Simple and complex febrile seizures: is there such a difference? Management and complications in an emergency department.

OBJECTIVE: We aimed to analyse the prevalence, characteristics, and management of simple and complex febrile seizures. The secondary objective was to compare the risk of underlying organic lesion and epilepsy in both types of seizures, with a particular focus on the different subtypes defining a complex febrile seizure. MATERIAL AND METHODS: We performed a retrospective cohort study including patients aged 0--16 years who were treated for febrile seizures in the paediatric emergency department of a tertiary hospital over a period of 5 years. Epidemiological and clinical variables were collected. Patients were followed up for at least 2 years to confirm the final diagnosis. RESULTS: We identified 654 patients with febrile seizures, with a prevalence of 0.20% (95% CI, 0.18-0.22); 537 (82%) had simple febrile seizures and 117 (18%) had complex febrile seizures. The clinical and epidemiological characteristics of both types were similar. Significantly more complementary tests were requested for complex febrile seizures: blood tests (71.8% vs 24.2% for simple febrile seizures), urine analysis (10.3% vs 2.4%), lumbar puncture (14.5% vs 1.5%), and CT (7.7% vs 0%). Similarly, admission was indicated more frequently (41.0% vs 6.1%). Underlying organic lesions (central nervous system infection, metabolic disease, tumour/intracranial space-occupying lesion, intoxication) were diagnosed in only 11 patients, 5 of whom had complex forms (4.3%; 95% CI, 0.6-7.9). Risk factors for developing epilepsy, identified in the multivariate analysis, were complex forms with recurrent seizures in a single attack (odds ratio [OR]: 4.94; 95% CI, 1.29-18.95), history of seizures (OR: 17.97; 95% CI, 2.26--143.10), and seizures presenting at atypical ages (OR: 11.69; 95% CI, 1.99-68.61). CONCLUSIONS: The systematic indication of complementary tests or hospital admission of patients with complex febrile seizures is unnecessary. The risk of epilepsy in patients with complex forms gives rise to the need for follow-up in paediatric neurology departments.

Crisis febril como manifestación inicial de COVID-19 / Febrile crisis as initial manifestation of COVID-19

Introducción: La COVID-19 es una enfermedad que ha mostrado una baja morbilidad y mortalidad en pediatría. Las crisis febriles constituyen una de las causas más frecuentes de admisión en los servicios de urgencia y de interconsultas con el neuropediatra. Objetivo: Interpretar la presencia de crisis febril simple en pacientes admitidos en servicios de urgencia pediátricos como manifestación inicial de COVID-19. Presentación del caso: Paciente femenina de un año de edad, con antecedentes de salud anterior que acude al cuerpo de guardia con fiebre de 38 ℃; y crisis motora, de inicio generalizada, tónico-clónica, que cedió con medidas antitérmicas. Por protocolo se realiza punción lumbar la cual resulta negativa. Se ingresa, 24 horas después comienza con manifestaciones catarrales, se hace otoscopia y se diagnostica una otitis media aguda, por lo que se inicia tratamiento antibiótico. Se realiza la prueba de reacción en cadena de la polimerasa para la COVID-19 con resultado positivo, por lo que se remite para el Hospital Pediátrico San Miguel del Padrón. Conclusiones: Se debe sospechar la presencia de COVID-19 ante paciente que acude a servicio de urgencias por una crisis febril, como único problema de salud. LA COVID-19 es una enfermedad que ha demostrado que se puede presentar de diversas formas(AU)

Introduction: COVID-19 is a disease that has shown low morbidity and mortality in pediatrics. Febrile crises are one of the most common causes of admission to emergency services and consultations with the neuropediatrician. Objective: Interpret the presence of simple febrile crisis in patients admitted to pediatric emergency services as an initial manifestation of COVID-19. Case presentation: One-year-old female patient, with a previous health history, who is attended in the emergency service with a fever of 38 ℃; and motor crisis, of widespread onset, with tonic-clonic seizures, which yielded with antypiretic measures. By protocol, lumbar puncture is performed which is negative. She was admitted, and 24 hours later there is an onset of catarrhal manifestations; an otoscopy is performed and acute otitis media is diagnosed, so antibiotic treatment is initiated. The polymerase chain reaction test for COVID-19 (PCR) is performed with a positive result, so, the patient is referred to the San Miguel del Padrón Pediatric Hospital. Conclusions: COVID-19 should be suspected in patients who attend to the emergency services due to a febrile crisis as the only health problem. COVID-19 is a disease that has been shown to occur in a variety of ways(AU)

Febrile seizures in an urban Tanzanian population: lessons learned from a community-based random cluster survey.

OBJECTIVE: To analyse the cumulative incidence of febrile seizures, to evaluate the accuracy of our screening questionnaire and to describe clinical characteristics of children with febrile seizure in an urban population in Tanzania. METHODS: A large random cluster sampled population was screened for a febrile seizure history as part of a larger epilepsy study using a standardised questionnaire in a two-stage door-to-door survey in Tanzania. A subset of screen positive participants was further examined for confirmation of diagnosis and evaluation of clinical characteristics. RESULTS: Overall, 49 697 people were screened for a febrile seizure history of whom 184 (0.4%) screened positive. Women more commonly screened positive than men (112 [0.4%] vs. 72 [0.3%]). There was no marked difference between age groups or education. The positive predictive value of the screening tool was 37% (95% CI 24-51%) but its accuracy varied with the age of interviewed individuals. Cumulative incidence rates were estimated between 1.1% and 2.0% after adjusting for the inaccuracy of the screening tool. Most febrile seizures occurred before the age of two (65%) and most children had more than one episode (80%). A large proportion of children had complex febrile seizure (65%), often caused by malaria or respiratory infections. CONCLUSIONS: The community-based cumulative incidence of a febrile seizure history in an urban Tanzanian population was similar to rates reported from other rural populations after adjusting for the inaccuracy of our screening tool. Based on the integrated nature of the febrile seizure questionnaire, screening positivity rates may have been too low. This has implications for the design of future studies. The majority of cases had complex febrile seizures often associated with malaria. This has implications for clinical case management.

Risk of febrile seizures among children conceived following fertility treatment: A cohort study.

BACKGROUND: Studies have shown that fertility treatment in mothers is associated with neurological problems in children. However, knowledge about any association between maternal use of fertility treatment and febrile seizures in children is lacking. OBJECTIVE: To determine whether maternal use of fertility treatment is associated with febrile seizures in children. METHODS: All liveborn children in Denmark during 1996-2012 (n = 1 065 901) were linked with the Danish Infertility Cohort and the Danish national registers and were followed from one year of age until the first episode of a febrile seizure, death, emigration, loss to follow-up, or end of follow-up (December 2015). Cox proportional hazard regression was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) with adjustment for potential confounders. RESULTS: Approximately 16% children (n = 172 140) were conceived by infertile women, and approximately 3% (n = 34 082) were diagnosed with febrile seizures during follow-up. Compared with children conceived by fertile women, children conceived following any fertility treatment (HR 1.11, 95% CI 1.06, 1.16), following specific fertility treatment, for example IVF (HR 1.15, 95% CI 1.05, 1.25), ICSI (HR 1.20, 95% CI 1.10, 1.32), and following fertility drugs (HR 1.06, 95% CI 1.00, 1.11) had slight increase in risk of febrile seizures, after adjusting for calendar year of birth, parental age, education, parity status, and maternal smoking during pregnancy. The associations were unchanged when children conceived naturally by infertile women were used as the reference group. CONCLUSIONS: Children conceived following fertility treatment had slightly increased relative risk for febrile seizures.

Severe Factor X Deficiency Presenting as Febrile Seizure in an Infant.

Factor X deficiency is a severe inherited coagulation disorder, which is characterized by severe systemic bleeding manifestations in affected individuals. It is a rare disorder with a frequency of around 1:1,000,000 in the general population. We present the case of an infant with factor X deficiency who presented with complex febrile seizure. Although febrile seizures are very common in children, a closer scrutiny leads to neuroimaging and finding of intracranial bleed. Hematologic and genetic investigations confirmed the diagnosis. A high index of suspicion should be maintained to diagnose uncommon bleeding disorders in children.

Incidence of primary vesicoureteral reflux in patients with febrile convulsions.

The aim of this study is to evaluate the incidence of vesicoureteral reflux (VUR) in patients with febrile convulsion (FC). For this, patients that were diagnosed with FC in 2018 were retrospectively reviewed. Those with epilepsy, motor/mental retardation, or spina bifida were excluded. Mid-stream urine samples were collected in children who were toilet trained while sterile bags were used in the smaller. Urinary tract infection (UTI) was defined as ˃ 5 leucocytes/HPF in urinalysis and a subsequent positive urine culture (≥ 10.000 CFU/ml). Children with UTI were further investigated via voiding cystourethrogram (VCUG) and dimercaptosuccinic acid (DMSA) scintigraphy for VUR. Urinalysis was present in 79 among a total of 181 patients (43.6%). Forty-five of the patients were male (57%). Mean age was 2.6 ± 1.4 years. UTI was diagnosed in 6 (7.6%) patients (5 females, 1 male). Three of the girls had recurrent febrile UTI and subsequently, VUR was diagnosed in two of them. VUR is found in 2.5% of the FC cases with urine sampling. Urinalysis should not be ignored in patients with FC as it may lead to diagnosis of VUR.

A qPCR expression assay of IFI44L gene differentiates viral from bacterial infections in febrile children.

The diagnosis of bacterial infections in hospital settings is currently performed using bacterial culture from sterile site, but they are lengthy and limited. Transcriptomic biomarkers are becoming promising tools for diagnosis with potential applicability in clinical settings. We evaluated a RT-qPCR assay for a 2-transcript host expression signature (FAM89A and IFI44L genes) inferred from microarray data that allow to differentiate between viral and bacterial infection in febrile children. This assay was able to discriminate viral from bacterial infections (P-value = 1.04 × 10-4; AUC = 92.2%; sensitivity = 90.9%; specificity = 85.7%) and showed very high reproducibility regardless of the reference gene(s) used to normalize the data. Unexpectedly, the monogenic IFI44L expression signature yielded better results than those obtained from the 2-transcript test (P-value = 3.59 × 10-5; AUC = 94.1%; sensitivity = 90.9%; specificity = 92.8%). We validated this IFI44L signature in previously published microarray and whole-transcriptome data from patients affected by different types of viral and bacterial infections, confirming that this gene alone differentiates between both groups, thus saving time, effort, and costs. Herein, we demonstrate that host expression microarray data can be successfully translated into a fast, highly accurate and relatively inexpensive in vitro assay that could be implemented in the clinical routine.

Crisis o convulsiones febriles: certezas y preguntas / Febrile seizures: questions and answers

Las crisis febriles están asociadas a fiebre en niños entre 6 y 60 meses de edad, sin infección del sistema nervioso central u otras causas de crisis sintomáticas agudas y sin historia de crisis afebriles previas. Ocurren en aproximadamente el 2-5% de los niños. Se debe considerar la posibilidad de una infección del sistema nervioso, a pesar de que la frecuencia es extremadamente baja cuando el examen físico posterior a la crisis no es orientador. Mientras que el manejo clínico de los niños con crisis febriles simples está bien definido, considerándolas como eventos benignos auto-limitados, la conducta en los niños con crisis febriles complejas es controvertida. Se asocian con un aumento relativamente pequeño del riesgo de epilepsia, el cual no puede ser prevenido mediante ninguna forma de tratamiento. El rol del electroencefalograma también es controvertido. Analizamos los datos de varios estudios y concluimos que las descargas epileptiformes tienen valores predictivos positivos bajos e implican pequeñas variaciones entre la probabilidad pre y post-prueba para el desarrollo de epilepsia posterior. Se ha propuesto realizar resonancias magnéticas encefálicas para detectar cambios a nivel hipocampal previos, agudos o posteriores a crisis focales o estatus febriles que pudieran relacionarse con el riesgo de esclerosis mesial temporal y de epilepsia temporal. La relación etiológica entre estas entidades continúa siendo un tema controvertido. En cualquier caso, los estudios alterados no van a cambiar el manejo clínico de las crisis febriles y pueden contribuir al sobre-diagnóstico.

A febrile seizure occurs in association with fever in a child aged 6 to 60 months, without central nervous system infection or other known cause of acute seizures in a child without a prior history of afebrile seizures. Febrile seizures occur in about 2-5% of children. Central nervous system infections should be considered in patients with febrile seizures, even though the frequency of this possibility is low, especially when patients do not return to baseline. Simple febrile seizures are considered benign events and there are clear guidelines about evaluation and management, but the evaluation of complex febrile seizures is controversial. They are associated with a small increased risk of epilepsy which cannot be prevented. The role of electroencephalography is controversial. We analyzed the data of many studies and concluded that epileptiform discharges have poor positive predictive value. Neuroimaging is recommended to look for acute or pre-existing hippocampal abnormalities following febrile status or focal febrile seizures that could be associated to the risk of developing mesial temporal sclerosis and temporal lobe epilepsy. The relationship between these disorders and febrile seizures remains a controversial issue. An abnormal electroencephalography or magnetic resonance imaging studies will not change the clinical management and could contribute to overdiagnosis.

Valoración del niño con una primera crisis no provocada / Evaluation of a child with a first unprovoked seizure

Los episodios paroxísticos son uno de los trastornos neurológicos más frecuentes en niños. Es importante diferenciar entre los trastornos paroxísticos no epilépticos, las crisis sintomáticas agudas, las crisis febriles y las crisis no provocadas. La historia clínica permite, en la gran mayoría de los casos, poder llegar al diagnóstico. Las crisis no provocadas únicas son frecuentes en el niño. La tasa de recurrencia tras una primera crisis no provocada varía entre un 23-96% en los dos años siguientes a la primera crisis. El objetivo de este trabajo es describir cómo evaluar al paciente con una primera crisis no provocada y revisar el valor predictivo de los distintos factores de riesgo de recurrencia. Entre dichos factores de riesgo, se evalúan datos como la presencia de antecedentes familiares de epilepsia, historia previa de crisis febriles, edad de debut, tipo de crisis, presencia de crisis prolongadas o crisis agrupadas, crisis de presentación en sueño, anomalías neurológicas, evidencia de una etiología definida, y presencia de anomalías epileptiformes en el electroencefalograma. Los factores de riesgo de recurrencia más importantes son la etiología de las crisis y la presencia de anomalías epileptiformes en el electroencefalograma.

Paroxysmal episodes are one of the most common neurological disorders in children. It is important to distinguish between paroxysmal non-epileptic events, symptomatic seizures, febrile seizures, and unprovoked seizures. Patient's history is the key to proper diagnosis in most of the cases. A single unprovoked seizure is a frequent phenomenon in the pediatric population. Studies of recurrence after a first unprovoked seizure show percentages between 23% and 96% over a median follow-up of two years. The aim of this study is to define how to evaluate the first unprovoked epileptic seizure in a child and to review the weight of the different recurrence risk factors. Several factors enable us to predict the recurrence risk after a first unprovoked seizure including family history of epilepsy, prior history of febrile seizures, age at onset, type of seizure, prolonged seizures at onset, multiple seizures in a single day, sleep state, neurological abnormalities, etiology, and abnormalities in the electroencephalogram. The most important of these risk factors are the etiology of the seizures and the evidence of epileptiform abnormalities in the electroencephalogram.

Serum Interleukin-6 Levels in Children with Febrile Seizures.

OBJECTIVE: To compare levels of Interleukin-6 (IL-6) in children with febrile seizures and febrile controls. METHODS: Study conducted in a tertiary-care hospital in Northern India from November 2013 to April 2015, enrolling 160 children (80 each with febrile seizures and febrile controls), aged 6 - 60 months. Serum IL-6 estimated by ELISA method. Iron study done as per standard technique. All the cases of febrile seizure were followed up at 1 week, 3 months and 6 months for recurrence of seizures. RESULTS: The mean serum IL-6 levels in children with febrile seizures was 62.0 (63.9) pg/mL and febrile controls was 86.9 (70.6) pg/mL (P=0.025). CONCLUSION: Serum IL-6 levels were significantly lower in children with febrile seizures as compared to febrile controls.

Crisis convulsivas febriles: revisión integral / Febrile Seizures: Comprehensive Review

Las convulsiones febriles (CF) son el trastor-no convulsivo más frecuente en la niñez y motivo común de consulta en los departa-mentos de emergencia. Son eventos críticos que coinciden con procesos febriles, en niños de 6 a 60 meses, sin infección del SNC, trastornos metabólicos, ni anomalías neurológicas previas. Estos trastornos son de carácter benigno y autolimitado, sin secuelas neurológicas a largo plazo. La prevalencia de CF es de aproximadamente 2 - 5 %, se ha descrito una mayor incidencia en varones en un 60%, con una relación 2:1. Los niños con antecedentes familiares en primer grado con CF, tienen un riesgo de 4-5 veces más alto que la población general de presentar CF, a pesar de esto algunos casos son esporádicos, lo que sugiere que elemen-tos genéticos y ambientales influyen en su aparición. El diagnóstico es fundamental-mente clínico, los exámenes complementa-rios deben reservarse para casos específi-cos y descartar otras patologías. El trata-miento únicamente está indicado en el manejo de las crisis agudas persistentes...(AU)

Do All Children Who Present With a Complex Febrile Seizure Need a Lumbar Puncture?

STUDY OBJECTIVE: We assess the prevalences of bacterial meningitis and herpes simplex virus meningoencephalitis (HSV-ME) in children with a complex febrile seizure and determine these prevalences in the subgroup of children with a clinical examination result not suggestive of meningitis or encephalitis. METHODS: This multicenter retrospective study was conducted in 7 pediatric emergency departments (EDs) in the region of Paris, France. Visits of patients aged 6 months to 5 years for a complex febrile seizure from January 2007 to December 2011 were analyzed. We defined a subgroup of patients whose clinical examination result was not suggestive of meningitis or encephalitis. Bacterial meningitis and HSV-ME were sequentially sought for by analyzing bacteriologic and viral data at the visit, looking for data from a second visit to the hospital after the index visit, and telephoning the child's parents. RESULTS: From a total of 1,183,487 visits in the 7 pediatric EDs, 839 patients presented for a complex febrile seizure, of whom 260 (31.0%) had a lumbar puncture. The outcomes bacterial meningitis and HSV-ME were ascertainable for 715 (85%) and 657 (78.3%) visits, respectively, and we found 5 cases of bacterial meningitis (0.7% [95% confidence interval [CI] 0.2% to 1.6%]) and no HSV-ME (0% [95% CI 0% to 0.6%]). Among the 630 visits of children with a clinical examination result not suggesting meningitis or encephalitis, we found no bacterial meningitis (0% [95% CI 0% to 0.7%]) and no HSV-ME (0% [95% CI 0% to 0.8%]). CONCLUSION: In children with a complex febrile seizure, bacterial meningitis and HSV-ME are unexpected events when the clinical examination after complex febrile seizure is not suggestive of meningitis or encephalitis.

Diagnostic and prognostic factors for acute encephalopathy.

BACKGROUND: Acute encephalopathy has the possibility of sequelae. While early treatment is required to prevent the development of sequelae, differential diagnosis is of the utmost priority. The aim of this study was therefore to identify parameters that can facilitate early diagnosis and prediction of outcome of acute encephalopathy. METHODS: We reviewed the medical charts of inpatients from 2005 to 2011 and identified 33 patients with febrile status epilepticus. Subjects were classified into an acute encephalopathy group (n = 20) and a febrile convulsion group (n = 13), and the parameters serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), lactate dehydrogenase (LDH), ammonia (NH3 ), cerebrospinal fluid (CSF) tau protein, and CSF interleukin-6 compared between them. Furthermore, the relationship between each parameter and prognosis was investigated in the encephalopathy group. RESULTS: Significant differences in serum AST, ALT, and LDH were observed between the febrile convulsion and acute encephalopathy group. Moreover, a significant difference in serum LDH was noted between the patients with and without developmental regression at the time of hospital discharge in the encephalopathy group. In particular, CSF tau protein was found to be highly likely to indicate progress, with CSF tau protein >1000 pg/dL associated with poor prognosis leading to developmental regression. CONCLUSION: Serum AST, ALT and LDH may be related to early diagnosis and prognosis, and should be carefully investigated in patients with encephalopathy. CSF tau protein could also be used as an indicator of poor prognosis in acute encephalopathy.

The Relationship Between Iron Deficiency and Febrile Convulsion: A Case-Control Study.

INTRODUCTION: Febrile seizure is among the most common convulsion disorders in children, which strikes 2% to 5% of children between 3 to 60 months of age. Some studies have reported that iron deficiency could be a risk factor for febrile seizure. The present study was conducted to compare the rate of iron deficiency anemia in febrile children with and without seizure. MATERIALS AND METHODS: This case-control study evaluated 200 children aged 6-60 month in two 100 person groups (febrile seizure and febrile without convulsion) in Kashan. The CBC diff, serum iron and TIBC were done for all of participants. Diagnosis of iron deficiency anemia based on mentioned tests. RESULTS: No significant differences were found in two groups regarding to the age, gender, and the disease causing the fever. The presence of iron deficiency anemia was 45% in the convulsion group and 22% in the group with fever without convulsion. The Chi Square test indicated a significant difference between two groups. CONCLUSIONS: The findings suggest that a considerable percentage of children having febrile seizure suffer from iron-deficiency anemia and low serum iron. This means the low serum iron and presence of anemia can serve as a reinforcing factor for the febrile seizure in children.

Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics.

Evidence-based guidelines, or recommendations, for the management of infants with seizures are lacking. A Task Force of the Commission of Pediatrics developed a consensus document addressing diagnostic markers, management interventions, and outcome measures for infants with seizures. Levels of evidence to support recommendations and statements were assessed using the American Academy of Neurology Guidelines and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. The report contains recommendations for different levels of care, noting which would be regarded as standard care, compared to optimal care, or "state of the art" interventions. The incidence of epilepsy in the infantile period is the highest of all age groups (strong evidence), with epileptic spasms the largest single subgroup and, in the first 2 years of life, febrile seizures are the most commonly occurring seizures. Acute intervention at the time of a febrile seizure does not alter the risk for subsequent epilepsy (class 1 evidence). The use of antipyretic agents does not alter the recurrence rate (class 1 evidence), and there is no evidence to support initiation of regular antiepileptic drugs for simple febrile seizures (class 1 evidence). Infants with abnormal movements whose routine electroencephalography (EEG) study is not diagnostic, would benefit from video-EEG analysis, or home video to capture events (expert opinion, level U recommendation). Neuroimaging is recommended at all levels of care for infants presenting with epilepsy, with magnetic resonance imaging (MRI) recommended as the standard investigation at tertiary level (level A recommendation). Genetic screening should not be undertaken at primary or secondary level care (expert opinion). Standard care should permit genetic counseling by trained personal at all levels of care (expert opinion). Genetic evaluation for Dravet syndrome, and other infantile-onset epileptic encephalopathies, should be available in tertiary care (weak evidence, level C recommendation). Patients should be referred from primary or secondary to tertiary level care after failure of one antiepileptic drug (standard care) and optimal care equates to referral of all infants after presentation with a seizure (expert opinion, level U evidence). Infants with recurrent seizures warrant urgent assessment for initiation of antiepileptic drugs (expert opinion, level U recommendation). Infantile encephalopathies should have rapid introduction and increment of antiepileptic drug dosage (expert opinion, level U recommendation). There is no high level evidence to support any particular current agents for use in infants with seizures. For focal seizures, levetiracetam is effective (strong evidence); for generalized seizures, weak evidence supports levetiracetam, valproate, lamotrigine, topiramate, and clobazam; for Dravet syndrome, strong evidence supports that stiripentol is effective (in combination with valproate and clobazam), whereas weak evidence supports that topiramate, zonisamide, valproate, bromide, and the ketogenic diet are possibly effective; and for Ohtahara syndrome, there is weak evidence that most antiepileptic drugs are poorly effective. For epileptic spasms, clinical suspicion remains central to the diagnosis and is supported by EEG, which ideally is prolonged (level C recommendation). Adrenocorticotropic hormone (ACTH) is preferred for short-term control of epileptic spasms (level B recommendation), oral steroids are probably effective in short-term control of spasms (level C recommendation), and a shorter interval from the onset of spasms to treatment initiation may improve long-term neurodevelopmental outcome (level C recommendation). The ketogenic diet is the treatment of choice for epilepsy related to glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency (expert opinion, level U recommendation). The identification of patients as potential candidates for epilepsy surgery should be part of standard practice at primary and secondary level care. Tertiary care facilities with experience in epilepsy surgery should undertake the screening for epilepsy surgical candidates (level U recommendation). There is insufficient evidence to conclude if there is benefit from vagus nerve stimulation (level U recommendation). The key recommendations are summarized into an executive summary. The full report is available as Supporting Information. This report provides a comprehensive foundation of an approach to infants with seizures, while identifying where there are inadequate data to support recommended practice, and where further data collection is needed to address these deficits.

Diagnosis and management of childhood epilepsy.

Epilepsy is a relatively common neurologic disorder in children that has important implications for development, parents, and society. Making the correct diagnosis starts with an accurate and complete history that consequently leads to a directed diagnostic workup. This article outlines a diagnostic and management approach to pediatric seizures and epilepsy syndromes. Making the correct diagnosis of epilepsy or nonepileptic imitators allows the practitioner to prescribe appropriate therapy. Initial management for typical epileptic syndromes and seizures and potential adverse effects are discussed. Alternative treatment options for pharmacologically resistant patients such as ketogenic diet, vagal nerve stimulation, and surgery are also discussed. While most children favorably respond to antiepileptic medications, early identification of medication failure is important to ensure optimal neurodevelopment.

¿Crisis febriles complejas o síndrome de Dravet?: Descripción de 3 casos clínicos / Complex febrile Seizures or Dravet syndrome?: Description of 3 case reports

Introduction: Dravet syndrome (DS) is one of the most intractable forms of epilepsy that begins in infancy. This syndrome is characterized by beginning with complex febrile seizures (FS) in a healthy infant and progresses to refractory epilepsy with psychomotor regression. The detection of a SCN1A mutation encoding the sodium channel can confirm the diagnosis. Objective: To report 3 confirmed cases of genetically DS. Case reports: We describe 3 girls diagnosed with complex FS that started when they were between 2 and 7 months old. FS were frequent, hemi generalized and myoclonic associated with recurrent febrile status epilepticus (SE). Despite FS and SE recurrence, the psychomotor development, electrophysiological studies and magnetic resonance imaging (MRI) of the brain were normal. After a year, they developed afebrile seizures progressing to refractory epilepsy with developmental regression. A molecular study detected SCN1A mutation confirming DS. The specific antiepileptic treatment and prevention of febrile episodes allowed partial control of epilepsy with some recovery of psychomotor skills. Conclusions: The high frequency complex FS associated with recurrent SE in a previously healthy infant should alert about the possibility of DS. Molecular diagnostics helps us to establish a drugs and non-drug therapies treatment, as well as long-term prognosis and genetic counseling.

Introducción: El Síndrome de Dravet (SD) es una de las formas más intratables de epilepsia que debuta en lactantes con convulsiones febriles (CF) complejas recurrentes que evolucionan posteriormente a epilepsia refractaria con regresión psicomotora. La detección de una mutación del canal de Sodio (SCN1A) permite certificar el diagnóstico. Objetivo: Reportar 3 casos de SD confirmados genéticamente. Casos clínicos: Se describen 3 niñas con diagnóstico de CF complejas iniciadas entre los 2 y 7 meses de edad. Las CF eran frecuentes, hemigeneralizadas, mioclónicas asociadas a status epilepticus (SE) febriles recurrentes. A pesar de la recurrencia de CF y SE, tanto el desarrollo psicomotor como los estudios electrofisiológicos y la resonancia magnética (RM) cerebral, fueron normales. Posterior al año iniciaron crisis afebriles que evolucionaron a epilepsia refractaria con regresión del desarrollo. El estudio molecular detectó la mutación SCN1A confirmando SD. El tratamiento antiepiléptico específico y la prevención de cuadros febriles permitieron un control parcial de la epilepsia con recuperación de algunas habilidades psicomotoras. Conclusiones: La alta frecuencia de CF complejas asociadas a SE recurrentes en un lactante previamente sano, debe alertar sobre la posibilidad de un SD. El diagnóstico molecular nos permite instaurar un tratamiento antiepiléptico y terapias no farmacológicas además de un pronóstico a largo plazo y consejería genética.

Disseminated tuberculosis presenting as febrile seizures with fatal evolution in an infant.

Disseminated tuberculosis with the involvement of brain, liver and gut is a rare disease in immunocompetent infant. Early diagnostic and instauration of anti-tuberculosis therapy is capital because the outcome is poor. Here, we report the case of an 11-month-old boy with disseminated tuberculosis of brain, liver abdominal lymph nodes, small bowel and lung, which presented with fever, generalized tonic-clonic seizure, hemodynamic instability and a history of recurrent respiratory tract infections. His father was diagnosed with active pulmonary tuberculosis six month ago and family members completed an anti-tuberculosis chemoprophylaxis regimen.