Use of Continuous Ketamine Infusion as an Adjunctive Agent in Young Infants With Refractory and Super Refractory Status Epilepticus: A Case Series.

Continuous ketamine infusions have been studied as an adjunctive agent for refractory status epilepticus (RSE) and super refractory status epilepticus (SRSE) in older children and adults. However, minimal information exists on the efficacy, safety, and dosing for continuous ketamine in young infants. We present the clinical course of 3 young infants with RSE and SRSE who received continuous ketamine in conjunction with other antiseizure medications. The condition of these patients was refractory to an average of 6 antiseizure medications before initiation of continuous ketamine infusion. For each patient, a continuous ketamine infusion was initiated at a rate of 1 mg/kg/hr with 1 patient requiring titration to a maximum of 6 mg/kg/hr. In 1 case, the concomitant use of continuous ketamine allowed for a reduction in the benzodiazepine continuous infusion rate. In all cases, ketamine was well tolerated especially in the setting of hemodynamic instability. Ketamine may provide a safe adjunct in the acute setting in severe RSE and SRSE. This is the first case series to document the use of continuous ketamine as a treatment modality in young infants with RSE or SRSE secondary to various underlying etiologies, without adverse events. Further studies are needed to evaluate the long-term safety and efficacy of continuous ketamine in this patient population.

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.

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)

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.

Ketogenic Diet in Super-Refractory Status Epilepticus.

Researchers from the Children's National Health System in Washington, D.C. studied the feasibility, rate of complications, and effect on seizures of initiating the Ketogenic Diet (KD) in pediatric patients with Super-Refractory Status Epilepticus (SRSE).

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.

Surgical treatment of focal symptomatic refractory status epilepticus with and without invasive EEG.

PURPOSE: Neurosurgery appears to be a reasonable alternative in carefully selected patients with refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE). We discuss the optimal timing of the surgery and the use of previous stereoelectroencephalography (SEEG) invasive evaluation. METHODS: We identified 3 patients (two pediatric and one adult) who underwent epilepsy surgery because of RSE or SRSE from our epilepsy surgery database, one of them with previous SEEG. RESULTS: Status epilepticus resolved acutely in all of them with no mortality and no substantial morbidity. At follow-up (median: 2 years), 1 patient was seizure-free, and 2 had significant improvement. CONCLUSION: Surgery should be considered in all cases of RSE and SRSE early in the course of the evolution of the disease.