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Electrical status epilepticus during sleep (ESES) is an epileptic syndrome characterised by the presence of very persistent slow spike-wave-type epileptic discharges during non-REM sleep. The management of this pathology, today, is heterogeneous and no controlled studies have been conducted with the treatments employed; similarly, whether or not they improve patients' cognitive development or not has still to be determined.A review was carried out of the patients diagnosed with ESES at four hospitals over a period of 15 years; data concerning their clinical presentation, therapeutic management and clinical course were collected and compared with the literature.Altogether 29 patients with ESES were detected, 20 of them idiopathic and 26 generalised. The drugs with which the greatest control of the electrical activity was achieved were corticoids/adrenocorticotropic hormone (ACTH), clobazam and levetiracetam. In the primary cases ESES lasted an average of six months and the duration was twice that time in the secondary cases. Findings showed that the intelligence quotient remained normal in 45% of patients and 40% presented differing degrees of cognitive disability in the course of the pathology.The developmental neuropsychological prognosis is usually unfavourable and the cognitive development seems to be related with the duration of ESES and the area where the epileptic activity is concentrated, which suggests that the poor prognosis can be avoided if treatment is established at an early stage. The antiepileptic drugs that are most commonly used are valproic acid, ethosuximide and levetiracetam, and in our milieu clobazam and lamotrigine were commonly employed. The most effective drugs for controlling ESES were corticoids/ACTH, clobazam and levetiracetam.Estado epileptico electrico durante el sueño: estudio retrospectivo multicentrico de 29 casos.Introduccion. El estado epileptico electrico durante el sueño (ESES) es un sindrome epileptico caracterizado por la presencia de descargas epilepticas tipo punta-onda lenta de manera muy persistente durante el sueño no REM. En la actualidad, el manejo de esta patologia es heterogeneo y no hay estudios controlados con los tratamientos utilizados, ni se ha comprobado si estos mejoran la evolucion cognitiva de los pacientes. Pacientes y metodos. Se revisan los pacientes diagnosticados de ESES durante 15 años en cuatro centros hospitalarios, se recoge la presentacion clinica, el manejo terapeutico y la evolucion clinica, y se compara con la bibliografia. Resultados. Se seleccionaron 29 pacientes con ESES, 20 de ellos idiopatico y 26 de ellos generalizado. Los farmacos con los que se consiguio mayor control de la actividad electrica fueron los corticoides/hormona adrenocorticotropa (ACTH), el clobazam y el levetiracetam. La mediana de duracion del ESES en los casos primarios fue de seis meses, y en los secundarios, el doble. El 45% de los pacientes mantuvo un cociente intelectual normal y un 40% presento en la evolucion discapacidad cognitiva de diferente grado. Conclusiones. El pronostico neuropsicologico evolutivo suele ser desfavorable y la evolucion cognitiva parece estar en relacion con la duracion del ESES y el area donde este concentrada la actividad epileptica, lo que sugiere que el mal pronostico, si se trata precozmente, se puede evitar. Los antiepilepticos mas frecuentemente utilizados son el acido valproico, la etosuximida y el levetiracetam, y en nuestra muestra tambien se utilizaron con frecuencia el clobazam y la lamotrigina. Los farmacos mas eficaces para el control del ESES fueron los corticoides/ACTH, el clobazam y el levetiracetam. |
