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Neonatal epileptic syndromes are part of the genetic and metabolic epilepsies in this age group. Although they are not the most frequent cause of neonatal seizures, their early recognition allows for better diagnostic and therapeutic approaches. These syndromes can be classified into self-limited neonatal syndromes and early infantile epileptic and developmental encephalopathies (EIDEE). While they may share semiology in some types of seizures, such as sequential, and even share alterations in common genes in their etiology, their evolution is very different. In self-limited neonatal syndromes, seizures typically resolve within the first months of life with normal psychomotor development, giving rise to the term self-limited. However, the term benign should not be used as some may present recurrence of seizures, movement disorders, or learning disorders. In the case of EIDEE, seizures are usually refractory to treatment, affecting brain functions and neurodevelopment. In this review, our aim was to describe the electroclinical phenotype of neonatal epileptic syndromes, the most frequently involved genes and their clinical spectrum, their diagnostic approach, as well as the recommended treatments.

Los síndromes epilépticos neonatales hacen parte de las epilepsias de origen genético y metabólico en este grupo edad y aunque no son la causa más frecuente de crisis neonatales, su reconocimiento temprano permite dirigir mejor su enfoque diagnóstico y tratamiento. Pueden clasificarse en síndromes neonatales autolimitados y encefalopatías epilépticas y del desarrollo infantil temprano (EIDEE). Aunque pueden mostrar semiología similar en algunos tipos de crisis, como las secuenciales, e incluso comparten alteraciones en genes comunes en su etiología, su evolución es muy diferente. En los síndromes autolimitados, las crisis remiten en los primeros meses de vida alcanzando un desarrollo psicomotor normal, lo que da su nombre de autolimitado; sin embargo, el término benigno no debe utilizarse dado que algunos pueden presentar recurrencia de crisis, trastornos del movimiento o trastornos del aprendizaje. En las EIDEE las crisis suelen ser refractarias al tratamiento y se comprometen funciones cerebrales y el neurodesarrollo. En esta revisión describiremos el fenotipo electroclínico de los síndromes epilépticos neonatales, los genes más frecuentemente involucrados y su espectro clínico, su enfoque diagnóstico, así como los tratamientos recomendados.

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Resumen La aparición de convulsiones es frecuente durante el periodo neonatal debido a las características de inma durez funcional del cerebro es este periodo. La aparición de estas convulsiones puede llevar a un diagnóstico de epilepsia neonatal, que suele estar asociado a alteracio nes estructurales del cerebro durante el neurodesarrollo. Aproximadamente el 50% de las personas con epilepsia activa padecen al menos un trastorno médico comórbi do, y esto hace que cambie la evolución de la epilepsia. La presencia de trastornos neurológicos que preceden a la aparición de la epilepsia indica que alteraciones es tructurales y/o funcionales del cerebro subyacentes pue den ser causa de la predisposición a padecer epilepsia y de los procesos comórbidos de manera independiente. En esta revisión describimos los procesos cerebrales estructurales y funcionales que subyacen a la aparición de epilepsia neonatal y sus comorbilidades.

Abstract The occurrence of seizures is frequent during the neonatal period due to the functional immaturity of the brain.The presence of these seizures may lead to a diagnosis of neonatal epilepsy, which is usually as sociated with structural alterations of the brain during neurodevelopment. Approximately 50% of people with active epilepsy have at least one comorbid medical di sorder, and the existence of a comorbid process changes the course of the epilepsy. The presence of neurologic disorders preceding the onset of epilepsy indicates that underlying neurobiological alterations may indepen dently cause the predisposition to epilepsy and comor bid processes. In this review we describe the structural and functional brain processes underlying the onset of neonatal epilepsy and its comorbidities.

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The occurrence of seizures is frequent during the neonatal period due to the functional immaturity of the brain.The presence of these seizures may lead to a diagnosis of neonatal epilepsy, which is usually associated with structural alterations of the brain during neurodevelopment. Approximately 50% of people with active epilepsy have at least one comorbid medical disorder, and the existence of a comorbid process changes the course of the epilepsy. The presence of neurologic disorders preceding the onset of epilepsy indicates that underlying neurobiological alterations may independently cause the predisposition to epilepsy and comorbid processes. In this review we describe the structural and functional brain processes underlying the onset of neonatal epilepsy and its comorbidities.

La aparición de convulsiones es frecuente durante el periodo neonatal debido a las características de inmadurez funcional del cerebro es este periodo. La aparición de estas convulsiones puede llevar a un diagnóstico de epilepsia neonatal, que suele estar asociado a alteraciones estructurales del cerebro durante el neurodesarrollo. Aproximadamente el 50% de las personas con epilepsia activa padecen al menos un trastorno médico comórbido, y esto hace que cambie la evolución de la epilepsia. La presencia de trastornos neurológicos que preceden a la aparición de la epilepsia indica que alteraciones estructurales y/o funcionales del cerebro subyacentes pueden ser causa de la predisposición a padecer epilepsia y de los procesos comórbidos de manera independiente. En esta revisión describimos los procesos cerebrales estructurales y funcionales que subyacen a la aparición de epilepsia neonatal y sus comorbilidades.

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Resumen Las crisis convulsivas tienen una alta incidencia en la etapa neonatal, representando la principal manifes tación de disfunción neurológica. Ciertas condiciones fisiológicas del cerebro neonatal facilitan su aparición. Su diagnóstico puede ser un reto debido a que su semio logía no es tan clara comparado con niños mayores, y además, es necesario la confirmación por medio de EEG continuo o aEEG. Su reconocimiento oportuno es muy importante para un adecuado tratamiento y así evitar un impacto negative en el pronóstico a largo plazo. En la siguiente revisión, recapitulamos la fisiopatología, las causas y la clasificación de las crisis convulsivas neo natales, además de su correcto abordaje y las mejores opciones terapéuticas para su tratamiento dependiendo de la causa.

Abstract Seizures have a high incidence in the neonatal stage, being the main manifestation of neurological dysfunc tion. Certain physiological conditions of the neonatal brain facilitate its appearance. Its diagnosis can be a challenging because its semiology is not as clear as in older children, furthermore, confirmation by either EEG or aEEG is necessary. Its timely recognition is very im portant for adequate treatment and thus avoid a nega tive impact on the long-term outcome. In the following review, we recapitulate the pathophysiology, causes, and classification of neonatal seizures, as well as their correct approach and the best therapeutic options for their treatment depending on the cause.

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Seizures have a high incidence in the neonatal stage, being the main manifestation of neurological dysfunction. Certain physiological conditions of the neonatal brain facilitate its appearance. Its diagnosis can be a challenging because its semiology is not as clear as in older children, furthermore, confirmation by either EEG or aEEG is necessary. Its timely recognition is very important for adequate treatment and thus avoid a negative impact on the long-term outcome. In the following review, we recapitulate the pathophysiology, causes, and classification of neonatal seizures, as well as their correct approach and the best therapeutic options for their treatment depending on the cause.

Las crisis convulsivas tienen una alta incidencia en la etapa neonatal, representando la principal manifestación de disfunción neurológica. Ciertas condiciones fisiológicas del cerebro neonatal facilitan su aparición. Su diagnóstico puede ser un reto debido a que su semiología no es tan clara comparado con niños mayores, y además, es necesario la confirmación por medio de EEG continuo o aEEG. Su reconocimiento oportuno es muy importante para un adecuado tratamiento y así evitar un impacto negative en el pronóstico a largo plazo. En la siguiente revisión, recapitulamos la fisiopatología, las causas y la clasificación de las crisis convulsivas neonatales, además de su correcto abordaje y las mejores opciones terapéuticas para su tratamiento dependiendo de la causa.

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BACKGROUND: Seizures are the most common sign of neurologic dysfunction, reflecting a wide variety of central nervous system disorders. METHODS: A retrospective cross-sectional study of neonates with a clinical diagnosis of seizures was conducted in order to verify relationships between clinical aspects and EEG findings. Patients were divided into 3 groups according to the EEG recording available as: 1) with confirmatory ictal EEG; 2) with altered but non-ictal EEG; and 3) without any EEG recording. Variables related to pregnancy and birth history, neonatal complications, and seizure semiology (by video or clinical description) were compared to EEG findings. RESULTS: 97 neonates were included (39.1% preterm, 54.6% male), 71 with available EEG data (56.3% with ictal EEG). The group without EEG presented clinical characteristics significantly different from the others such as extreme prematurity, low birth weight, and higher neonatal mortality (P = 0.002, 0.001, and 0.003, respectively). The most common etiology was hypoxic-ischemic encephalopathy (HIE) (46.4%) followed by vascular disorders, which predominated in extremely preterm neonates (P = 0.006). Sequential seizure was the most common type (44.6%) and was more frequently identified in term neonates (46%). In 51.2% of the ictal recordings the main finding was electrographic seizure without clinical manifestation. Discharge using antiseizure medication was higher among those with ictal or altered non-ictal EEG (P < 0.001). CONCLUSIONS: HIE is still a frequently etiology for neonatal seizures. Even if the patients in the sample were not under continuous EEG, the substantial proportion of electrographic seizures without clinical manifestations detected suggests the importance of continuous EEG monitoring in neonates at increased risk of seizures.

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RESUMEN Introducción: la melanosis neurocutánea es un trastorno congénito no hereditario que se caracteriza por la asociación de nevus pigmentados múltiples o de gran tamaño y una excesiva proliferación de melanocitos en el sistema nervioso central. La incidencia es similar en ambos sexos, y se observa historia familiar de melanoma en un único caso. Presentación del caso: se trata de un neonato masculino que nace en Hospital General de Luanda en Angola, con mancha melánica gigante que se extiende desde el cuello, cara, tórax, abdomen, espalda y miembros superiores, requiere una vigilancia de las lesiones dérmicas y un control de las crisis convulsivas. Discusión: se realizaron revisiones de la literatura médica disponible sobre el tema, consultando el programa de genética Oxford, y se tomaron fotos de las características clínicas sobresalientes. Por lo general los síntomas neurológicos son de temprana aparición en la etapa neonatal o de lactante con presencia de convulsiones de difícil control, al crear un pronóstico reservado. Conclusiones: se considera importante el seguimiento del neurodesarrollo de forma multidisciplinario para intervención oportuna si fuera necesario.

ABSTRACT Introduction: neurocutaneous melanosis is a non-hereditary congenital disorder characterized by the association of multiple or large pigmented nevi and an excessive proliferation of melanocytes in the central nervous system. The incidence is similar in both sexes, and a family history of melanoma is observed in a single case. Case presentation: this is a male neonate born at the General Hospital of Luanda in Angola, with a giant melanic spot that extends from the neck, face, chest, abdomen, back and upper limbs, requires surveillance of dermal lesions and control of seizures. Discussion: reviews of the available medical literature on the subject were conducted, consulting the Oxford genetics program, and photos of outstanding clinical features were taken. Usually the neurological symptoms are of early onset in the neonatal or infant stage with the presence of seizures that are difficult to control, creating a reserved prognosis. Conclusions: it is considered important to monitor neurodevelopment in a multidisciplinary way for timely intervention if necessary.

RESUMO Introdução: a melanose neurocutânea é uma doença congênita não hereditária caracterizada pela associação de nevi pigmentado múltiplo ou grande e uma proliferação excessiva de melanócitos no sistema nervoso central. A incidência é semelhante em ambos os sexos, e um histórico familiar de melanoma é observado em um único caso. Apresentação do caso: trata-se de um recém-nascido no Hospital Geral de Luanda, em Angola, com um ponto melanico gigante que se estende do pescoço, rosto, tórax, abdômen, costas e membros superiores, requer vigilância de lesões dérmicas e controle de convulsões. Discussão: foram realizadas revisões da literatura médica disponível sobre o tema, consultando o programa de genética de Oxford e fotos de características clínicas de destaque. Geralmente os sintomas neurológicos são de início precoce no estágio neonatal ou infantil com a presença de convulsões de difícil controle, criando um prognóstico reservado. Conclusões: é considerado importante monitorar o neurodesenvolvimento de forma multidisciplinar para intervenção oportuna, se necessário.

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OBJECTIVE: To compare key seizure and outcome characteristics between neonates with and without cardiopulmonary disease. STUDY DESIGN: The Neonatal Seizure Registry is a multicenter, prospectively acquired cohort of neonates with clinical or electroencephalographic (EEG)-confirmed seizures. Cardiopulmonary disease was defined as congenital heart disease, congenital diaphragmatic hernia, and exposure to extracorporeal membrane oxygenation. We assessed continuous EEG monitoring strategy, seizure characteristics, seizure management, and outcomes for neonates with and without cardiopulmonary disease. RESULTS: We evaluated 83 neonates with cardiopulmonary disease and 271 neonates without cardiopulmonary disease. Neonates with cardiopulmonary disease were more likely to have EEG-only seizures (40% vs 21%, P < .001) and experience their first seizure later than those without cardiopulmonary disease (174 vs 21 hours of age, P < .001), but they had similar seizure exposure (many-recurrent electrographic seizures 39% vs 43%, P = .27). Phenobarbital was the primary initial antiseizure medication for both groups (90%), and both groups had similarly high rates of incomplete response to initial antiseizure medication administration (66% vs 68%, P = .75). Neonates with cardiopulmonary disease were discharged from the hospital later (hazard ratio 0.34, 95% CI 0.25-0.45, P < .001), although rates of in-hospital mortality were similar between the groups (hazard ratio 1.13, 95% CI 0.66-1.94, P = .64). CONCLUSION: Neonates with and without cardiopulmonary disease had a similarly high seizure exposure, but neonates with cardiopulmonary disease were more likely to experience EEG-only seizures and had seizure onset later in the clinical course. Phenobarbital was the most common seizure treatment, but seizures were often refractory to initial antiseizure medication. These data support guidelines recommending continuous EEG in neonates with cardiopulmonary disease and indicate a need for optimized therapeutic strategies.

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OBJECTIVE: We sought to characterize intracranial hemorrhage (ICH) as a seizure etiology in infants born term and preterm. For infants born term, we sought to compare seizure severity and treatment response for multisite vs single-site ICH and hypoxic-ischemic encephalopathy (HIE) with vs without ICH. STUDY DESIGN: We studied 112 newborn infants with seizures attributed to ICH and 201 infants born at term with seizures attributed to HIE, using a cohort of consecutive infants with clinically diagnosed and/or electrographic seizures prospectively enrolled in the multicenter Neonatal Seizure Registry. We compared seizure severity and treatment response among infants with complicated ICH, defined as multisite vs single-site ICH and HIE with vs without ICH. RESULTS: ICH was a more common seizure etiology in infants born preterm vs term (27% vs 10%, P < .001). Most infants had subclinical seizures (74%) and an incomplete response to initial antiseizure medication (ASM) (68%). In infants born term, multisite ICH was associated with more subclinical seizures than single-site ICH (93% vs 66%, P = .05) and an incomplete response to the initial ASM (100% vs 66%, P = .02). Status epilepticus was more common in HIE with ICH vs HIE alone (38% vs 17%, P = .05). CONCLUSIONS: Seizure severity was greater and treatment response was lower among infants born term with complicated ICH. These data support the use of continuous video electroencephalogram monitoring to accurately detect seizures and a multistep treatment plan that considers early use of multiple ASMs, particularly with parenchymal and high-grade intraventricular hemorrhage and complicated ICH.

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PURPOSE: The purpose of this study is to determine the frequency and radiological predictors of recurrent acute symptomatic seizures (RASS) and motor impairment at discharge after a neonatal arterial ischemic stroke (NAIS). METHODS: In a nonconcurrent cohort study, 33 full-term newborns with NAIS confirmed by MRI are admitted into our hospital between January 2003 and December 2012. Stroke size, calculated as stroke volume divided by whole brain volume (WBV), was categorized as > or < 3.3% of WBV. A univariate analysis of categorical variables was performed using Fisher's exact test. A multivariate analysis was performed using logistic regression models including all variables with a p value < 0.1 in the univariate analysis. RESULTS: The median age at NAIS was 2 days (IQR, 1-5.6), 36.4% were girls. The stroke size was > 3.3 of WBV in 48.5% of the cases, and 54.5% showed multifocal lesions. Involvement of the cerebral cortex (54.5%), thalamus (48.5%), posterior limb of the internal capsule (36.4%), basal ganglia (36.4%), and brainstem (28.2%) were found. At discharge, 45.5% of newborns had a motor deficit, and 27.3% had at least two seizures. Multivariate analyses revealed that stroke size > 3.3% of WBV (OR: 8.1, CI: 1.2-53.9) and basal ganglia involvement (OR: 12.8, CI: 1.7-95.4) predicted motor impairment at discharge. Cortical involvement of temporal and frontal lobes (OR: 14, CI: 2.2-88.1; and OR: 9.1, CI: 1.2-72.6) were predictive of RASS. CONCLUSION: Stroke size and location are independent risk factors for adverse short-term neurological outcomes in full-term newborns following a NAIS.

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Resumen: Introducción: La trombosis senovenosa cerebral neonatal (TSVC), es una patología rara y generalmente grave, de la cual se conoce poco sobre los mecanismos fisiopatológicos responsables y, aunque controvertido, se ha sugerido que la trombofilia genética, puede desempeñar un rol en la patogénesis. Debido a los temores de un sangrado intracraneal el tratamiento anticoagulante con heparina de bajo peso mole cular es controvertido. Objetivo: presentar un recién nacido con una trombosis senovenosa cerebral neonatal, discutir los factores de riesgo trombofílico, y el manejo con heparina de bajo peso molecu lar de la trombosis venosa cerebral. Caso Clínico: Recién nacido de término que debutó a los 8 días de vida con convulsiones clónicas, rechazo al pecho más hipoactividad motora. La neuroimagen con RM mostró una TSVC involucrando múltiples senos venosos, un infarto hemorrágico talámico dere cho y congestión venosa de la sustancia blanca frontal. El estudio de trombofilia puso de relieve una mutación homocigota del gen MTHFR C677T. El tratamiento con heparina de bajo peso molecular se asoció a repermeabilización del seno sagital superior a los 23 días de iniciada la terapia. Conclusio nes: La presentación clínica de la TSVC en el neonato es inespecífica, probablemente en relación con la extensión y gravedad de la lesión y el desarrollo de complicaciones asociadas, como infartos he morrágicos venosos intraparenquimatosos o hemorragia intraventricular. Estas complicaciones son detectables mediante Ecografia o Resonancia Magnética, y deben hacer sospechar una TSVC. En esta experiencia el tratamiento anticoagulante mostró ser seguro y prevenir la extensión de la trombosis.

Abstract: Introduction: Neonatal cerebral sinovenous thrombosis (CSNT) is a rare and generally serious con dition about which there is little knowledge of the responsible pathophysiological mechanisms and, although controversial, it has been suggested that genetic thrombophilia may play a role in its patho genesis. Out of concern for intracranial bleeding, the anticoagulant treatment with low-molecular- weight heparin is controversial. Objective: To present a case of a newborn with neonatal CSNT, to analyze the thrombophilic risk factors, and the management of cerebral venous thrombosis with low-molecular-weight heparin. Clinical Case: Full-term newborn who presented at eight days of life breastfeeding rejection, clonic seizures, and locomotor hypoactivity. The MRI neuroimaging showed a CSNT involving multiple venous sinuses, a right thalamic hemorrhagic infarction, and venous congestion in frontal white matter. Thrombophilia study highlighted a homozygous MTHFR C677T mutation. Treatment with low-molecular-weight heparin was associated with repermeabilization of the superior sagittal sinus after 23 days of starting therapy. Conclusions: The clinical presentation of CSNT in the neonate is nonspecific, probably related to the extent and severity of the injury and the development of associated complications, such as venous hemorrhagic infarctions and intraparenchymal or intraventricular hemorrhage. These complications are detected through ultrasound or MRI, and they should make us suspect a CSNT. In this experience, the anticoagulant treatment proved to be safe and prevents thrombus propagation.

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ABSTRACT Seizures in the newborn are associated with high morbidity and mortality, making their detection and treatment critical. Seizure activity in neonates is often clinically obscured, such that detection of seizures is particularly challenging. Amplitude-integrated EEG is a technique for simplified EEG monitoring that has found an increasing clinical application in neonatal intensive care. Its main value lies in the relative simplicity of interpretation, allowing nonspecialist members of the care team to engage in real-time detection of electrographic seizures. Nevertheless, to avoiding misdiagnosing rhythmic artifacts as seizures, it is necessary to recognize the electrophysiological ictal pattern in the conventional EEG trace available in current devices. The aim of this paper is to discuss the electrophysiological basis of the differentiation of epileptic seizures and extracranial artifacts to avoid misdiagnosis with amplitude-integrated EEG devices.

RESUMO Las convulsiones neonatales están asociadas a una alta morbi-mortalidad por lo que su correcto diagnóstico y tratamiento es fundamental. Las convulsiones en los recién nacidos son frecuentemente subclínicas lo que hace que su detección sea dificultosa. La electroencefalografía integrada por amplitud es una técnica de monitoreo electroencefalográfico simplificado que ha encontrado una creciente aplicación clínica en las unidades de terapia intensiva neonatales. Su principal ventaja es la relativa simplicidad de su interpretación lo que permite a personal no especializado del equipo neonatal diagnosticar convulsiones electrográficas en tiempo real. Sin embargo, para evitar diagnosticar erróneamente artefactos rítmicos como crisis epilépticas es necesario reconocer los patrones electrofisiológicos ictales en el EEG convencional disponible en los dispositivos actuales. El objetivo de este artículo es describir las bases electrofisiológicas para la diferenciación de convulsiones neonatales y artefactos extracraneanos para evitar errores diagnósticos con el uso de EEG integrado por amplitud.

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Neonatal seizures are common expression of acute brain injury in the perinatal period and could potentiate the degree of neuronal injury. The majority of events are electroencephalographic and the clinical seizures can be subtle and difficult to identify by medical personnel. Neonatal seizures are usually short and frequent at onset and have a tendency to subside after a short period. Continuous video-EEG monitoring is the gold standard to detect seizures, but amplitude integrated EEG is a useful tool when conventional EEG is not available. EEG monitoring is important not only to monitor frequency and duration of seizures but to provide important prognostic information.

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Las convulsiones neonatales son una expresión común de lesiones cerebrales agudas durante el periodo perinatal y podrían incrementar el daño neuronal. La mayoría son electroencefalográficas y las clínicas pueden ser sutiles y difíciles de identificar por el personal médico. Las convulsiones neonatales son usualmente cortas pero frecuentes al inicio y tienden a desaparecer en un periodo corto. El video-EEG continuo es el test ideal para detectar estas convulsiones, pero el EEG de amplitud es útil cuando el EEG convencional no está disponible. El monitoreo con EEG no solo es necesario para evaluar la frecuencia y duración de estas convulsiones, también puede proporcionar información pronóstica importante.

Neonatal seizures are common expression of acute brain injury in the perinatal period and could potentiate the degree of neuronal injury. The majority of events are electroencephalographic and the clinical seizures can be subtle and difficult to identify by medical personnel. Neonatal seizures are usually short and frequent at onset and have a tendency to subside after a short period. Continuous video-EEG monitoring is the gold standard to detect seizures, but amplitude integrated EEG is a useful tool when conventional EEG is not available. EEG monitoring is important not only to monitor frequency and duration of seizures but to provide important prognostic information.

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El periodo neonatal corresponde a una etapa en el desarrollo en el que las convulsiones constituyen la expresión clínica de disfunción del sistema nervioso central. Estas se manifiestan por una alteración en la función neurológica que puede ser motora, autonómica, de la conducta o una combinación de ellas. Dado que en este periodo el desarrollo anatómico, bioquímico y fisiológico, presentan características muy diferentes al desarrollo del niño mayor, las convulsiones pueden ser muy difíciles de identificar y pueden confundirse con eventos clínicos paroxísticos no epilépticos del recién nacido. Lo anterior se explica porque las convulsiones presentan patrones poco organizados, suelen no ser bien definidas y el registro electroencefalográfico es diferente al del niño mayor. En su mayoría las CN son secundarias a una etiología específica, por lo que es indispensable encontrar la causa lo cual se encuentra fuertemente relacionado al pronóstico. También es necesario diferenciar los eventos no epilépticos, para un óptimo manejo.

The neonatal period corresponds to a developmental stage in which seizures are the clinical expression of central nervous system dysfunction. These are manifested by a change in neurological function that can be motor, autonomic, behavior or a combination of them.Given that in this period the anatomical, biochemical and physiological development present with very different characteristics to those of the older child, seizures can be very difficult to identify, and can be confused with paroxysmal non epileptic clinical events of the newborn. This is explains why seizures have little organized patterns are often not well defined and the Electroencephalographic record is different from that found in the older child. For the most part, neonatal seizures are secondary to a specific etiology, so it is essential to find the cause, which is strongly related to the prognosis. It is also necessary to differentiate non-epileptic events, for optimal handling.

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OBJECTIVES: To determine whether certain characteristic electroencephalography (EEG) features are indicative of a genetic cause in early-life epilepsy. STUDY DESIGN: We enrolled a total of 100 patients with infantile-onset (<3 years) epilepsy due to known genetic cause (n = 50) and nongenetic cause (acquired, structural, or unknown, n = 50). The genetic group was classified into synaptopathies, channelopathies, mTOR (mammalian target of rapamycin)-opathies, and chromosomal abnormalities. The nongenetic group included epilepsy of unknown cause and structural abnormalities such as brain tumor, focal cortical dysplasia and encephalomalacia. The clinical features, magnetic resonance imaging, and video EEG obtained before 3 years of age and again at follow-up were reviewed. Specifically, the background rhythms and patterns of interictal epileptiform discharges were analyzed to define the EEG characteristics. RESULTS: The genetic group was more likely to have seizure recurrence beyond infancy and significant developmental delay (P <.01). The genetic and nongenetic groups showed different EEG patterns in the initial EEGs that persisted in follow-up EEGs. Diffuse slowing with pleomorphic focal/multifocal epileptiform discharges were present more often in the genetic (86%) compared with the nongenetic group (20%) in the initial EEGs (P <.01). The last available follow-up EEG features were similar (81% in genetic versus 17% in nongenetic) to the EEG performed prior to 3 years of age. CONCLUSIONS: Our findings suggest a simple guide for genetic screening in children with early-onset epilepsy. Genetic testing may be indicated and useful in infants with delayed development, no obvious cause, and significant EEG background slowing with pleomorphic focal or multifocal epileptiform discharges.

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We aimed to define determinants of duration of treatment for acute symptomatic neonatal seizures in a contemporary multicenter observational cohort study. After adjustment for potential confounders, only study site and seizure etiology remained significantly associated with the chance of continuing antiseizure medication after discharge to home.

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OBJECTIVE: To determine the contemporary etiology, burden, and short-term outcomes of seizures in neonates monitored with continuous video-electroencephalogram (cEEG). STUDY DESIGN: We prospectively collected data from 426 consecutive neonates (56% male, 88% term) ≤44 weeks' postmenstrual age with clinically suspected seizures and/or electrographic seizures. Subjects were assessed between January 2013 and April 2015 at 7 US tertiary care pediatric centers following the guidelines of the American Clinical Neurophysiology Society for cEEG for at-risk neonates. Seizure etiology, burden, management, and outcome were determined by chart review by the use of a case report form designed at study onset. RESULTS: The most common seizure etiologies were hypoxic-ischemic encephalopathy (38%), ischemic stroke (18%), and intracranial hemorrhage (11%). Seizure burden was high, with 59% having ≥7 electrographic seizures and 16% having status epilepticus; 52% received ≥2 antiseizure medications. During the neonatal admission, 17% died; 49% of survivors had abnormal neurologic examination at hospital discharge. In an adjusted analysis, high seizure burden was a significant risk factor for mortality, length of hospital stay, and abnormal neurological examination at discharge. CONCLUSIONS: In this large contemporary profile of consecutively enrolled newborns with seizures treated at centers that use cEEG per the guidelines of the American Clinical Neurophysiology Society, about one-half had high seizure burden, received ≥2 antiseizure medications, and/or died or had abnormal examination at discharge. Greater seizure burden was associated with increased morbidity and mortality. These findings underscore the importance of accurate determination of neonatal seizure frequency and etiology and a potential for improved outcome if seizure burden is reduced.

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Status epilepticus (SE), an acute condition characterized by repetitive or ongoing seizures activity, may produce long-term deleterious consequences. Previous data demonstrated that Wistar rats subjected to neonatal SE displayed autistic behavior, characterized by social play impairment, low preference by novelty, deficit in social discrimination; anxiety related behavior and stereotyped behavior with no changes in locomotor activity (doi: http://dx.doi.org/10.1007/s00702-010-0460-1, doi: http://dx.doi.org/10.3389/fnbeh.2013.00036, doi: http://dx.doi.org/10.1007/s00702-014-1291-2[1], [2], [3]). Taking into account the bi-directional relationship between the state of anxiety and social interaction (doi: http://dx.doi.org/10.1007/s10567-009-0062-3[4]), we evaluated the impact of the state of anxiety on social interaction. Male Wistar rats at postnatal day 9 were subjected to pilocarpine-induced neonatal SE (380 mg/kg, ip) and the controls received 0.9% saline (0.1 ml/10 g). The groups received saline or diazepam (1.0 mg/kg) 45 min prior each behavioral testing that started from 60 days of postnatal life. In the open field, rats subjected to neonatal seizure exhibited less central zone activity as compared to animals treated with diazepam, with no changes in the total locomotor activity. In elevated plus maze, rats subjected to neonatal seizure and treated with diazepam exhibited higher locomotor activity and spent more time on the open arms as compared to untreated animals. In approach phase of sociability paradigm, animals subjected to neonatal seizures similarly to controls, regardless the treatment, spent more time with social stimulus as compared to non social stimulus. In social novelty phase of sociability paradigm, animals subjected to neonatal seizures differently of controls, regardless the treatment, spent similar time with familiar and novel stimulus.

RESUMO

Objetivo: Analisar a gravidade da asfixia perinatal e sua evolução baseada na classificação de Sarnat & Sarnat. Métodos: Estudo descritivo observacional, longitudinal, prospectivo, iniciado em maio de 2009 a partir da avaliação de todo recém-nascido com diagnóstico clínico e/ou laboratorial de asfixia, nas unidades de Neonatologia de hospital pediátrico de nível terciário de atenção (Hospital Infantil Albert Sabin/Fortaleza/CE). A classificação de gravidade da asfixia de Sarnat foi correlacionada com as variáveis: idade gestacional, sexo, peso de nascimento, Apgar do quinto minuto, necessidade de cuidados de terapia intensiva, suporte de ventilação mecânica, sinais e sintomas clínicos na primeira semana de vida, presença de convulsões, uso de anticonvulsivantes, presença de mal-epilético e desfecho. Foi aplicada análise estatística simples pelos programas Excel e SPSS v17.0. Aplicação de teste do Quiquadrado nas análises bivariadas. Resultados: Foram analisados 200 neonatos com asfixia perinatal. A amostra contou com 67% de pacientes do sexo masculino, 92% a termo e 64% com peso superior a 2,5 kg. Cerca de 75% tinham registros de reanimação ao nascimento. A crise convulsiva precoce foi a manifestação neurológica mais referida (55%), sobretudo no primeiro dia de vida (70%). A encefalopatia leve foi mais prevalente (45%), seguida pela moderada (41%). As alterações sistêmicas mais registradas foram: infecção sistêmica, acidose metabólica, sangramento, bradicardia e hipoglicemia. A disfagia foi a alteração neurológica mais comum a longo prazo (84%). Conclusão: A classificação neurológica de Sarnat & Sarnat se associa com risco de convulsão, estado de mal convulsivo, hiper-reflexia, hipotonia e necessidade de terapia intensiva.