Tratamiento quirúrgico en estado epiléptico super refractario relacionado con malformación arterio venosa

Introducción: Las malformaciones arteriovenosas cerebrales (MAV) son un conjunto anormal de arterias y venas dilatadas del cerebro y caracterizadas por la pérdida de la organización vascular con una derivación arteriovenosa anormal. La probabilidad de ruptura de una MAV es baja, pudiendo causar déficit hasta en el 45% de los pacientes. La epilepsia puede asociarse con MAV siendo raro el estado epiléptico. La evolución a estado epiléptico refractario o estado epiléptico super refractario (EESR) es excepcional. Se presenta paciente con epilepsia asociada a MAV, con embolización y posterior complicación que evoluciona a EESR, donde se logró una resolución favorable con cirugía. Caso clínico: Paciente masculino de 70 años, con MAV temporal derecha no rota y epilepsia, libre de crisis desde el diagnóstico de ambas condiciones. Se realizó embolización logrando exclusión, pero con evolución a EESR. Tras estudio se realiza resección de MAV y resección parcial de zona de inicio de actividad epileptógena parieto-temporal. Además, se realizó una desconexión de región fronto-parietal central donde había evidencia de propagación de la actividad epiléptica. Paciente logra salir de estado crítico, con recuperación de conciencia y normalización de electroencefalogramas posquirúrgicos. A los 2 años evoluciona con epilepsia controlada (Engel IIa). Discusión y conclusiones: El abordaje quirúrgico es una opción en EESR y debe plantearse en un tiempo precoz de evolución (1-2 semanas). La cirugía se realizó para controlar EESR y fue una intervención exitosa. Esta estrategia puede lograr un cambio dramático en el pronóstico. La cirugía resectiva está indicada cuando hay evidencia etiológica en imágenes y focalidad eléctrica consistente en los estudios.

Introduction: Brain Arteriovenous Malformations (AVM) are an abnormal set of dilated arteries and veins within the brain and are characterized by loss of vascular organization with an abnormal arteriovenous shunt. The probability of AVM rupture is low, but it can cause deficits in up to 45% ofpatients. Epilepsy could be associated with AVM, and status epilepticus is rare. The evolution to refractory status epilepticus or super refractory status epilepticus (SRSE) is very rare. The objective is to present a patient with epilepsy associated with non-ruptured AVM, treated with embolization and a subsequent complication that evolves into SRSE, in which a favorable resolution was achieved after surgery Clinical case: 70-year-old male, with a diagnosis of non-ruptured right temporal AVM and epilepsy, who has been seizure free since the diagnosis, is reported. Embolization of the AVM was performed, achieving total exclusion, after the procedure presented seizures that evolved into SRSE. After investigation, an AVM resection and partial resection of the ictal onset zone on the right parieto-temporal region was performed, including a disconnection of the central fronto-parietal region, where there was evidence of propagation of ictal activity. Patient recovered from the critical condition after surgery, and also presented electrographic normalization. After 2 years, the epilepsy is well controlled (Engel IIa). Discussion and conclusions: The surgical approach is an option in SRSE, and it should be proposed early on, within a reasonable time of evolution (1-2 weeks). The active approach in this case, where the surgery was done for SRSE control, was a successful intervention. Especially when the features are consistent -there is etiological evidence in imaging and electrical focality in studies- there can be a dramatic change in the prognosis.

Super refractory status epilepticus in a parturient with preeclampsia undergoing cesarean delivery under spinal anesthesia

Super refractory status epilepticus (SRSE) is a rare but serious neurological emergency. This case report aims to highlight the challenges faced during the diagnosis and management of SRSE in a 36-year-old preeclamptic patient taken for cesarean delivery under spinal anesthesia. The patient developed a seizure episode minutes after administration of intrathecal bupivacaine, necessitating administration of general anesthesia. Postoperatively, SRSE developed which did not respond to multiple anti-epileptics and thiopentone sodium infusion. Although the clinical picture and magnetic resonance imaging findings supported the diagnosis of posterior reversible encephalopathy syndrome, the role of intrathecal bupivacaine in causing SRSE could not be ruled out due to the temporal association of events. The patient had a prolonged and complicated hospital stay and despite a multimodal approach to the treatment, suffered neurological sequelae.

Application of 18F-flurodeoxyglucose positron emission tomography in patients with status epilepticus / 中华神经科杂志

Status epilepticus is a neurological emergency with unknown pathogenesis, controversial treatment options, and poor prognosis. In recent years, with the development of 18F-flurodeoxyglucose positron emission tomography ( 18F-FDG PET) imaging technology, further studies on status epilepticus have been carried out from the perspective of molecular metabolism. This article reviews the current role of 18F-FDG PET in patients with status epilepticus on etiology, disease activity, electric activity, location of epileptogenic foci, and prognosis evaluation.

Anti-NMDA-receptor encephalitis in Filipino adults: Case series and outcomes in a tertiary government hospital in the Philippines

INTRODUCTION@#We performed a case series of all five (5) confirmed adult Filipino cases of Anti-N-Methyl-D-Aspartate receptor (anti-NMDA-R) encephalitis in a tertiary government hospital in the Philippines admitted in the past three years. Two cases were identified with unique features: (1) a 23-year old female who presented with combined refractory seizures and persistent chorea and orofacial dyskinesias; and (2) a 22-year old male who presented with refractory epilepsia partialis continuua. The rest of the patients were hereby presented.@*BACKGROUND@#In the past years, anti-NMDA-R encephalitis has been considered a diagnosis of exclusion in lieu of other infectious causes of encephalitis. It is rare and an emerging disease with an incidence estimated at approximately 2-3 cases per million. Recent literature recorded severe cases of anti-NMDA-R encephalitis that presented as intractable first onset seizures, combined with hyperkinetic movement disorders, acute psychosis without a premorbid condition, and dysautonomia. @*OBJECTIVES@#To present the clinicodemographic profile and to discuss the management and outcomes of patients with anti-NMDAR encephalitis in a tertiary hospital in the Philippines.@*RESULTS@#Here, we report five confirmed cases of anti-NMDA-R encephalitis admitted in 2019-2021. The mean age is 23 years old, with 4:1 female to male ratio with a median length of hospitalization of 58 days. All patients presented with acute psychiatric symptoms without premorbid condition, focal and generalized seizures, decreased consciousness, dyskinesias, and autonomic instability. Four patients needed airway support for central hypoventilation, one had first onset seizure that developed into refractory epilepsia partialis continuua, one had persistent chorea and orofacial dyskinesia. Imaging studies of the brain included contrast-enhanced CT Scan and MRI with unremarkable findings. No female patients had an ovarian teratoma as revealed in the whole abdominal ultrasound. All CSF analysis for anti-NMDA-receptor was done in the same laboratory outside the hospital which revealed positive for NMDA-receptor antibodies, while CSF lymphocytic pleocytosis was only seen in 1/5 and protein elevation in 4/5. All of the patients underwent electroencephalogram (EEG) studies which revealed diffuse delta-theta slowing without epileptiform discharges. The patient who had persistent chorea and orofacial dyskinesias showed extreme delta brush, while one had normal EEG findings. They all received high-dose steroid and intravenous Immunoglobulin (IVIg); three patients were able to undergo Rituximab infusion. Only one female patient had mild deficits, one female was discharged fully functional and ambulatory from being weaned off from the mechanical ventilator, one female had aborted cardiac arrest and was discharged bedridden at GCS 10, and two died due to the other concomitant medical conditions. The Modified Rankin Scale (MRS) and Mini-mental Status Examination (MMSE) were used to assess the neurological and functional outcomes of our patients. @*CONCLUSION@#Anti-NMDA-R encephalitis is an emerging neurological disorder that warrants early identification as it impacts timeliness of management and long-term outcomes.

Optimizing seizure detection by quantitative EEG in paediatric refractory status epilepticus

@#Background & Objective: Continuous electroencephalography (cEEG) is valuable in the diagnosis and management of refractory status epilepticus (RSE) but requires intensive skilled interpretation. Density spectral array (DSA) is a quantitative analytic tool used to screen cEEG recordings for seizures. This study aims to determine the optimal amplitude setting and to compare the use of single-averagetrendgraph display and eight-trendgraph display in seizure detection with DSA. Methods: Five excerpts from pediatric cEEG recordings with RSE were identified. In Phase 1 of the study, each of 4 readers determined the DSA amplitude setting one most preferred for each excerpt, and marked all seizures in one excerpt using one’s preferred setting. Inter-rater agreement in seizure detection was measured. In Phase 2, readers marked all seizures in all excerpts, first using single-average-trendgraph display, and then using eight-trendgraph display after a wash-out period. Intra-rater agreement in seizure detection between the two display methods was calculated. Results: In Phase 1, DSA readers’ choice of preferred amplitude settings varied widely but inter-rater agreement in seizure detection was high. In Phase 2, seizure detection using single- and eight-trendgraph displays showed high agreement with each other and, where they disagreed, single-average-trendgraph was more sensitive. Additionally, low seizure-to-background amplitude ratio in EEG recordings was associated with worse detection sensitivity/specificity. Conclusions: DSA amplitude settings do not affect seizure detection. Single-trendgraph display is comparable to eight-trendgraph display in screening cEEG for seizures. Seizure detection with DSA performs better in cEEG recordings with high seizure-to-background amplitude ratio.

Estado epiléptico refractario en UCIP. Experiencia en un hospital de la Provincia de Buenos Aires / Refractory status epilepticus in the PICU. Experience at a hospital in the Province of Buenos Aires

Introducción: El estado epiléptico (EE) es la emergencia neurológica más frecuente en pediatría. Los pacientes que no responden al tratamiento estándar con dosis adecuadas de benzodiacepinas seguido de una droga antiepiléptica aceptable son definidos como Estado epiléptico Refractario (ER). Objetivo: caracterizar la población de niños con EE que ingresan a UCIP y determinar qué factores son predictores de refractariedad en esta población. Métodos: Estudio de casos y controles, retrospectivo. Población: niños con EE internados en UCIP desde Febrero 2015 a Febrero 2017. Casos: Estado epiléptico Refractario (ER). Controles: Estado epiléptico No Refractario (ENR). Se calculó el Odds Ratio (OR) individual para las distintas variables en Med Calc. Resultados: Se internaron 35 pacientes de los cuales 12 fueron casos y 23 controles. Hubo fiebre en 77% de los pacientes. En el total de niños estudiados hubo 11% con antecedente de convulsión febril, 11% con antecedente de epilepsia y 9% con antecedente de malformación del SNC. Los niños con antecedente de convulsión febril tuvieron 2,5 veces mayor riesgo de ER (OR: 2,58; IC 95%: 1,17-5,68). Los niños con EE que tenían antecedentes de enfermedad neurológica previa presentaron riesgo de ER 2,6 veces mayor que el grupo control (OR 2,60; IC 95%: 1,24-5,42). Discusión: Dado el aumento en la mortalidad de los pacientes con ER sería importante disponer de más herramientas para predecir este desenlace e iniciar tratamiento oportuno. Resultaría útil entrenar a los padres de niños con antecedente de convulsión febril en la aplicación de medicación antiepiléptica prehospitalaria, esto podría prevenir la farmacorresistencia, el daño neurológico y las complicaciones que acarrea el ingreso a UCIP. (AU)

Introduction: Status epilepticus (SE) is the most common neurologic emergency in children. Patients that do not respond to standard treatment with adequate doses of benzodiazepines followed by an acceptable antiepileptic drug are defined as having refractory status epilepticus (RSE). Objective: To characterize the population of children with SE admitted to the PICU and to determine predictive factors for refractoriness in this population. Methods: A retrospective case-control study was conducted. Population: Children with SE admitted to the PICU between February 2015 and February 2017. Cases: Refractory status pilepticus (RSE). Controls: Non-refractory status epilepticus (NRSE). Individual Odds Ratio (OR) was calculated for different variables using Med Calc. Results: 35 patients were admitted of whom 12 were cases and 23 controls. Overall, 77% of the patients had fever. Of all the children, 11% had a history of febrile seizures, 11% had history of epilepsy and 9% had a CNS malformation. Children with a history of febrile seizures had a 2.5-fold higher risk of developing RSE (OR: 2.58; 95% CI: 1.17-5.68). Children with SE that had a history of neurologic disease had a 2.6-fold higher risk of developing RSE than controls (OR 2.60; 95% CI: 1.24-5.42). Discussion: Given the increased mortality in children with RSE, availability of tools to predict this outcome in order to initiate early treatment is important. It would be useful to train the parents of children with a history of febrile seizures in the prehospital administration of antiepileptic drugs as this may prevent pharmaco-resistance, neurologic damage, and complication related to PICU admission (AU)

Consensus definition and development of new-onset refractory status epilepticus and the related syndromes / 中华实用儿科临床杂志

New-onset refractory status epilepticus(NORSE)is a clinical syndrome,not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue,toxic or metabolic cause. Febrile in-fection-related epilepsy syndrome(FIRES)is a subcategory of NORSE,applicable for all ages,occurring in a prior fe-brile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus( RSE)with fever or not. Proposed consensus definitions are also applicable for infantile hemiconvulsion - hemiplegia and epilepsy syn-drome,RSE,super-RSE,prolonged RSE and prolonged super-RSE. These consensus definitions will promote much more effective communication and multicenter research,and ultimately improve recognition and treatment of these condi-tions.

Consensus definition and development of new-onset refractory status epilepticus and the related syndromes / 中华实用儿科临床杂志

New-onset refractory status epilepticus(NORSE) is a clinical syndrome, not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue, toxic or metabolic cause.Febrile infection-related epilepsy syndrome(FIRES) is a subcategory of NORSE, applicable for all ages, occurring in a prior febrile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus(RSE) with fever or not.Proposed consensus definitions are also applicable for infantile hemiconvulsion-hemiplegia and epilepsy syndrome, RSE, super-RSE, prolonged RSE and prolonged super-RSE.These consensus definitions will promote much more effective communication and multicenter research, and ultimately improve recognition and treatment of these conditions.

Refractory and super refractory status epilepticus in the Philippines: A 10-year retrospective study

PURPOSE@#Super refractory status epilepticus (SRSE) is an uncommon, but important clinical problem with high morbidity and mortality. Studies concerning SRSE has been limited and there are no existing Philippine data. The study aims to determine the status of Refractory (RSE) and SRSE within a 10-year period in a Tertiary Hospital.@*METHODS@#This is a retrospective study of adult patients with prolonged seizures admitted at The Medical City, Philippines from January 2009- July 2018. Multinomial Logistic Regression was used to assess probability of good or poor outcome. Significant Correlation is defined by P value of <0.05. @*RESULTS@#The Incidence of RSE is as high as 38% (n=64) and 35% (n=58) for SRSE. Mortality rate is 39.1% in RSE and 62.1% in SRSE. Poor functional outcome is observed in RSE and SRSE wherein the majority was Alive Dependent. Significant factor increasing likelihood of being Alive Dependent is the absence of Arrest. @*CONCLUSION@#Factors associated with likelihood of being alive and independent includes Status Epilepticus and younger age therefore aggressive seizure control to prevent progression to SRSE will give higher likelihood of good functional outcome and elderly patients warrant closer and more adept seizure control for better functional outcome.

Experience in rescue and treatment of patients with super-refractory status epilepticus and coma / 中国中西医结合急救杂志

Objective To investigate the efficacy and prognosis of using midazolam combined with atracurium for treatment of a patient with super-refractory status epilepticus (SRSE) and coma. Methods A case of SRSE and coma admitted to the Department of Critical Care Medicine of Renmin Hospital of Wuhan University in May 2018 was enrolled. The symptoms, signs, laboratory examinations and treatment process were summarized, and combined with the literature review, the therapeutic course and prognosis were analyzed. Results A male patient, 24 years old, had convulsions after 4-day fever, blood routine, blood biochemistry, coagulation function and infection indexes were basically normal on admission. There was no obvious abnormality in cerebrospinal fluid examination; blood pathogenic microorganism examination suggested that viral infection possibly occur. Electroencephalogram (EEG) showed that the lesions involved the right frontal pole-frontal area-temporal area; cranial magnetic resonance imaging (MRI) suggested that sinusitis and meningeal and hippocampal infections might be present. Although several antiepileptic drugs had been used, the convulsions could not be controlled, after combined with the application of midazolam and atracurium, the convulsions were controlled, in the mean time, antiviral drug, glucocorticoid and immunoglobulin and therapies for prevention of complications were applied. Forty-three days later, the patient's consciousness was recovered and muscle strength was gradually improved. Conclusion The combination of midazolam and atracurium is effective for controlling epileptic activities and alleviating sequelae in patients with SRSE and coma.

Early immunotherapy in cryptogenic new onset refractory status epilepticus (NORSE), a case series

@#To date, there are no clear guidelines for treatment of cryptogenic new onset refractory status epilepticus (NORSE) syndrome. Immunotherapy was effective for some patients, but the initiation time was often somewhat late. We experienced two cryptogenic NORSE syndrome patients with favorable outcomes with early immunotherapy. A 59 years old male patient and a 58 years old female patient visited our hospital in status epilepticus, who were later diagnosed as cryptogenic NORSE after full evaluation. Since seizures were not controlled by two or more antiepileptic drugs in both patients, immunotherapy began on the fifth day of hospitalization. There was improvement on EEG and clinical symptoms, one or two days after initiation of immunotherapy. Early diagnosis of NORSE and early immunotherapy may result in favorable outcome.

Estatus epiléptico. Factores asociados a una evolución desfavorable en un centro terciario / Status Epilepticus. Prognostic Variables for an Unfavorable Outcome in a tertiary center of care

Resumen Introducción: El Estatus Epiléptico (EE) es una causa frecuente de emergencia neurológica. Escasos son los estudios realizados en Suramérica para evaluar los factores pronósticos de mortalidad y discapacidad en pacientes con EE. Objetivo: Determinar las variables asociadas a una evolución desfavorable (ED) al alta hospitalaria en pacientes con EE. Método: Se realizó un estudio retrospectivo durante el periodo enero 2016-junio 2017. Un total de 26 pacientes fueron diagnosticados con EE en sus diferentes variantes. Se evaluó el efecto de las variables clínicas, radiológicas y electroencefalográficas sobre la evolución hospitalaria final acorde la escala de Rankin. Resultados: 12 (46,2%) de los pacientes tuvieron una ED al alta hospitalaria, con una mortalidad que alcanzó el 23%. Existió un predominio del sexo masculino (76,9%). Las variables asociadas de forma independiente con una ED fueron el número de comorbilidades (p=0.01, OR: 4.27-95%CI1.33-13.6), lesiones en la imagen por Resonancia Magnética (IRM) (p=0.04, OR: 3.92-95%CI1.05-14.61) y el EE refractario (p=0.01, OR: 12.52-95%CI1.85-84.44), y la edad (p=0.07 OR: 1.03-95%CI0.99-1.07). Mientras que, un buen estado clínico inicial acorde la escala de Glasgow fue un factor protector (p=0.00 OR: 0.49-IC95%0.29-0.84) de tener una ED. Conclusiones: Tener una ED se asocia a la edad, el estado clínico inicial del paciente acorde la escala de Glasgow, así como lesiones en la IRM. EE refractario y más de 4 comorbilidades también fueron predictores de una ED al alta hospitalaria.

Abstract Introduction: Status Epilepticus (SE) is a frequent neurologic emergency. Little research has been done in South America to evaluate the prognostic variables of mortality and disability in patients with SE. Objective: To determine the variables associated to an unfavorable outcome at hospital discharge in the patients who were treated for SE. Methods: A retrospective study was performed during the period of January 2016-June 2017. A total of 26 patients were diagnosed of SE and its different variants. The effects of clinical, radiological, and electroencephalographic features on hospital outcome according Rankin scale were evaluated. Results: Twelve (46.2%) patients had an unfavorable outcome at hospital discharge, while the mortality rate reached 23.1%. There was a predominance of males with a 76.9% of all the patients. The independent variables associated with an unfavorable outcome were the number of comorbidities (p=0.01, OR: 4.27-95%CI1.33-13.6), structural lesions on the Magnetic Resonance Image (MRI) (p=0.04, OR: 3.92-95%CI1.05-14.61) and refractory SE (p=0.01, OR: 12.52-95%CI1.85-84.44). There was also a trend for age (p=0.07 OR: 1.03-95%CI0.99-1.07). While an initial good clinical condition, according to the Glasgow Scale represent a protective factor (p=0.00 OR: 0.49-IC95% 0.29-0.84) of an unfavorable outcome. Conclusions: The unfavorable outcome was marginally associated with patient age, clinical status at the onset of SE according to the Glasgow Coma Scale, as well as brain lesions on brain MRI. Refractory SE and more than 4 comorbidities are predictors of an unfavorable outcome at hospital discharge.

Refractory status epilepticus secondary to Neurexin-3a encephalitis: A case report

@#New–onset refractory status epilepticus (NORSE) is a rare and challenging disease entity that is associated with high morbidity and mortality. Among the NORSE cases with a proven aetiology, autoimmune encephalitis has been identified as a leading cause. Here we describe a young man who presented with NORSE and was eventually found to have serum autoantibodies against Neurexin-3a, a synaptic molecule that has recently been implicated as a target in the pathogenesis of a novel form of autoimmune encephalitis. The patient’s seizures responded to plasmapheresis, though the patient eventually died from sepsis. This case shows that Neurexin-3a encephalitis is a possible cause of NORSE, and plasmapheresis may be an effective treatment.

An update on the management of status epilepticus in adults / Una actualización del tratamiento del estado epilético en el adulto / Uma actualização sobre a gestão do estado epilético em adultos

Abstract: Status epilepticus (SE) is the state of continuous seizures, whether they are convulsive or non-convulsive, lasting longer than 5 minutes without return to the neurologic baseline between seizures. This neurologic emergency accounts for 20% of emergency department (ED) admissions and requires the clinician's expeditious recognition and prompt management in order to prevent irreversible neurological damage and further complications related to the prolonged hospital stay. This paper reviews the most recent and up-to-date information aimed at caring for these critically ill patients.

Resumen: El estado epiléptico, es una enfermedad convulsiva continua, que puede ser convulsiva o no, de más de cinco minutos de duración sin retorno al estado neurológico basal entre las crisis convulsivas. Esta emergencia neurológica representa el 20% de las admisiones del departamento de emergencias y requiere del inmediato reconocimiento del médico y de un pronto manejo para evitar daño neurológico irreversible y mayores complicaciones relacionadas con una estancia hospitalaria prolongada. Este trabajo revisa la información más reciente y actualizada sobre el cuidado de estos pacientes críticos.

Resumo: O estado epiléptico é uma desordem de apreensão contínua, que pode ser convulsiva ou não, de mais de 5 minutos de duração, sem retorno ao estado neurológico basal entre as crises. Esta emergência neurológica é responsável por 20% das internações do departamento de emergência e requer o reconhecimento imediato do médico e de um rápido tratamento para evitar danos neurológicos irreversíveis e complicações maiores associadas com a hospitalização prolongada. Este estudo analisa as informações mais recentes e actualizadas sobre os cuidados a estes pacientes em estado crítico.

Revisión del estado epiléptico convulsivo pediátrico y su manejo antiepiléptico / Management of pediatric status epilepticus

Pediatric Status Epilepticus (SE) is an emergency situation with high morbidity and mortality that requires early and aggressive management. The minimum time criterion to define SE was reduced from 30 to 5 minutes, defined as continuous seizure activity or rapidly recurrent seizures without resumption of consciousness for more than 5 minutes. This definition considers that seizures that persist for > 5 minutes are likely to do so for more than 30 min. Those that persist for more than 30 minutes are more difficult to treat. Refractory SE is the condition that extends beyond 60-120 minutes and requires anesthetic management. Super-refractory SE is the state of no response to anesthetic management or relapse during withdrawal of these drugs. The aim of this review is to provide and update on convulsive SE concepts, pathophysiology, etiology, available antiepileptic treatment and propose a rational management scheme. A literature search of articles published between January 1993 and January 2013, focused on pediatric population was performed. The evidence about management in children is limited, mostly corresponds to case series of patients grouped by diagnosis, mainly adults. These publications show treatment alternatives such as immunotherapy, ketogenic diet, surgery and hypothermia. A 35% mortality, 26% of neurological sequelae and 35% of recovery to baseline condition is described on patient’s evolution.

The Effectiveness and Tolerability of Topiramate and Lidocaine Add-on Therapy in Children with Refractory Status Epilepticus / 대한소아신경학회지

PURPOSE: This study was aimed to compare the effectiveness and tolerability of topiramate add-on therapy versus lidocaine add-on therapy in children with refractory status epilepticus. METHODS: We performed a retrospective analysis of children with refractory status epilepticus who were hospitalized at Kyungpook National University Hospital during the period 2005 to 2012. We divided them into two groups, Group A (midazolam, MDZ, and Topiramate, TPM:11 patients) and Group B (midazolam and lidocaine, LDC:7 patients). Furthermore, they were sub-categorized according to children's sex, age, seizure duration, and seizure type. To comepare the effectiveness and tolerability between the two groups, we evaluated seizure freedom, >50% seizure reduction, adverse events and mortality rates, respectively. RESULTS: In MDZ/TPM group, average dosage of midazolam infusion was 10.8+/-6.8 mcg/kg/min, seizure freedom was achieved in 1 out of 11 patients (9%) and >50% seizure reduction was in 2 out of 11 patients (18%). On the other hand, in MDC/LDC group, average dosage of midazolam infusion is 11.3+/-5.2 mcg/kg/min, seizure freedom was achieved in 2 out of 7 patients (29%) and >50% seizure reduction was 3 out of 7 patients (43%). Aside from the promising effects, there was no significant difference between the two groups. CONCLUSION: In children with refractory status epilepticus, topiramate or lidocaine add-on therapy seemed to be effective and fairly tolerated and showed no serious adverse effects. Therefore they can be an alternative treatment option. However, further studies are required.

Fatal Propofol Infusion Syndrome Associated with Refractory Partial Status Epilepticus in a Patient with Cerebral Hyperperfusion Syndrome

Status epilepticus is rare complication of cerebral hyperperfusion syndrome and is a critical medical emergency that requires potent anesthetics. Propofol has the advantages of being rapid-acting and possessing a weaker cardiopulmonary suppression profile, but its use is associated with serious propofol infusion syndrome. We report herein a case of cerebral hyperperfusion syndrome after carotid endarterectomy presenting with refractory partial status epilepticus and fatal outcome associated with propofol infusion syndrome.

Anesthetic experience for performing a cesarean section for a woman with refractory status epilepticus: A case report

The occurrence of seizure increases 15-30% in women who become pregnant while being treated for epilepsy, due to pharmacological changes in the antiepileptic drugs and, changes in the emotional status and hormones. It is reported that 1-2% of pregnant women experience status epilepticus. When refractory status epilepticus occurs in a pregnant woman, parturition may be an important method of treatment. We report here on a case of a 28 weeks pregnant woman who had epilepsy for 21 years and she had status epilepticus in a refractory status and so she underwent general anesthesia for cesarean section.

Progress of treatment for status epilepticus in children / 中国小儿急救医学

Status epilepticus(SE)is one of the most common medical emergencies in children.There has been some recent understanding of this fife-threatening condition with reference to the definition,forms of ignorable seizure,drug treatment and role of EEG monitoring.This review discusses the current information regarding the definition,diagnosis,treatment of status epilepticus and the latest advance on management of refractory SE in children.

High Dose Intravenous Valproic Acid for the Management of Prolonged Refractory Status Epilepticus : A Case Report of Satisfactory Neurologic Recovery / 대한소아신경학회지

Refractory status epilepticus (RSE) is defined as seizure activity that continues after treatment with conventional anticonvulsants. Mortality rates of range from 2% to 100%. RSE are associated with significant morbidity and high mortality as evidenced by older age of the patient, acute etiology for the seizure, no previous history of seizures, low initial Glasgow Coma Scale, and the significant duration of the RSE. An intravenous valproic acid (VPA) was suggested as a useful drug in controlling RSE. A continuous infusion VPA was an efficient method of rapidly achieving VPA concentrations in the upper region of the therapeutic range while minimizing adverse effects. A 10-year-old female suffered a generalized tonic clonic seizure that developed into refractory status epilepticus. Her Glasgow Coma Scale was deteriorated to 4. Status epilepticus was refractory to phenytoin, phenobarbital, midazolam, thiopental. After 4days of unsatisfactory control of seizure activity, high dose VPA was administered for 2 months. The clinical and electrolgraphic seizure improved and the patient has made an satisfactory neurologic recovery