE or SC who were treated with IV LCM between December 2009 and February 2011 in two Austrian centers were analyzed retrospectively. Clinical information was extracted from patients' charts. Forty-eight patients (26f/22m) aged median 62 years (range 17-95 years) were identified. Thirty-five percent of patients (17 of 48) had SC and 65% (31 of 48) had SE. SE was nonconvulsive in 10 (32%), convulsive in 11 (36%), and focal in 10 (32%) patients. SE was acute symptomatic in six (20%) and remote symptomatic in 11 (35%) patients. Fourteen (45%) had preexisting epilepsy. Median initial bolus dose was 200 mg (range 200-400 mg) in patients with SE and 200 mg in patients with SC. Maximum infusion rate was 60 mg/min. Cessation was observed in 42 patients (88%). Success rate in patients with SE receiving LCM as first or second drug was 100% (8 of 8), as third drug 81% (11 of 15), and as fourth or later drug 75% (6 of 8). There were no side effects observed except for pruritus and skin rash in two patients. These data support use of IV LCM as a potential alternative to standard antiepileptic drugs for acute treatment of seizure emergency situations, although randomized controlled studies are needed.

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

Summary Introduction Juvenile myoclonic epilepsy (JME) is a genetic generalized epilepsy syndrome. Under appropriate antiepileptic drugs (AED) up to 85% of patients become seizure-free, but many may have a relapse after AED withdrawal. Methods We retrospectively studied 242 patients with JME at the Department of Neurology, Medical University Innsbruck, Austria (1975–2006). We analyzed age at seizure onset, age at last follow up, seizure types, photosensitivity, seizure outcome and neuroimaging findings; inclusion criterion was a medical treatment period of >2 years; exclusion criteria were traumatic or infectious brain injury before the onset of JME and/or gross structural pathology on neuroimaging. Results We identified 175 patients (111 women) with a median age at seizure onset of 15 years, (range 3–46) and a median age at follow-up (FU) of 38 years (range 14–87; median FU 8 years, range 2–38). Fourteen percent showed (24/175) photosensitivity on routine EEG. Seizure outcome: 62% (109/175) were seizure-free of myoclonic seizures (MS), generalized tonic clonic seizures (GTCS) and absence seizures (AS) for >1 year, and 53% (94/175) for >2 years, including 16 patients (9%) without AEDs. Thirty-one percent (54/175) were seizure-free between 2and 5 years, 15% (26/175) between 6 and 10, and 8% (14/175) >10 years; 38% (66/175) were not seizure-free. Not seizure-free patients had more often MS, AS and GTCS within the first year of epilepsy than those who were seizure-free at last FU (11% vs. 3%, Chi 2 =4.679, df=1, p =0.043). Seizure-free patients had more often MS and GTCS as last seizure types in the year before becoming seizure-free (37% vs. 15%, p =0.003), whereas in not seizure-free group MS only and GTCS only persisted. Conclusions JME does not always need lifelong treatment, as a substantial minority of patients remain seizure-free without AEDs. AS, MS and GTCS at onset of the disease are indicators of poor long-term seizure control.

Status epilepticus (SE) represents the most severe form of epilepsy. It is one of the most common neurologic emergencies, with an incidence of up to 61 per 100,000 per year and an estimated mortality of 20 %. Clinically, tonic-clonic convulsive SE is divided into four subsequent stages: early, established, refractory, and super-refractory. Pharmacotherapy of status epilepticus, especially of its later stages, represents an "evidence-free zone," due to a lack of high-quality, controlled trials to inform clinical decisions. This comprehensive narrative review focuses on the pharmacotherapy of SE, presented according to the four-staged approach outlined above, and providing pharmacological properties and efficacy/safety data for each antiepileptic drug according to the strength of scientific evidence from the available literature. Data sources included MEDLINE and back-tracking of references in pertinent studies. Intravenous lorazepam or intramuscular midazolam effectively control early SE in approximately 63-73 % of patients. Despite a suboptimal safety profile, intravenous phenytoin or phenobarbital are widely used treatments for established SE; alternatives include valproate, levetiracetam, and lacosamide. Anesthetics are widely used in refractory and super-refractory SE, despite the current lack of trials in this field. Data on alternative treatments in the later stages are limited. Valproate and levetiracetam represent safe and effective alternatives to phenobarbital and phenytoin for treatment of established SE persisting despite first-line treatment with benzodiazepines. To date there are no class I data to support recommendations for most antiepileptic drugs for established, refractory, and super-refractory SE. Limiting the methodologic heterogeneity across studies is required and high-class randomized, controlled trials to inform clinicians about the best treatment in established and refractory status are needed.