A case of Ohtahara syndrome with olivary-dentate dysplasia and agenesis ofmamillary bodies

We report a patient with early infantile epileptic encephalopathy (EIEE) wi th suppression-burst (Ohtahara syndrome) associated with olivary-dentate dy splasia and agenesis of mamillary bodies is reported. Although those with O htahara syndrome are a heterogeneous group, virtually all reported cases. a re secondary to neuronal migrational disorders, sometimes only identified b y detailed neuropathologic examination, as in this case report, which descr ibes mamillary body agenesis as a not-yet-recognized anomaly associated wit h Ohtahara syndrome. All children with Ohtahara syndrome should have high-r esolution magnetic resonance imaging (MRI) and detailed postmortem neuropat hologic examinations.