Epilepsia benigna de la infancia con punta centrotemporal
C. Casas-Fernández, T. Rodríguez-Costa
Bol. Pediatr. 2003; 43 (183): 32 - 45
Se lleva a cabo un análisis del síndrome epiléptico más frecuente en la
edad infantil, la Epilepsia Benigna de la Infancia con Punta
Centrotemporal (EBICT), conocida desde hace casi cincuenta años en
virtud de la escuela epileptológica francesa. Debe entenderse como el
ejemplo genuino de las epilepsias parciales benignas y sirve para
demostrar, asimismo, cómo no en todas las epilepsias deben emplearse
fármacos antiepilépticos (FAEs), pudiendo incluso ser éstos un factor
responsable del empeoramiento de las mismas, circunstancia que alcanza
un máximo interés en éste síndrome. Se exponen los razonamientos que
permiten plantear dudas sobre la aceptación del frecuentemente aceptado
pronóstico benigno del síndrome, dado que es conocida la posible
aparición de diferentes complicaciones evolutivas, que alcanzan su
máxima expresividad en un déficit de rendimiento y aprendizaje escolar,
junto con la aparición de crisis epilépticas de morfología inhabitual
en el síndrome clásico, habiendo descrito Aicardi. Se revisan los
criterios que configuran el fenotipo clínico y electroencefalográfico
de éste síndrome epiléptico, cuya etiología idiopática ha permitido
realizar investigaciones genéticas, iniciadas por Neubauer, con
aparición posterior de diferentes hallazgos que permiten considerar la
existencia de una heterogeneidad genética.
Abstract
An analysis is carried out on the most frequent syndrome in childhood
age, Benign Childhood Epilepsy with Centrotemporal Spikes (BECT), known
since almost fifty years ago because of the French epileptology school.
It should be understood as the genuine example of partial benign
epilepsies and also serve to demonstrate how antiepileptic drugs (AED)
should not be used in all epilepsies, these even being the factor
responsible for its aggravation, circumstance that reaches maximum
interest in this syndrome. The reasons that make it possible to pose
doubts on the acceptance of the frequently accepted benign prognosis of
the syndrome are explained, given that the possible appearance of
different evolutive complications are known. These reach their maximum
expressivity in a performance and scholastic learning deficit, together
with the appearance of epileptic episode of unusual morphology in the
classic syndrome, it being described by Aicardi. A review is made of
the criteria that shape the clinical and electroencephalographic
phenotype of this epileptic syndrome, whose idiopathic etiology has
made it possible to carry out genetic investigations, initiated by
Neubauer, with the later appearance of different findings that permit
the consideration of the existence of a genetic heterogeneity. Finally,
the differential diagnosis is analyzed and a discussion is opened on
the possible therapies, analyzing three aspects: a) the decision to
initiate or delay prolonged prophylactic drug treatment, b) the
evidence available regarding the efficacy of the different existing
AEDs, c) the different recommendations on the length of the drug
treatment, in the cases in which it has been initiated. It is concluded
with the affirmation of being faced with an epileptic syndrome usually
having good evolution, but in which the therapeutic measures on the one
hand and the natural evolution on the other are not exempt of offering
evolutive problems of a deeper neurologic significance and that should
be well known to solve them, minimize them and even avoid them.
Artículo completo (PDF) (76 kb.)
