A patient with the typical craniofacial features and clinical course of Miller-Dieker syndrome (MDS) was found on autopsy to have focal pachygyria rather than lissencephaly. The brainstem and cerebellum were hypoplastic, but thalami and basal ganglia were normal. We believe that MDS is a syndrome in which multiple specific pathways of neuronal migration are affected selectively, such as migration to the neocortex, migration via corpus pontobulbare, and cerebellar migration. However, another migration pathway (via corpus gangliothalamicum) is spared.

El tratamiento de la epilepsia en las últimas dos décadas ha experimentado grandes avances, que han sido sido más notables en la mejoría de las epilepsias refractarias. En este período, más que en ningún otro, han aparecido numerosos medicamentos con efectos antiepilépticos. Ello ha reducido el número de pacientes con epilepsia intratable y también ha mejorado la vida de muchos enfermos en los cuales el control de la epilepsia no ha sido total. Además de los nuevos medicamentos, y gracias a los avances tecnológicos, el tratamiento quirúrgico se ha establecido como un Tratamiento de la epilepsia por medio de la estimulación vagal L.A. Álvarez a,b , P. Dean a , P. Jayakar a,b , M. Duchowny a,b , T. Resnick a,b , C. Dunoyer a , S. Koh a VAGAL NERVE STIMULATION IN THE TREATMENT OF EPILEPSY Summary. Introduction. Vagal nerve stimulation is the latest therapeutic modality for the treatment of epilepsy. It consists of a lead implanted in the left vagal nerve which is connected to a subcutaneous stimulator implanted in the left axillary or pectorial region. Development. The stimulator is programmed to intermittently stimulate the vagal nerve throughout the day and a magnet also allows the patient to control the stimulation from the outside. This treatment has been used in patients with intractable partial seizures who are not candidates for epilepsy surgery. The results reported have varied but in general the procedure appears promising with at least 50% of the implanted having over 50% improvement in their seizure frequency and many having complete control without significant side effects. Conclusion. Further review of the results are still needed to fully determine the true value of this treatment and to identify the subgroups of patients which will benefit the most [REV NEUROL 1999; 29: 385-7].

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