Epilepsy affects 0.5% of the population. A 25% of the patients referred to epilepsy services have paroxysmal clinical events associated with motor activities, sensory or emotional alterations, or consciousness impairment, which are not epileptic seizures. The non-epileptic seizures can be classified as psychogenic, which do not have a medical cause, and are associated with primary or secondary psychological problems, and as of physiological origin. The non-epileptic events are manifested by paroxysmal or repetitive behaviors that might be confused with epileptic seizures. The diagnosis is very important in order to avoid unnecessary tests and iatrogenic therapies. This study will present clinical cases and review of the main non-epileptic physiological events (sleep disorders, movement disorders, hypoxic-ischemic phenomena) and psychogenic events (somatoform disorders, fictitious disorders and anxiety disorder).

La epilepsia afecta al 0.5% de la población y el 25% de los pacientes derivados a los servicios de epilepsia presentan eventos clínicos paroxísticos asociados con actividades motoras, alteraciones sensitivas, sensoriales, emocionales o afectación de la conciencia, que no son crisis epilépticas. Las crisis no epilépticas pueden ser clasificadas como psicogénicas, aquellas que no tienen causa médica y se asocian a problemas psicológicos primarios o secundarios, y aquellas de origen fisiológico. Estos eventos no epilépticos se manifiestan por comportamientos paroxísticos o movimientos repetitivos y que se confunden con crisis epilépticas. El diagnóstico es muy importante para evitar pruebas innecesarias y terapias iatrogénicas. En este estudio serán presentados once casos clínicos y la revisión de los principales eventos no epilépticos fisiológicos (disturbio del sueño, trastornos motores paroxísticos), fenómenos hipóxico isquémicos y psicogénicos (trastornos somatoformes, trastornos facticios y trastorno de ansiedad) que se manifiestan en niños y adolescentes.

Aim: To review the syncope in infants and adolescents as well as its etiology, clinical manifestations, physiopathology, diagnostic methods and treatment. Development: The syncope is a clinical entity characterized by sudden and transient loss of consciousness and postural tone, with a quick and complete recovery. The syncope is frequent in children and adolescents. The most common cause is neurocardiogenic (vasovagal), which has a benign evolution. Other causes may be neurological, cardiac and metabolic diseases, with a variable prognosis, which may come to be severe and potentially lethal. Evaluation of the syncope is mainly based on present clinical history, and on a detailed clinical examination. Basic complementary exams may help on the diagnosis, as a glycemia test, determination of electrolytes (sodium, potassium, magnesium), a hemogram and an electrocardiogram. The Tilt Test is "the" test to confirm the neurocardiogenic syncope. Treatment is indicated in syncopes with recurrent episodes or with risk of physical lesions. Prevention and education are indicated for all patients. Specific treatment should be individually given to each patient. In the case of neurocardiogenic syncope, beta-blockers, alpha-adrenergic stimulants, serotonin reuptake inhibitors, and fludrocortisone are used. In the frequently recurrent neurocardiogenic syncope, when preventive actions are not enough or there is risk of lesions in consequence of falls, beta-blockers, alpha-adrenergic stimulants, serotonin reuptake inhibitors, and fludrocortisone are used, as well as a pacemaker for severe cases non-responsive to pharmacological treatment. Conclusion: The syncope is a frequent clinical entity in infancy and adolescence, with benign evolution but potentially lethal. Diagnosis and correct etiology are important for the implementation of specific and early treatment.

Objetivo. Revisar el Síncope en la infancia y adolescencia, su etiología, manifestaciones clínicas, fisiopatología, métodos diagnósticos y tratamiento. Desarrollo. El síncope es una entidad clínica caracterizada por una alteración o pérdida abrupta y transitoria de la conciencia y del tono postural, con recuperación espontánea y completa. El síncope es frecuente en niños y adolescentes. La causa más frecuente es el neurocardiogénico (vasovagal) que presenta una evolución benigna. Otras causas son las enfermedades cardíacas, neurológica, metabólica, psicológica o psiquiátrica e intoxicaciones con pronósticos variables, pudiendo llegar a ser severas y ocasionalmente fatales. La evaluación del síncope se basa principalmente en una buena historia clínica y un examen clínico detallado. Pruebas complementarias básicas pueden ayudar en el diagnóstico, como estudios metabólicos, hemogramas y electrocardiograma. La prueba de Inclinación es la prueba por excelencia para confirmar el diagnóstico del Síncope Neurocardiogénico. El tratamiento está indicado en síncopes con episodios recurrentes o con el riesgo de lesiones. La prevención y las medidas educacionales están indicadas en todos los pacientes con síncope. Un tratamiento específico debe ser indicado para cada paciente individualmente. En el Síncope Neurocardiogénico con recurrencia frecuente, donde fallan las medidas preventivas o hay riesgo de lesiones con las caídas, son utilizados fármacos como los beta bloqueantes, estimulantes alfa adrenérgicos, inhibidores de la captación de serotonina, fludrocortisona y marcapaso para los casos severos que no responden a los tratamientos farmacológicos. Conclusiones. El Síncope es una entidad clínica frecuente en la infancia y adolescencia con evolución benigna, pero potencialmente letal en los casos de origen cardíaco. Es importante el diagnóstico, y la etiología para elegir el tratamiento específico y precoz.

O presente estudo comparou achados de tomografia computadorizada (TC) com aspectos clínicos de pacientes com esquizencefalia. Sete crianças eram meninas e quatro meninos. Seis apresentavam tetraparesia, três hemiparesia e um não tinha déficit motor. Seis eram epilépticos. Dez apresentavam atraso do desenvolvimento neuropsicomotor. De acordo com os achados encontrados na TC, sete pacientes apresentaram fendas bilaterais e quatro defeito unilateral. Oito tinham fendas de lábios abertos e quatro de lábios fechados. A anomalia associada mais comum foi a ausência de septo pelúcido (n=9), seguida por nódulos subependimários (n=4), hidrocefalia (n=2) e microcefalia (n=1). Embora a ressonância magnética seja o exame padrão-ouro para diagnosticar anomalias de migração neuronal, a TC é mais disponível e pode ser útil no diagnóstico das esquizencefalias, se os aspectos típicos forem reconhecidos.

A retrospective study comparing clinical and computerized tomography (CT) in 11 patients diagnosed as having schizencephaly was conducted. Seven of these patients were girls and four boys. Six of them had tetraparesis, three hemiparesis and one no motor deficits. Six had epilepsy and ten developmental delay. On CT examinations, 7 patients were found as having bilateral clefts and four unilateral defect. Eight had opened lip clefts and four had a closed lip defect. The commonest associated anomaly was an absent septum pellucidum (n=9), followed by subependymal nodules (n=4), hydrocephalus (n=2) and microcephaly (n=1). Despite magnetic resonance image is the gold-standard to diagnose neuronal migration anomalies, CT can be useful in showing typical aspects of schizencephaly.

Morte súbita inexplicada e inesperada em epilepsia (SUDEP) é responsável por 2 a 17% das mortes em pacientes epilépticos. Este estudo visa determinar as circunstâncias de SUDEP e os achados de autópsia destes pacientes. Foram identificados 53 pacientes cuja causa de morte foi associada a crise epiléptica; em 30 destes, parentes ou amigos foram entrevistados quanto às circunstâncias das mortes e outros aspectos. Também foram revisados os certificados de óbito destes pacientes. Foram encontrados 20 casos de SUDEP. A maioria foi encontrada morta na sua cama por parentes, sem evidência de crise convulsiva, e a causa de morte foi edema cerebral e\ou pulmonar. A incidência e o risco relativo de SUDEP só podem ser adequadamente definidos se houver autópsia em todas as pessoas que morrem subitamente. Um consenso sobre SUDEP permitiria maior acurácia aos dados de mortalidade nacional.

Sudden unexpected, unexplained death in epilepsy (SUDEP) has been reported to be responsible for 2 to 17% of all deaths in patients with epilepsy. This study was conducted to determine the circumstances of SUDEP and the autopsy findings in these patients. Fifty-three individuals whose cause of death was related to epilepsy were identified and in 30 cases relatives or friends were interviewed about the circumstances of death and other information which allowed to classify the patients as SUDEP or not. The death certificates were also reviewed. We found 20 cases of SUDEP. Most of them were found dead lying on the bed with no evidence of seizure event, and most of them had pulmonary and/or cerebral edema as the cause of death. The incidence and the risk of SUDEP can only be fully ascertained if all sudden deaths had postmortem examination. Consensus in certifying SUDEP cases would allow better accuracy in national mortality rate.