Form Of Status Epilepticus Research Articles

Factors in the development of refractory status epilepticus in status epilepticus patients.

Status epilepticus (SE) is a severe neurological condition associated with a poor prognosis. Refractory status epilepticus (RSE) is a treatment-resistant form of SE with an even worse prognosis. The exact mechanisms underlying the development of RSE are not fully understood. The aim of this study was to investigate the factors contributing to the development of RSE in SE patients and to identify predictors of RSE occurrence. This retrospective study was conducted on patients diagnosed with SE and RSE between 2014 and 2024. Demographic information, comorbid conditions, and blood sample data of the patients were recorded for statistical analysis. The statistical analyses used included the Mann-Whitney U test, Chi-square test, Fisher's exact test, ROC curve analysis, and logistic regression. A total of 82 SE patients were included in the study. Of these, 44 were non-RSE patients (control group), and 38 were RSE patients. Significant differences were observed between the groups in terms of median age (p=0.001), blood glucose level (p=0.023), pan-immune inflammation value (PIV) (p=0.002), Monocyte/Lymphocyte Ratio (MLR) (p=0.009), Neutrophil/Albumin Ratio (NAR) (p=0.003), Systemic Immune Inflammation Index (SII) (p=0.013), Eosinophil/Lymphocyte Ratio (ELR) (p=0.016), Eosinophil/Neutrophil Ratio (ENR) (p=0.006), and Eosinophil/Monocyte Ratio (EMR) (p=0.002). The multivariate logistic regression model identified the presence of arterial hypertension as the only factor significantly associated with the development of RSE (p<0.001). In the ROC curve analysis, PIV (AUC=0.696) and NAR (AUC=0.689) were found to be predictive factors for RSE. The findings obtained in the current study suggest that systemic inflammation and arterial hypertension may be associated with the progression of SE to RSE. Further research is needed to confirm these findings and integrate them into routine clinical practice.

Status epilepticus and benzodiazepine treatment: Use, underdosing and outcome - insights from a retrospective, multicentre registry

To explore the reasons for and outcomes of non- or undertreatment with benzodiazepines (BZDs) in status epilepticus (SE). We retrospectively analysed all SE patients from the urban area of Cologne over two years. 328 SE patients were eligible, and only 72% were initially treated with BZDs. Of these, only 21.6% were treated sufficiently with BZDs according to current guidelines. SE patients not initially treated with BZDs were significantly older, had less often known epilepsy, had a prolonged arrival time to the emergency room, and presented more often with a non-generalised convulsive semiology. Regarding adequate dosages, patients with a generalised convulsive SE seemed to benefit from a sufficient BZD dosing with significantly shortened mean ventilation duration (37.1 to 208h), decreased mean intensive care unit (1.7 to 5 days) and in-hospital stay (4.1 to 8.8 days). In contrary, aggressive BZD treatment in non-generalised convulsive SE resulted in a longer inpatient stay (9.2 to 5.8 days) and lower favourable outcome rates at discharge (16% to 63%). The current SE treatment guidelines for first-line BZD therapy in SE were violated in most patients. Sufficient BZD dosing was beneficial in generalised convulsive SE, but not in other forms of SE. SE semiology might be crucial for treatment decisions with BZDs. Further treatment evidence especially in non-generalised convulsive SE is urgently needed.

Causes of New-Onset Seizures and Their Treatment in Children With Non-CNS Malignancies: A Retrospective Study in a Tertiary Care Center

Seizures occur in up to 13% of children with non-central nervous system (CNS) malignancies, but little is known about their causes and optimal diagnostic and therapeutic approaches. Here we sought to determine etiologies and clinical trajectories of new-onset seizures in this patient population. A retrospective chart review over a 10-year period was conducted at the American University of Beirut Medical Center to identify children with non-CNS malignancies and at least one new-onset seizure. Data were collected on the underlying malignancy, seizure etiology, clinical course, treatments, electroencephalograms, and brain imaging. New-onset seizures occurred in 56 children (2-year median follow-up), most commonly in the context of acute lymphoblastic leukemia, lymphomas, and sarcomas. In 19 children, the first seizure consisted of status epilepticus. The most common etiologies were cerebrovascular accidents, posterior reversible encephalopathy syndrome, and metastasis. Forty-nine patients received anti-seizure medications (ASMs). Withdrawal of ASMs was successful in 19 children with normal initial or follow-up brain imaging but failed in three patients with persistent brain lesions. The remaining children, all of whom except two had structural brain abnormalities, received chronic ASMs and remained seizure free for a median period of 2years at the last follow-up in survivors. Not only are seizures in children with non-CNS cancers often indicative of a serious brain insult, but they can also be challenging in the form of status epilepticus. An urgent diagnostic evaluation is therefore needed to expedite treatment, which should be tailored to the chronicity of the underlying cause.

Prognostic effects of treatment protocols for febrile convulsive status epilepticus in children

BackgroundFebrile status epilepticus is the most common form of status epilepticus in children. No previous reports compare the effectiveness of treatment strategies using fosphenytoin (fPHT) or phenobarbital (PB) and those using anesthetics as second-line anti-seizure medication for benzodiazepine-resistant convulsive status epilepticus (CSE). We aimed to examine the outcomes of various treatment strategies for febrile convulsive status epilepticus (FCSE) in a real-world setting while comparing the effects of different treatment protocols and their presence or absence.MethodsThis was a single-center historical cohort study that was divided into three periods. Patients who presented with febrile convulsive status epilepticus for ≥60 min even after the administration of at least one anticonvulsant were included. During period I (October 2002–December 2006), treatment was performed at the discretion of the attending physician, without a protocol. During period II (January 2007–February 2013), barbiturate coma therapy (BCT) was indicated for FCSE resistant to benzodiazepines. During period III (March 2013–April 2016), BCT was indicated for FCSE resistant to fPHT or PB.ResultsThe rate of electroencephalogram monitoring was lower in period I than period II+III (11.5% vs. 85.7%, p<0.01). Midazolam was administered by continuous infusion more often in period I than period II+III (84.6% vs. 25.0%, p<0.01), whereas fPHT was administered less often in period I than period II+III (0% vs. 27.4%, p<0.01). The rate of poor outcome, which was determined using the Pediatric Cerebral Performance Category scale, was higher in period I than period II+III (23.1% vs. 7.1%, p=0.03). The rate of poor outcome did not differ between periods II and III (4.2% vs. 11.1%, p=0.40).ConclusionsWhile the presence of a treatment protocol for FCSE in children may improve outcomes, a treatment protocol using fPHT or PB may not be associated with better outcomes.

Neuron Specific Enolase, S100-beta protein and progranulin as diagnostic biomarkers of status epilepticus.

Status epilepticus (SE) is a life-threatening prolonged epileptic seizure. A rapid diagnosis is fundamental to initiate antiepileptic treatment and to prevent the development of neurological sequels. Several serum and cerebrospinal fluid biomarkers have been proposed to help in the diagnosis of SE. Nevertheless, previous studies were conducted on too small patient cohorts, precluding the utilization of interesting biomarkers for the SE diagnosis. Here, we aimed to assess the ability of Neuron Specific Enolase (NSE), S100-beta protein (S100B) and progranulin to help in the diagnosis of SE in a large cohort of patients (36 control patients, 56 patients with pharmacoresistant epilepsy and 82 SE patients). Blood NSE, S100B and progranulin levels were higher in SE patients when compared with control patients or patients with pharmacoresistant epilepsy. Both NSE and progranulin levels were higher in cerebrospinal fluid from SE patients when compared with control patients. The receiver-operating characteristics curves revealed good accuracy at detecting SE for serum S100B (AUC 0.748) and plasma progranulin (AUC 0.756). The performances were lower for serum NSE (AUC 0.624). Eighty-four percent of patients with serum S100B levels above 0.09ng/mL presented with a SE, whereas 90% of patients without SE had serum S100B levels lower than 0.09ng/mL. Serum S100B levels were not significantly different according to SE etiology, SE semiology or SE refractoriness. Our results confirm that NSE, S100B and progranulin levels are increased after SE. We suggest that serum S100B levels might be added to clinical evaluation and electroencephalogram to identify difficult-to-diagnose form of SE.

Pharmacotherapy and intensive care aspects of status epilepticus: update 2020/2021

Die gezielte Therapie epileptischer Ereignisse und im Speziellen des Status epilepticus (SE) setzt das sichere Erkennen der Krankheitsbilder voraus, wofür gerade bei Formen mit vorwiegend nichtmotorischen Symptomen klinische und elektroenzephalographische Expertise notwendig ist. Die im Jahr 2020 erfolgte Fortschreibung der deutschen Leitlinie zur Behandlung des SE hält an der streng stufengerechten Therapie fest, die eskalierend die Anwendung von Benzodiazepinen, spezifischen Antiepileptika und Anästhetika vorsieht. Bisher ist die Eingrenzung eines in den allermeisten Fällen wirksamen sowie zugleich sicheren und interaktionsfreien Antiepileptikums nicht gelungen. Individuelle Vorerkrankungen und aktuelle Begleitumstände gehen daher genauso wie Erfahrungen des Behandlerteams in die differenzierte Behandlung des SE ein. Insbesondere bei therapierefraktären Formen des SE erweist sich die Therapie als durchaus kompliziert und hat regelhaft intensivmedizinische Implikationen. Mithin ergeben sich im Zuge der modernen SE-Behandlung zahlreiche interdisziplinäre Schnittstellen. Zukünftige wissenschaftliche Fragstellungen werden sich u. a. mit der optimalen Therapie des nonkonvulsiven SE und hier v. a. dem Ausmaß und dem Zeitpunkt von adäquaten Therapieschritten sowie mit assoziierten ethischen Fragen einer Therapieeskalation beschäftigen.

“Struggling status” as a form of status epilepticus in an infant with hypothalamic hamartoma: A case report with video-electroencephalogram

Hypothalamic hamartoma (HH) is a rare congenital brain malformation that leads to intractable epilepsy. Gelastic seizures (GS) are a hallmark of HH-related epilepsy, but development of other seizure types is possible (Supplementary table). In childhood, epilepsy usually begins with GS, but the seizure duration tends to be shorter than in adults [1]. Herein, we report video-electroencephalogram (EEG) findings of a female infant with HH with long-lasting unique seizure semiology.

Status epilepticus admissions during the COVID-19 pandemic in Salzburg-A population-based study.

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.

A Case of Myoclonic Epilepsy Presenting with Status Epilepticus in an Elderly Male Patient.

Myoclonic epilepsy in the form of status epilepticus is an extremely rare reported presentation. Herein, we describe an 87-year old male patient presenting with abrupt-onset rhythmic myoclonic jerks that were evaluated as synchronous positive and negative myoclonus. Further etiological investigations revealed that the myoclonus was associated with ictogenesis, and appropriate antiepileptic treatment provided total cessation of the movements. To my knowledge, this is a unique case of myoclonic status epilepticus in an elderly patient, which may have been associated with a cerebrovascular disease. In the presentation of this case, we will review the related literature and discuss some considerations to explain the pathophysiology of epileptic myoclonic movements and the possible role of pontine lesions.

Prognostic Significance of Cyclic Seizures in Status Epilepticus.

Status epilepticus (SE) is a commonly encountered neurologic condition associated with high mortality rates. Cyclic seizures (CS) are a common form of SE, but its prognostic significance has not been well established. In this retrospective study, the mortality of cyclic versus noncyclic forms (NCSs) of SE are compared. A total of 271 patients were identified as having seizures or SE on EEG reports, of which 65 patients were confirmed as having SE. Based on EEG characteristics, the patients were then classified as cyclic or noncyclic patterns. Cyclic seizures were defined as recurrent seizures occurring at nearly regular and uniform intervals. Noncyclic form included all other patterns of SE. Pertinent clinical data were collected and reviewed for each case. Of the 65 patients with SE, 25 patients had CS and 40 patients had NCS. Patients with CS showed a lower rate of in-hospital mortality although not statistically significant (P = 0.19). When looking at patients younger than 75 years, the CS group had significantly lower in-hospital mortality rate (P = 0.007). The findings of this study suggest that CS may have a more favorable outcome compared with NCS in patients younger than 75 years. This study is also the first to report the rate of CS among all cases of confirmed SE (38%). Future studies with a larger sample size are needed to further evaluate the difference in outcome between CS and NCS.

Status epilepticus in patients with genetic (idiopathic) generalized epilepsy.

Aim of the studyGenetic (idiopathic) generalized epilepsies (GGEs) account for nearly one-third of all epilepsies. The frequency of status epilepticus (SE) in patients with GGEs has been poorly studied. Therefore, this study aimed to evaluate the frequency of different forms of SE in a cohort of patients with GGEs.Materials and methodsAmong 153 patients with GGEs treated at the university epilepsy clinic in the period between 1998 and 2018, those with SE were retrospectively identified.ResultsAbsence SE was diagnosed in 8 patients (13 episodes), while myoclonic SE was found in 2 patients (2 episodes). No cases of tonic–clonic SE were detected in the study cohort. Most SE episodes were found to be provoked by ill-advised antiepileptic drugs or changes in drug regimen. In all the subjects, SE was stopped by intravenous administration of diazepam and/or valproate. Long-term outcome of epilepsy was good, with most patients (70%) being seizure-free.ConclusionStatus epilepticus is not a rare phenomenon in patients with genetic generalized epilepsies, with absence SE being the most common type. Most cases of SE are provoked by ill-advised AEDs or changes in drug regimen. Status epilepticus in GGEs can be easily treated with benozdiazepines and/or valproate. Status epilepticus in GGEs can be easily treated with benozdiazepines and/or valproate.

Cyclic seizures in critically ill patients: Clinical correlates, DC recordings and outcomes

To describe EEG and clinical correlates, DC recordings and prognostic significance of cyclic seizures (CS). We reviewed our prospective continuous EEG database to identify patients with CS, controls with non-cyclic status epilepticus (SE) and controls without seizure matched for age and etiology. EEG was reviewed with DC settings. 39/260 (15%) patients with electrographic seizures presented with CS. These patients were older (62 vs. 54years; p=0.04) and more often had acute or progressive brain injury (77% vs. 52%; p=0.03) than patients with non-cyclic SE and had a lower level of consciousness, were more severely ill, than matched controls. CS almost always had focal onset, often from posterior regions. Patients with CS trended towards worse prognosis. When available (12 patients), DC recordings showed an infraslow cyclic oscillation of EEG baseline synchronized to the seizures in all cases. CS occur mostly in older patients with acute or progressive brain injury, are more likely to be associated with poor outcome than patients with other forms of nonconvulsive SE, and are accompanied by synchronous oscillations of the EEG baseline on DC recordings. CS are a common form of non-convulsive status epilepticus in critically ill patients and provide further insights into the relationship between infraslow activity and seizures; further study on this relationship may shed light on the mechanisms of seizure initiation and termination.

Etiology and outcome of infants and children in a tertiary hospital in the Philippines with convulsive status epilepticus

Introduction: Among the paediatric neurological disorder, seizure ranks first. Status Epilepticus (SE) is a neurological emergency. It is associated with significant morbidity and mortality. The objectives of the presented study were to determine the clinical profile and outcome of status epilepticus in infant and children at a tertiary care hospital in Philippines. Methodology: It was a retrospective study conducted in Department of Pediatrics, National Children's Hospital, Quezon City, Philippines. All the patients in the age range 1 month to 18 years, admitted and diagnosed as cases of SE between January 2008 to December 2014 formed the sample size. Results: The seven-year average hospital based incidence of SE was 138.5 per 100,000 admissions. The most common underlying cause of SE was seizure disorder (29.89%), followed by bacterial meningitis (13.8%), and cerebral palsy (10.3%). Conclusion: Febrile SE is the common form of convulsive SE. There is high morbidity and mortality associated with SE.

A definition and classification of status epilepticus--Report of the ILAE Task Force on Classification of Status Epilepticus.

The Commission on Classification and Terminology and the Commission on Epidemiology of the International League Against Epilepsy (ILAE) have charged a Task Force to revise concepts, definition, and classification of status epilepticus (SE). The proposed new definition of SE is as follows: Status epilepticus is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally, prolonged seizures (after time point t1 ). It is a condition, which can have long-term consequences (after time point t2 ), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures. This definition is conceptual, with two operational dimensions: the first is the length of the seizure and the time point (t1 ) beyond which the seizure should be regarded as "continuous seizure activity." The second time point (t2 ) is the time of ongoing seizure activity after which there is a risk of long-term consequences. In the case of convulsive (tonic-clonic) SE, both time points (t1 at 5min and t2 at 30min) are based on animal experiments and clinical research. This evidence is incomplete, and there is furthermore considerable variation, so these time points should be considered as the best estimates currently available. Data are not yet available for other forms of SE, but as knowledge and understanding increase, time points can be defined for specific forms of SE based on scientific evidence and incorporated into the definition, without changing the underlying concepts. A new diagnostic classification system of SE is proposed, which will provide a framework for clinical diagnosis, investigation, and therapeutic approaches for each patient. There are four axes: (1) semiology; (2) etiology; (3) electroencephalography (EEG) correlates; and (4) age. Axis 1 (semiology) lists different forms of SE divided into those with prominent motor systems, those without prominent motor systems, and currently indeterminate conditions (such as acute confusional states with epileptiform EEG patterns). Axis 2 (etiology) is divided into subcategories of known and unknown causes. Axis 3 (EEG correlates) adopts the latest recommendations by consensus panels to use the following descriptors for the EEG: name of pattern, morphology, location, time-related features, modulation, and effect of intervention. Finally, axis 4 divides age groups into neonatal, infancy, childhood, adolescent and adulthood, and elderly.

P23 – 2520: Clinical or (and) genetic diagnosis of Dravet syndrome: Report of two cases

We report two boys, first aged 2.5 years and second aged 13 months. The first boy was hospitalized for the first time at the age of 8 mo due to generalized seizure. The other boy is now followed-up for 5 months, first time at the age of 8 mo had convulsive status epilepticus during fever. Brain MRI and interictal EEG in both children were normal. Other couses of seizures were excluded. The clinical progress in the first boy was marked by the development of focal seizures, with no unilateral domination, mainly in the form of status epilepticus triggered by fever. Ictal EEG showed paroxysmal brain activity correlated with clinical hemiconvulsions. He had pharmacoresistant epilepsy. Later, form of seizures has changed, with dominant atypical absence seizures. Psychomotor evaluation at the age of 2 years revealed mild deterioration. We performed genetic testing for Dravet syndrome. The mutation of SCN1A and SCN2 gene was negative. Analysis for GABRA1, GABRG2 and STXBP1 mutations is in progress. The clinical progress in second boy was marked by second status epilepticus during fever after we performed SCN1A gene mutation which is positive. The boy is now 13 months old, since now he had 6 episodes of status epilepticus, without other type of seizures. His interictal EEG and psychomotor development were normal. Clinical progress of the disease in the first boy is complient with Dravet syndrome and could be included among the 30% of patients without the mutation of SCN1A gene. Second boy has good clinical course after 5 mo follow-up, despite of the SCN1A gene mutation. Early diagnosis of Dravet syndrome is important for the avoiding unnecessary examinations and false therapies as well as for genetic counselling. In both children remains an open question of using specific and/or preventive therapy with stiripentol as well as long-term prognosis. We report two boys, first aged 2.5 years and second aged 13 months. The first boy was hospitalized for the first time at the age of 8 mo due to generalized seizure. The other boy is now followed-up for 5 months, first time at the age of 8 mo had convulsive status epilepticus during fever. Brain MRI and interictal EEG in both children were normal. Other couses of seizures were excluded. The clinical progress in the first boy was marked by the development of focal seizures, with no unilateral domination, mainly in the form of status epilepticus triggered by fever. Ictal EEG showed paroxysmal brain activity correlated with clinical hemiconvulsions. He had pharmacoresistant epilepsy. Later, form of seizures has changed, with dominant atypical absence seizures. Psychomotor evaluation at the age of 2 years revealed mild deterioration. We performed genetic testing for Dravet syndrome. The mutation of SCN1A and SCN2 gene was negative. Analysis for GABRA1, GABRG2 and STXBP1 mutations is in progress. The clinical progress in second boy was marked by second status epilepticus during fever after we performed SCN1A gene mutation which is positive. The boy is now 13 months old, since now he had 6 episodes of status epilepticus, without other type of seizures. His interictal EEG and psychomotor development were normal. Clinical progress of the disease in the first boy is complient with Dravet syndrome and could be included among the 30% of patients without the mutation of SCN1A gene. Second boy has good clinical course after 5 mo follow-up, despite of the SCN1A gene mutation. Early diagnosis of Dravet syndrome is important for the avoiding unnecessary examinations and false therapies as well as for genetic counselling. In both children remains an open question of using specific and/or preventive therapy with stiripentol as well as long-term prognosis.

Epidemiology-based mortality score in status epilepticus (EMSE).

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.

Efficacy and safety of intravenous valproate for status epilepticus: a systematic review.

IntroductionThe 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).AimOur 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.MethodsData sources included MEDLINE, back tracing of references in pertinent studies, and contact with the manufacturer of VPA (Sanofi-Aventis).ResultsOverall, 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 (<10 %), mainly dizziness, thrombocytopenia, and mild hypotension, which was independent of infusion rates. Of note, good cardiovascular and respiratory tolerability was observed in these studies, even at high doses and fast infusion rates (up to 30 mg/kg at 10 mg/kg/min), despite multiple morbidities or other antiepileptic drugs. The most serious concern relates to the possibility of acute encephalopathy, sometimes related to hepatic abnormalities or hyperammonemia.ConclusionsThe published experience is consistent with VPA being a safe and effective therapeutic option for patients with established SE who have previously failed conventional first-line treatment with benzodiazepines, but high-quality randomized controlled trials are needed to inform clinicians on its comparative effectiveness in SE.

Clinical experience with intravenous valproate as first-line treatment of status epilepticus and seizure clusters in selected populations

Objective: The aim of this study was to evaluate the efficacy and safety of intravenous valproate (i.v. VPA) as first-line treatment of status epilepticus (SE) and seizure clusters in selected patient populations. Methods: We enrolled 23 patients (11 females and 12 males; mean age: 61 years) with SE who received i.v. VPA as first-line therapy (25 mg/kg in 100 mL saline infused over 15 min). ECG tracing was monitored before, during, and after infusion. Liver function and serum ammonia tests were conducted after 24 and 72 h of treatment. We evaluate the response of SE to i.v. therapy and short-term outcome. Results: In 15 out of 23 patients (65%), i.v. VPA was effective. In our population, we retrospectively identified three different subgroups: patients with cardiorespiratory comorbidities discouraging the use of traditional SE first-line drugs, patients with specific epileptic subsyndromes (such as idiopathic generalized epilepsy), and patients affected by psycho-organic syndromes. No significant adverse effects were detected. Discussion: Our study shows the clinical relevance of i.v. VPA as first-line therapy of SE in patients with medical conditions contraindicating the use of traditional first-line antiepileptic drugs for SE, and in those presenting with specific forms of SE.

Predicting outcome in adults with status epilepticus

Status epilepticus (SE) is a life-threatening state of persisting or repetitive seizure activity with often permanent altered level of consciousness. Despite its high morbidity and mortality there is no consensus about the best strategy to treat specific forms of SE. The compromise between the danger related to untreated and persistent seizure activity and the possible damage induced by unnecessary aggressive treatments is challenging. Knowledge about the determinants and reliable prediction models of outcome early in the course of SE is fundamental for rapid treatment modulation and for planning the level of monitoring. This review compiles the current evidence for outcome prediction based on clinical determinants in adult SE patients.

Clinical and electroencephalographic polymorphism of malignant migrating partial seizures in infancy

Malignant migrating partial seizures in infancy (MMPSI) are a rare epileptic syndrome that occurs in the first 6 months of life and is characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns with involvement of different independent areas of both hemispheres and with arrested psychomotor development. Is proposed the definition of this epileptic syndrome as: «malignant epilepsy of infancy with migrating multifocal seizures» or «Coppola-Dulac syndrome». The paper describes an observation of 19 MMPSI patients examined and treated at Departament of Psychoneurology №2, Russian Children Clinical Hospital. Video-EEG monitoring showed that all the patients had very frequent and polymorphous seizures — at least 5 types in every child with ictal patterns originating from different areas of both hemispheres. The infants with MMPSI were found to have 4 subtypes of the syndrome: (1) a classic form with drug-resistant migrating status epilepticus (SE) of migrating multifocal seizures and with absolutely poor prognosis (n = 7); (2) a severe mixed form (MMPSI + early myoclonic encephalopathy (EME) with a combination of electroclinical characteristics of MMPSI with migrating multifocal SE and EME with chaotic erratic myoclonus and a suppression-burst pattern with diffuse polyspikewaves on EEG (n = 5); (3) a moderate type with reverse evolution to monofocal or multifocal epilepsy with a decrease in seizure frequency and better prognosis of life and psychomotor development (n = 5); (4) a subtle form manifesting itself as slightly identified minimal motor and inhibitory seizures, subclinical migrating multifocal SE pattern on EEG, multiple partial awakenings during sleep due to ictal patterns, and as severely delayed psychomotor development (n = 2). MMPSI is a severe form of SE in infancy with high drug resistance. At the same time, only the injection form of valproate (convulex) could stabilize progressive worsening and reduce seizure frequency in 3 cases of benzo-diazepine-resistant SE in MMPSI. Intravenous convulex is a rational alternative to benzodiazepines in SE in infancy, especially in cases of bulbar innervation disturbances and at high risk for depressed respiratory and cardiac functions.