ABSTRACT
Resumen Los eventos paroxísticos no epilépticos (EPNE) se definen como episodios de aparición brusca y de breve duración que imitan a una crisis epiléptica, originados por una disfunción cerebral de origen diverso y a diferencia de la epilepsia no obedecen a una descarga neuronal excesiva. Su incidencia es mucho más elevada que la epilepsia y pueden aparecer a cualquier edad, pero son más frecuentes en los primeros años de vida. La inmadurez del sistema nervioso central en la infancia favorece que en este período las manifestaciones clínicas sean muy floridas y diferentes de otras edades. Fenómenos normales y comunes en el niño pueden también confundirse con crisis epilépticas. El primer paso para un diagnóstico correcto es establecer si este primer episodio corresponde a una crisis epiléptica o puede tratarse de un primer episodio de EPNE. Es importante seguir un protocolo de diagnóstico, valorando los antecedentes personales y familiares, sin olvidar el examen físico, analizar los posibles factores desencadenantes, los pormenores de cada episodio, si es posible un registro de los episodios, aplicar el sentido común y la experiencia y solamente proceder a los exámenes complementarios básicos como el registro EEG u otras exploraciones en caso de duda o para con firmación diagnóstica. En algunos casos se ha demostrado una base genética. Las opciones terapéuticas son escasas y la mayoría de EPNE tienen una evolución favorable.
Abstract Non-epileptic paroxysmal events (NEPE) are defined as episodes of sudden onset and short duration that mimic an epileptic seizure, caused by a brain dysfunction of diverse origin and, unlike epilepsy, are not due to excessive neuronal discharge. Its incidence is much higher than epilepsy and can appear at any age, but are more frequent in the first years of life. The immaturity of the central nervous system in childhood favors that in this period the clinical manifestations are more spectacular and different from other ages. Normal and common phenomena in children can also be confused with epileptic seizures. The first step for a correct diagnosis is to establish whether this first episode corresponds to an epileptic seizure or could be a first episode of NEPE. It is important to follow a diagnostic protocol, assessing the personal and family history, without forgetting the physical examination, analyzing the possible triggering factors, the details of each episode, if it's possible a record of the episodes, applying common sense and experience and only carrying out basic complementary tests such as EEG recording or others in case of doubt or for diagnostic confirmation. In some cases, a genetic basis has been demonstrated. Therapeutic op tions are scarce and the majority of NEPE have a favorable evolution.
ABSTRACT
Objective To study the clinical value of video-electroencephalogram (VEEG) on the diagnosis and prognosis of neonatal seizure.Method From January 2016 to December 2017,the medical records of 118 neonates who had seizure and received VEEG in our hospital were collected.The results of VEEG and medical records were analyzed using x2 test,Fisher's exact test or rank sum test.Result Among the 118 neonates,94 cases(79.6％) had abnormal VEEG results,including 59 mildly abnormal cases,21 moderately abnormal cases,and 14 severely abnormal cases.The characteristics of mildly abnormal VEEG was delayed mature,and moderately and severely abnormal VEEG were paroxysmal abnormal activities.All of the severely abnormal VEEGs showed abnormal background activities.The incidence of abnormal background activities of severely abnormal group was higher than mildly and moderately abnormal group,the difference was significant (P＜0.001).Neonates with abnormal background activities had higher rates of epileptic seizure and delayed maturation than those with normal background,and the differences were significant (P＜0.001).Among 32 neonates with paroxysmal events,17 cases had non-epileptic events including subtle seizures,myoclonus seizures,and symmnetrical tonic seizures;15 cases had epileptic electrographic seizures and electro-clinical seizures,12 cases had focal seizures.The degree of abnormal VEEG had positive correlations with the incidences of epileptic seizures and delayed maturation (P＜0.001).Conclusion Neonates with seizure has higher rate of abnormal VEEG.Non-epileptic events presents as subtle seizures and myoclonus seizures,and epileptic seizures as focal seizures.The background activities of neonatal VEEG has important predictive value for prognosis.The worse the VEEG is,the higher the possibility of epileptic seizure and delayed maturation.
ABSTRACT
Los eventos paroxísticos no epilépticos (EPNE) son una causa frecuente de consulta en pediatría. Constituyen un grupo de movimientos o conductas abruptas, recurrentes y estereotipadas que comprometen la función cerebral con recuperación espontánea a la normalidad. Se revisan las principales maneras de reconocerlos y diagnosticarlos. Palabras Claves: eventos paroxísticos, movimientos anormales, distonía, temblores.
Non-epileptic paroxysmal events (EPNE) are a frequent cause of consultation in Pediatrics. They are a group of abrupt, recurrent and stereotyped movements or behaviors that compromise brain function, with spontaneous recovery. We review the main ways to recognize and diagnose them. Key words: dystonia, paroxysmal events, abnormal movements, jitteriness.
ABSTRACT
Objetivo: mostrar los resultados de la introducción del video-electroencefalograma en la evaluación de los eventos paroxísticos neurológicos de naturaleza no precisada y/o en la epilepsia de difícil control en pacientes pediátricos. Métodos: se estudiaron 121 niños (edad 5,90 ± 4,34 años), divididos en 4 grupos atendiendo a la finalidad del estudio de video-electroencefalograma: I. Determinar la naturaleza (epiléptica o no) del evento paroxístico; II. Confirmar el síndrome epiléptico; III. Cuantificar las crisis epilépticas y IV. Estudiar candidatos para cirugía de la epilepsia. Resultados: la eficacia diagnóstica fue de un 86,77 %, y provocó modificaciones en el diagnóstico y/o el tratamiento inicial en un 32,38 %. Las modificaciones en el diagnóstico en el grupo I fueron en 16/43 pacientes (2 epilepsias, 7 trastornos del movimiento, 4 trastornos del sueño y 3 crisis psicógenas). En el grupo II se clasificaron 47 síndromes epilépticos (11 epilepsias focales, 31 epilepsias generalizadas y 5 indeterminadas). Para el grupo III el número de crisis registradas fue 7,31 ± 4,21, y el tiempo de registro del video-electroencefalograma fue de 3,86 ± 1,10 horas. En el grupo IV se estudiaron 2 pacientes, y se confirmaron como candidatos para hemisferectomía funcional. Conclusiones: el monitoreo por video-electroencefalograma ratificó ser una herramienta de gran valor y utilidad en el diagnóstico de los eventos paroxísticos neurológicos en niños, lo que incide en establecer una adecuada conducta terapéutica y contribuye a disminuir ciertas limitaciones en la aplicación de los criterios de la Liga Internacional contra la Epilepsia.
Objective: to show the results of the use of video electroencephalogram in the evaluation of undetermined neurological paroxysmal events and/or difficult epilepsy in pediatric patients. Methods: one hundred and 21 children (aged 5.90 ± 4.34 years), divided into 4 groups according to the objective of the video-EEG based study, were researched on. This study was aimed at 1) determining the nature of the paroxysmal event -either epileptic or not-; 2) confirming the epileptic syndrome; 3) quantifying the epileptic attacks; and 4) studying candidates for epilepsy surgery. Results: the diagnostic efficacy was 86.77 % and caused changes in diagnosis and/or in the initial treatment in 32.38 % of cases. Changes in diagnosis of group I occurred in 16 out of 43 patients (2 epilepsies, 7 movement disorders, 4 sleeping disorders and 3 psychogenic crises). Forty seven epileptic syndromes were classified in the second group (11 focal epilepsy, 31 generalized epilepsy and 5 undetermined). The number of recorded crises was 7.31 ± 4.21 in the third group and the video-EEG recording time was 3.6±1.10 hours. Two patients in the fifth group were studied and confirmed as candidates for functional hemispherectomy. Conclusions: video-electroencephalogram monitoring ratified that it is a very useful tool in the diagnosis of neurological paroxysmal events in children, which influences in setting a proper therapeutic behaviour and in assisting in the reduction of certain restrictions for the application of the International League against Epilepsy criteria.
ABSTRACT
La desviación paroxística de la mirada hacia arriba es un síndrome infantil descrito por primera vez en 1988 y desde entonces solo se registran alrededor de cincuenta reportes en el mundo. Su etiología no es clara y, aunque su pronóstico es variable, la mayoría de los informes señalan que durante el crecimiento los episodios tienden a disminuir en frecuencia y duración hasta desaparecer. El caso que se reporta corresponde a un niño de 16 meses de edad, quien desde los 11 meses presenta episodios de supraversión sostenida de la mirada conjugada de duración variable con flexión cervical compensatoria y nistagmo vertical a la fijación ocular sin alteración del estado de conciencia. Los episodios son de predominio matinal y se exacerban por situaciones de estrés como el ayuno o el insomnio; sin embargo, ceden con la conciliación del sueño. Los exámenes neurológico y oftalmológico resultan normales y a su vez las neuroimágenes y electroencefalograma no tienen hallazgos relevantes.
The paroxysmal upgaze deviation is a syndrome that described in infants for first time in 1988; there are just about 50 case reports worldwide ever since. Its etiology is unclear and though it prognosis is variable; most case reports indicate that during growth the episodes tend to decrease in frequency and duration until they disappear. It describes a 16-months old male child who since 11-months old presented many episodes of variable conjugate upward deviation of the eyes, compensatory neck flexion and down-beat saccades in attempted downgaze. These events are predominantly diurnal, and are exacerbated by stressful situations such as fasting or insomnia, however and improve with sleep. They have normal neurologic and ophthalmologic examination, and neuroimaging and EEG findings are not relevant.
O desvio paroxístico do olhar para cima é um síndrome infantil foi descrito por primeira vez no ano 1988 e desde então só se registram cerca de cinquenta reportes no mundo. Sua etiologia não é clara e, mesmo que seu prognóstico é variável, a maioria dos informes assinalam que durante o crescimento, os episódios tendem a diminuir em frequência e duração até desaparecer. O caso que é reportado corresponde a uma criança de 16 meses de idade, que desde os 11 meses, apresenta episódios de supraversão sustentada do olhar conjugado de duração variável com flexão cervical compensatória e nistagmo vertical à fixação ocular sem alteração do estado de consciência. Os episódios são de predomínio matinal e se exacerbam por situações de estresse como o jejum ou a insônia; no entanto, cedem com a conciliação do sono. Os exames neurológico e oftalmológico resultam normais e a sua vez as neuroimagens e electroencefalograma não têm resultados relevantes.