Research advances in early-onset epileptic encephalopathy / 国际儿科学杂志

Early-onset epileptic encephalopathy (EEE) represents a group of devastating epileptic disorders that appear in neonatal or infantile period of life,characterized as pharmacoresistant generalized or focal seizures,severe electroencephalography (EEG) abnormalities,mental retardation and behavioral impairments.The interictal epileptic discharges are age-dependent and closely related to cognitive deterioration.EEE includes five epileptic syndromes,which are early myoclonic encephalopathy,ohtahara syndrome,and malignant migrating partial seizures in infancy,West syndrome and Dravet syndrome.The etiologies of EEE are highly heterogeneous,and most of them remain unknown.In many cases with EEE,seizures are resistant to treatment including anti-epileptic drugs and other methods.The prognosis for EEE is poor,and most of the children have severe mental retardation,some even are under the risk of sudden death.

Epilepsy and quality of life: socio-demographic and clinical aspects, and psychiatric co-morbidity / Epilepsia e qualidade de vida: aspectos sociodemográficos, clínicos e comorbidade psiquiátrica

To study socio-demographic and clinical aspects, as well as psychiatric co-morbidity that influence the quality of life of adult epileptic patients.

Estudar os aspectos sociodemográficos, clínicos e comorbidades psiquiátricas que influenciam a qualidade de vida de pacientes adultos com epilepsia.

Síndrome opercular o síndrome perisilviano / Operculum syndrome or perisylvian syndrome

El interés de los autores es llamar la atención sobre el síndrome opercular, y estimular con ello su identificación en la práctica neuropediátrica. Se realizó una búsqueda en PubMed desde febrero de 2005 hasta septiembre de 2010, y se comentaron los artículos que, a consideración de los autores, mostraban los diferentes aspectos del concepto, historia, características clínicas, causas, así como del diagnóstico, evolución y pronóstico. El síndrome opercular puede ser de causa congénita o adquirida; en los adultos es más frecuente por infarto cerebral opercular bilateral, no así en los niños, en los que se puede presentar por diferentes causas, desde trastornos de la migración neuroblástica, hasta en la epilepsia. En niños epilépticos se debe estar atento a su evolución, ya que tanto por el tipo de epilepsia o síndrome epiléptico, como por la medicación antiepiléptica usada, puede presentarse este síndrome, teniendo una gran significación su identificación rápida y tratamiento adecuado

The interest of authors is to attract attention on operculum syndrome and thus to stimulate its identification in the neuropediatric practice. A search in PubMed from February, 2005 to September, 2010 was made commenting on papers that according authors showed the different features of concept, history, clinical features, causes, as well as diagnosis, evolution and prognosis. The operculum syndrome may be congenital or acquired; ion adults is more frequent by bilateral operculum cerebral infarction, but not in children in whom it may be present by different causes, from neuroblast migration to epilepsy. In the case of epileptic children it is necessary to pay attention to its course since due to the type of epilepsy or epileptic syndrome or due to antiepileptic drug used, this syndrome may be present, considering very much its fast identification and appropriate treatment

Anesthetic experience of a patient with Ohtahara syndrome: A case report / 대한마취과학회지

Ohtahara syndrome (OS) is a rare epileptic encephalopathy that is characterized by an abnormal electroencephalogram (EEG) and intractable seizures in the neonatal and early infantile period. The patient of this reported case was delivered normally at 39 weeks of gestation without any complication. One week after birth, seizures that were refractory to anticonvulsants started with repetitive clustered tonic spasms. The child had no abnormal findings on the initial laboratory investigations. But he was diagnosed with OS according to the frequent tonic spasms, an abnormal EEG pattern of suppression-burst and magnetic resonance imaging of cortical dysplasia. He was planned to undergo an operation for brain lesion. This report describes our experience with the general anesthetic management when we performed craniotomy and right hemispherotomy for a patient with OS.

Epilepsias parciales benignas de la infancia: Correlación entre factores de riesgo con la evolución clínica y hallazgos electroencefalográficos y su aplicación en el establecimiento de criterios terapéuticos / Benign partial epilepsy of infancy: The Correlation between risk factors, clinical evolution and electroencephalographic findings and the application it has over the therapeutic criteria

Definición: Las epilepsias parciales benignas de la infancia (EPBI) representan el síndrome epiléptico mas común en niños en edad preescolar y escolar, correspondiendo al 15-24% de las epilepsias, diagnosticadas entre los 3 y 13 años. Remiten espontáneamente al llegar a la adolescencia. Las epilepsias rolándicas constituyen las EPBI de mayor incidencia, su principal característica es ocasionar crisis parciales, con anartria, hemiconvulsiones faciales, en algunos casos con hemigeneralización secundaria. El electroencefalograma interictal evidencia espigas focales centrotemporales. En el 40% de los casos existe historia familiar de convulsiones febriles, epilepsias clínicas, o descargas epilépticas en el electroencefalograma. Además, el 7-10% evidencian antecedentes personales de convulsiones febriles en su primera infancia. Algunos autores sugieren no medicar estos pacientes, sin embargo no existe un consenso al respecto.Objetivos:•Determinar si los antecedentes convulsivos familiares o personales representan una influencia en la evolución de la enfermedad y si su presencia es un criterio para iniciar medicación antiepiléptica. Proponer criterios terapéuticos de medicación y de observación.Metodología: Estudio monocéntrico, longitudinal, tipo cohorte histórico, realizado con pacientes de consulta externa del hospital Pediátrico Dr. Roberto Gilbert Elizalde, durante 3 meses (noviembre/2003 Enero/2004). Criterios de inclusión: convulsiones no febriles de inicio entre 3 y 13 años con neurodesarrollo normal, estudios de imágenes normales, examen neurológico normal y trazado electroencefalográfico compatible con EPBI. Se clasificó a los pacientes en dos subgrupos: con y sin antecedentes convulsivos familiares o personales, y se comparó las diferencias clínicas -en cuanto al intervalo interictal- y electroencefalográficas.

Definition: Benign partial epilepsy of infancy represents the most common epileptic syndrome in preschool and school children. It accounts for 15-24% of epilepsies diagnosed between the ages of 3 and 13 years. Rolandic epilepsy is one of BMEI with a high incidence its main characteristic is that it causes partial seizures, anartia, hemiseizures. The electroencephalogram shows centrotemporal spikes. In the 40% of the cases exist family history of febrile seizures, clinical epilepsy or epileptic discharges in the electroencephalogram. Also, 7-10% have a clinical history of febrile seizures in infancy. Some authors suggest to not medicate this patients. Objectives: XDetermine the family and history of seizures that can influence the evolution of the illness and if its presence is a criteria to start antiepileptic medication. Propose therapeutic criteria to medicate and of observation. Method: Monocentric, longitudinal, cohort study with patients that consulted Pediatric hospital of Dr. Roberto Gilbert Elizalde during the period of three months. (November 2003 V January 2004) Criteria to be included in this study: seizures without fever that began between the ages of 3 and 13 years of age with a normal neurodevelopment, normal image study, neurological exam and electroencephalogram that shows BMEI. Patients were classified in two subgroups: with or without family or clinical history of seizures and clinical differences were compared using.Results: Of the 57 patients 52 people were our study group out of which 67% were men and 33% were woman. The average of age was 9.26 years old +/- 2.99. The 63% of the patients had seizures while they were sleeping. Only 48% of the cases showed discharges are the electroencephalographic reading. Important clinical or electroencephalographic differences did not exist between the two groups. (with or with out clinical or family history).

Epilepsy : Epidemiology and Classification

Epilepsy is defined as the condition having the propensity of recurrent seizures and its clinical diagnosis is based on the two or more spontaneous seizures occurring more than 24 hrs apart or one episode of spontaneous seizure associated with an well defined brain pathology. There are two classification systems; one for the epileptic seizures and the other for the epilepsies and epileptic syndromes. The former is simple and clinically useful for the choice of antiepileptic drugs, however, it does not provide any further information regarding the etiology, prognosis, or other clinical characteristics of given patients. For that reason, the latter classification system is being more widely applied at the epilepsy community. However, the syndromic classification of epilepsies, which is based on the clustering of symptoms and signs occurring together, is still at its evolving stage and it should be regarded as the classification system developing into the etiological or pathogenesis-based classification. The incidence of epilepsy is quite variable in different regions, but usually around 40 to 70 cases per 100,000 annually in developed nations with bimodal peaks at the young children and the elderly over age 65. The recent trend of incidence was remarkable for a gradual reduction in young children, contrasted by a rapid rise in elderly population, which might be related to the rapid increase in the proportion of elderly in modern societies. The age-adjusted prevalence of epilepsies is around 0.5 to 1.0% with similar distribution patterns to the incidence. It has been estimated that about 4% of population is expected to develop epilepsy throughout the life time until the age of 80. The natural course of epilepsy is characterized by a spontaneous cure or prolonged remission in about 70% of patients over a prolonged follow-up period (50% without antiepileptic drug treatment and 20% with treatment). These findings clearly indicate that epilepsy is a treatable condition.

Syndromic Diagnosis at the Epilepsy Clinic: Role of MRI / 대한간질학회지

PURPOSE: To evaluate the application of MRI to the ILAE classification of epilepsies and epileptic syndromes in the setting of epilepsy clinic. METHODS: We reviewed epilepsy registry forms, EEG, and MRI of 300 patients who were consecutively registered to the Yonsei Epilepsy Clinic. The algorhithm of syndromic classification consisted of 3 steps ; 1) clinical diagnosis based on the clinical informations described in the registry form, 2) clinical-EEG correlations, and 3) clinical-EEG-MRI correlations. The interictal epileptiform discharges (IEDs) in EEG were divided into focal, multilobar/multifocal, and generalized. MRI-lesions were divided into focal and non-focal (multilobar/multifocal, and diffuse) lesions. The clinical-EEG, EEG-MRI, and MRI-clinical correlations were categorized as concordant, not discordant, and discordant. RESULTS: Among 300 patients evaluated, 249 patients were found to have epilepsies and both EEG and MRI. By clinical analysis, 190 of 249 patients were diagnosed as localization-related epilepsies (LRE), 24 patients were generalized epilepsies (GE), 34 patients were undetermined epilepsies (UDE), and one patient had alcohol related epilepsy. EEG revealed IEDs in 124 patients and altered the clinical diagnosis in 79 patients. MRI lesions were found in 106 patients with focal lesions in 65 patients and non-focal lesions in 41 patients. MRI lesions were found in 47 of 125 patients with negative EEG. Concordance rates of clinical-EEG, EEG-MRI, and MRI-clinical correlations in 54 patients with lobar epilepsies, who had positive EEG and MRI, were 39%, 54%, and 52%, respectively, and discordant rates were 17%, 11% and 7%, respectively. The complete concordance of all 3 correlations was found in only 33% of them. In 20 patients diagnosed as GE by clinical-EEG correlations, MRI lesions were found in only 3 patients and none of them changed the diagnostic categories due to MRI lesions. CONCLUSION: In lobar epilepsies, the sensitivity of MRI was quite comparable with EEG and the clinical-MRI correlation was superior to the clinical-EEG correlations. MRI provided additional and complimentary informations and should be incorporated to the ILAE-classification system as the category of 'lesional epilepsy'.