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)

Estado de mal epiléptico / Status epilepticus

El estado de mal epiléptico (EME) es la emergencia más frecuente en la neuropediatría. Es el resultado de un fallo en los mecanismos responsables de terminar la convulsión o de la iniciación de mecanismos que provocan una convulsión anormalmente prolongada. Esta definición se relaciona con el momento de iniciar el tratamiento. En general, el primer punto de tiempo o t1, es el momento cuando el tratamiento debería comenzarse, que es a los 5 minutos para las convulsiones tónico-clónicas generalizadas y a los 10 minutos para las focales con o sin compromiso de la conciencia. El segundo punto de tiempo o t2 marca el momento en el cual el daño neuronal o de las redes neuronales puede comenzar e indica que el EME debería ser controlado, que para los casos de mal tónico-clónico generalizados debe ser de 30 minutos. Todos los protocolos de tratamiento diferencian estadios en donde se utilizan diferentes fármacos: temprano o 1, establecido o 2, refractario o 3, súper-refractario o 4; y enfatizan el rápido reconocimiento y tratamiento de la actividad epiléptica persistente en cada estadio con el objetivo de reducir la morbimortalidad y las secuelas a largo plazo (después de t2).

Status epilepticus (SE) is one of the most common neurologic emergencies in pediatrics. It is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which leads to abnormally, prolonged seizures. This definition provides a good guidance, when emergency treatment must be considered. In general, time point t1 is the time when treatment should be started, which is at 5 minutes for generalized tonic-clonic seizures, and at 10 min for focal seizures with or without impairment of consciousness. Time-point t2 marks the time at which neuronal damage or self-perpetuating alteration of neuronal networks may begin and indicates that SE should be controlled latest by that time; 30 min in case of generalized tonic-clonic seizures. All treatment protocols recognize a staged approach to treatment with different drugs used in early (stage I), established (stage II), refractory (stage III) and super-refractory SE (stage IV); and emphasize prompt recognition and treatment of persisting seizure activity at each stage aiming to reduce morbidity, mortality, and long-term consequences of status epilepticus (beyond t2).

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.

Short-term mortality and prognostic factors related to status epilepticus / Mortalidade a curto prazo e fatores prognósticos de status epilepticus

Objective Status epilepticus (SE) is associated with significant morbidity and mortality, and there is some controversy concerning predictive indicators of outcome. Our main goal was to determine mortality and to identify factors associated with SE prognosis. Method This prospective study in a tertiary-care university hospital, included 105 patients with epileptic seizures lasting more than 30 minutes. Mortality was defined as death during hospital admission. Results The case-fatality rate was 36.2%, which was higher than in previous studies. In univariate analysis, mortality was associated with age, previous epilepsy, complex focal seizures; etiology, recurrence, and refractoriness of SE; clinical complications, and focal SE. In multivariate analysis, mortality was associated only with presence of clinical complications. Conclusions Mortality associated with SE was higher than reported in previous studies, and was not related to age, specific etiology, or SE duration. In multivariate analysis, mortality was independently related to occurrence of medical complications. .

Objetivos Status epilepticus (SE) está associado com morbidade e mortalidade importantes. Diversos estudos avaliaram determinantes de prognóstico relacionados com SE, havendo controvérsias neste sentido. O objetivo deste estudo foi avaliar mortalidade no SE e seus fatores determinantes. Método Estudo prospectivo, em Ribeirão Preto, incluiu 105 pacientes, entre fevereiro e dezembro de 2000. Mortalidade foi definida como óbito no período de internação hospitalar. Resultados O índice de mortalidade foi de 36.2%, superior ao verificado em estudos prévios. Em análise univariada, mortalidade foi associada com idade, antecedente de epilepsia, presença de crises focais complexas, etiologia, recorrência e refratariedade do SE, presença de complicações clínicas e classificação focal do SE. Em análise multivariada, a ocorrência de complicações clínicas relacionou-se significativamente com prognóstico. Conclusões Em nossa amostra, a mortalidade foi mais elevada do que previamente descrito na literatura, não relacionada com idade, etiologia ou duração do SE, mas, em análise multivariada, com complicações médicas durante o tratamento. .

Convulsões relacionadas ao alcoolismo: atualização / Seizures related to alcoholism: update

OBJETIVO: Revisão sobre crises convulsivas relacionadas ao alcoolismo,discutindo sua classificação, fisiopatologia, investigação diagnóstica e seu tratamento. MÉTODO: Revisão não sistemática de artigos utilizando-se os unitermos: "alcoholism", "alcohol", "seizures" e "withdrawal". Priorizou-se a utilização de artigos que apresentassem associação desses unitermos no título. Foram utilizadas as bases de dados do PubMed, Lilacs e Google Scholar. RESULTADOS: Foram encontrados 2.362 artigos associando os unitermos no título, tendo sido escolhidos 26 artigos em inglês, 3 em português, 1 manual e 1 tese em inglês para a elaboração desta revisão. CONCLUSÃO: As crises convulsivas relacionadas ao álcool representam uma das mais graves complicações do alcoolismo. O diagnóstico e o tratamento corretos melhoram o prognóstico desses indivíduos, diminuindo o risco de complicações, a recorrência de crises, a ocorrência de status epilepticus ou a evolução para um quadro de delirium tremens.

OBJECTIVE: Review alcoholism related seizures, discussing classification,pathophysiology, diagnosis and treatment. METHOD: A non-systematic review was performed of articles using the keywords: "alcoholism", "alcohol", "seizures", and "withdrawal". Articles with the combination of these keywords in the title were favored. The search was performed on PubMed, Lilacs database and Google Scholar. RESULTS: Using these search terms 2,362 articles were found, being selected 26 articles in English, 3 articles in Portuguese, 1 English manual, and 1 thesis in English to elaborate this review. CONCLUSION: Seizures related to alcohol are one of the most serious complications of alcoholism. The correct diagnosis and treatment improves the prognosis of these individuals, decreasing the risk of complications,seizure recurrence, status epilepticus and the progression to delirium tremens.

Evaluation of the efficacy of sodium valproate in convulsive status epilepticus following to ıschemic stroke / Avaliação da eficácia do valproato de sódio no status epilepticus convulsivo devido ao acidente vascular cerebral isquêmico

Objective : Convulsive status epilepticus (CSE) is very rarely observed after ischaemic stroke. Sodium valproate (SV) is one of the agents used in the treatment of CSE, but its role still controversial, and its degree of efficacy in treating CSE that develops following stroke is unclear. Method : We evaluated 19 patients who were treated with intravenous (IV) SV (20 mg/kg, 2 mg/kg/h-12h) after diazepam. Patients’ modified Rankin scores (mRS), SE types, and changes in biochemical parameters after treatment were assessed. Results : CSE was successfully treated in 12 (63.15%) patients. Side effects such as hypotension and allergic reactions were observed in two patients. Refractory SE development was observed in 5 (29.4%) patients with high mRS (˃ 3). No significant deterioration in patients’ laboratory evaluations, conducted before and after status, was observed. Conclusion : SV may be safe and effective in the treatment of CSE observed after ischaemic stroke, especially in patients with low mRS. .

Objetivo : Status epilepticus convulsivo (SEC) é muito raramente observado após acidente vascular cerebral isquêmico. Valproato de sódio (VS) é um dos agentes utilizados no tratamento do SEC, mas seu papel ainda é controverso e seu grau de eficácia não é claro no SEC pós acidente vascular. Método Avaliamos 19 pacientes que foram tratados com AV endovenoso (EV) (20 mg/kg, 2 mg/kg/h-12h) após diazepam. Valores da escala modificada de Rankin (mRS) dos pacientes, tipos de SE e mudanças nos parâmetros bioquímicos foram avaliados. Resultados SEC foi tratado com sucesso em 12 pacientes (63,15%). Efeitos colaterais como hipotensão e reações alérgicas foram observados em dois pacientes. Desenvolvimento de SE refratário foi observado em cinco pacientes (29,4%) com altos valores de mRS (˃ 3). Não houve deterioração significativa nas avaliações laboratoriais dos pacientes feitas antes ou depois do status. Conclusão AV pode ser eficaz no tratamento do SEC observado após acidente vascular cerebral isquêmico, especialmente nos pacientes com baixo mRS. .

Estado epiléptico convulsivo en adultos atendidos en el Instituto Nacional de Ciencias Neurológicas de Lima, Perú 2011-2013. / Convulsive status epilepticus in adults attending the Instituto Nacional de Ciencias Neurológicas in Lima, Perú 2011-2013.

Objetivos: describir las características clínicas y la evolución del estado epiléptico convulsivo (EEC) en pacientes adultos admitidos en el Servicio de Emergencia del Instituto Nacional de Ciencias Neurológicas de Lima, Perú entre los años 2011 y 2013. Métodos: Se realizó un estudio prospectivo de serie de casos. Resultados: Se incluyeron 28 pacientes mayores de 17 años, la mediana de edad fue 31 años. El 57% fueron hombres y el 89% tenían antecedentes de epilepsia. La principal causa del EEC fue el incumplimiento de la medicación antiepiléptica (54%). El diazepam seguido de la fenitoína fue el tratamiento antiepiléptico más empleado (75%) y la frecuencia de EEC refractario fue del 4%. Conclusiones: los pacientes fueron mayoritariamente varones, adultos jóvenes, con antecedentes de epilepsia que presentaron un EEC debido el incumplimiento de la medicación antiepiléptica y respondieron favorablemente al tratamiento con diazepam seguido de fenitoína.

Objectives: to describe the characteristics features and outcome of convulsive status epilepticus (CSE) in adult patients admitted to the Emergency Department at the Instituto Nacional de Ciencias Neurológicas in Lima, Peru between 2011 and 2013. Methods: a prospective study was conducted. Results: twenty-eight patients older than 17 years were included, the median age was 31 years old. 57% were male and 89% had a history of epilepsy. Main cause of CSE was antiepileptic medication noncompliance (54%). Diazepam followed by phenytoin was the most used therapeutic regimen (75%) and the frequency of refractory CSE was 4%. Conclusion: patients were mostly male, young adults with a history of epilepsy who presented a CSE caused by the antiepileptic medication noncompliance and the response to treatment based on diazepam followed by phenytoin was positive.

Clinical profile and outcome of children admitted with status epileptics in PICU of a developing country

Status epilepticus [SE] is a common, life-threatening neurologic disorder. The exact incidence is not known. The frequency of occurrence of status epilepticus is 17-23 per 100, 000 persons per year, with the higher incidences occurring in developing countries. In children the mortality from SE ranges from 3-10%. To describe the etiology, clinical profile and immediate-outcome of children with status epilepticus at a Tertiary Care Hospital in Karachi. This is a descriptive and retrospective cohort study on all children admitted in our institution with diagnosis of status epileptics [ICD 9 code 3453]. Demographic, pertinent clinical variables and outcomes were collected on structured questionnaire. During the study period, fifty patients were identified. Mean age was 51 months [age range was 1-168 months with SD +/- 41 months]. There were 29 male: and 21 female. Generalized tonic clonic seizures were the most common [86%] form of seizures observed. Twenty- five [50%] patients were newly diagnosed with no prior history of seizure. Acute febrile illness or infections were the most common etiology [52%]. No biochemical abnormalities were observed in our study. Abnormal EEG was reported in 62% of patients. CSF abnormalities were observed in 22%. Minimum 2 and maximum 8 anti-epileptic drugs were used [mean= 4.33]. The most commonly parenteral drugs included phenytoin, phenobarbitone, levetiracetam and valproic acid. Thirty- one [62%] patients required continuous midazolam infusion. In majority [44%], status was controlled after more than 60mins. Thirty-three patients [66%] required PICU admission for seizure control. Thirty one [62%] required mechanical ventilation, twenty five [50%] required inotropic support. Mean PICU stay duration was 3.89 days [range=1-15 days]. Survival rate was 92%. The cause of death [n=4] was related to underlying systemic illness. No complications were observed in forty-one [82%] patients. In our report, young children had a high incidence of SE and the most common etiology was acute febrile illness. Majority of cases were refractory SE although the mortality rate was low

Estado de mal epiléptico en pediatría / [Status epilepticus in childhood].

Status epilepticus is a medical emergency which presents seizures by 30 minutes or more of continuous activity, or two or more consecutive crises without full recovery of consciousness between them. Currently, it is considered that a seizure convulsive or not, that last more than 5 minutes should be considered a status epilepticus. Different drugs for the treatment of this disease have been used. There is a general consensus in an aggressive handling should be done to reduce their morbidity and mortality, without forgetting that the cause of status is important for its management, control, and its aftermath.

Estado epiléptico no convulsivo infantil / Non convulsive status epilepticus in children

Introduction: Status Epilepticus (SE) is a medical emergency with high morbimortality. Non-convulsive Status Epilepticus (NCSE) is defined as electroencephalographic crises in the absence of clinical motor symptoms. Objective: A restrospective study of NCSE in our population. Patients and Methods: Charts of 30 patients in a Child Neurology Clinic seen between December 1999 and June 2008 were reviewed. According to the characteristics of the episode, they were classified as a) Typical absence, b) Atypical absence, c) Partial Complex episode. Results: Of the 30 patients, 15 (50%) were males. Median age was 46 months. Clinically, 63% of the children suffered from complex partial seizures, 30% atypical absences, 7% typical absences. Cryptogenic SE was most frequent (47%), with a mortality of 3%. Discussion: Pediatric patients have a higher risk of NCSE than adults do. Most patients already carried a diagnosis of Epilepsy, cerebral infarcts were the second most frequent cause of NCSE. They should all be monitored through EEGs. Neuroimages are of great value since outcome depends on the etiology. In summary, clinical suspicion, a history of epilepsy supported by an EEG (best if prolonged) allow early diagnosis and treatment.

Introducción: El Estado Epiléptico (EE) es una emergencia médica con alta morbimortalidad. El Estado Epiléptico No Convulsivo (EENC) es definido por la presencia de crisis electroencefalograficas en ausencia de crisis motoras clínicas. Objetivo: Realizar una descripción retrospectiva de los EENC que se presentaron en la población pediátrica atendida en nuestro centro. Pacientes y Método: Se revisaron los registros de 30 pacientes controlados neurología infantil que presentaron EENC entre diciembre de 1999 y junio de 2008. Según el tipo de crisis se clasificó en a) EENC ausencia típica; b) EENC ausencias atípicas y c) EENC parcial complejo. Según etiología se uso la clasificación de Hauser modificada. Resultados: Se analizaron 30 pacientes. Quince (50%) varones. La mediana de edad fue 46 meses. 63% fueron EENC parcial complejo, 30% EENC ausencias atípicas y 7% EENC ausencias típicas. El EE criptogénico fue el más frecuente (47%). La mortalidad fue de 3%. Discusión: Los pacientes pediátricos tienen mayor riesgo de EENC en relación a los adultos. La gran mayoría de los pacientes con EENC ya tenían el diagnóstico de epilepsia. Los infartos cerebrales fueron la segunda causa de EENC, estos deben ser monitorizados con EEG ante la sospecha de EENC. Las neuroimágenes para precisar el diagnóstico son de gran valor dado que el pronóstico de los pacientes con EENC depende de la etiología subyacente. En conclusión, la sospecha clínica, el antecedente de epilepsia y el apoyo con un EEG, especialmente prolongado, permite el diagnóstico oportuno y el tratamiento precoz.

Status epilepticus and capillary leak syndrome in a neonate related to perinatal hypoxic-ischemic encephalopathy.

Hypoxic-ischemic cerebral injury that occurs during the perinatal period is one of the most commonly recognized cause of long-term neurological deficit in children, often referred to as cerebral palsy. We describe a case with capillary leak syndrome and seizures to co-morbid status epilepticus related to perinatal hypoxicischemic encephalopathy in newborn period.

Tratamiento del estado epiléptico refractario con topiramato oral / Treatment of refractory status epilepticus with topiramate: report of three cases

Refractory status epilepticus is a catastrophic illness of the central nervous system, with a mortality rate that reaches 50 percent. We report three patients admitted with refractory status epilepticus: a 24 year-old male that discontinued antiepileptic medications, a 46 year-old male with a focal epilepsy secondary to an encephalitis that discontinued medications due to gastrointestinal problems and a 59 year-old male with an ischemic encephalopathy AH were treated with topiramate, delivered through a nasogastric tube with a good response.

Status epilepticus as a presenting sign of reversible posterior leukoencephalopathy syndrome in a liver transplant recipient

Reversible posterior leukoencephalopathy syndrome [RPLE] is an increasingly recognized disorder, most commonly associated with immunosuppressive and cytotoxic agent, malignant hypertension and toxemia of pregnancy. Neurological symptoms are headache, mental and visual disturbance, and seizures. Brain MRI findings are symmetrical bilateral hyper intense areas of occipital and parietal lobes in T2-weighted magnetic resonance [MR] images. This finding is reversible with prompt successful treatment which may take days to weeks for full reversal. We observed a case of status epilepticus which occurred 32 days after liver transplantation in a patient on regimen consisting of Tacrolimus [FK 506]. He had no history of neurologic disease or metabolic abnormalities. Typical findings of RPLE were present on brain MRI. The recurrence of seizures terminated after a few hours with adding Na-valproate to Phenytoin, Phenobarbital and Midazolam. The radiological finding of brain MRI gradually diminished after several weeks. A physician should be alert of seizures during early post transplant period of liver transplantation. He should bear in mind the diagnosis of RPLE and consider discontinuation of immunosuppressive drugs

Child abuse: inflicted traumatic brain injury.

We report a one year old boy with clinical and neuroimaging findings of inflicted traumatic brain injury. The clinicians often overlook this form of physical abuse. The family structure also plays an important role in neglecting this form of problem.

Diabetic nonketotic hyperosmolar state: interesting imaging observations in 2 patients with involuntary movements and seizures.

We report two patients of diabetic nonketotic hyperosmolar state presenting acutely with "self-limiting hemichorea - hemiballismus" and "generalized convulsive status epilepticus". CT scan in both the patients revealed a hyperdense nonenhancing basal ganglia. Magnetic resonance imaging brain of patient 1 showed it to be hyperintense on T1W image and iso-hyper intense on T2W image, minimally enhancing with contrast injection.

Estado confusional agudo como manifestação de status epilepticus não-convulsivo em criança previamente hígida: relato de caso / Acute confusional state in a child with non-convulsive status epilepticus: case report

INTRODUÇÃO: O estado confusional em crianças pode ter várias causas. Entre elas está o estado de mal não-convulsivo. OBJETIVO: Relatamos o caso de um paciente que se apresentou ao serviço de emergência com confusão mental. Após investigação preliminar, o EEG mostrou tratar-se de estado de mal de ausência. RESULTADO: A administração de Diazepam endovenoso seguiu-se normalização do traçado eletroencefalográfico e melhora clínica do paciente. CONCLUSÃO: O EEG é fundamental para se diagnosticar e tratar quadros de estado de mal epiléptico não-convulsivo.

INTRODUCTION: There are many causes to acute confusional state in children. Non-convulsive status epilepticus is one of them. OBJECTIVE: We report the case of a patient who came to our emergency service with confusion. After preliminary investigation the EEG revealed abnormalities consistent with absence status. RESULTS: After treatment with diazepam intravenously occurred normalization of the EEG and, clinically, the patient was better. CONCLUSION: The EEG is crucial in the evaluation of patients with non-convulsive status epilepticus.

Wilson's disease presenting as status epilepticus.

A patient of Wilson's disease having neurological as well as psychiatric manifestations who presented with status epilepticus is being reported. The diagnosis was confirmed by biochemical investigations and 'face of giant panda' sign was present on MRI brain.