Abstract
We report a 12-year-old girl with double cortex syndrome. She was moderately retarded and had bilateral tonic seizures of upper extremities and atonic seizures of lower extremities with consciouness disturbances. An interictal EEG showed two foci of spikes, left frontpolar and left posteriortemporal.
She was hospitalized due to fluctuating disturbance of consciousness from a nearly normal to an almost unresponsive state. An electro-encephalogram recorded during the unresponsive state disclosed a diffuse but posteriorly predominant 4-5 Hz hypersynchronous waves that began as a 10-11 Hz focal rhythmic waves at left parietooccipital or frontal area. 2-3 Hz spike and wave complex, following right frontal dominant 12-13 Hz wave, sometimes accompanied this high voltage wave. This pattern continued for a few minutes and the consciousness returned to a nearly normal state as these waves disappeared. This complex partial status was composed of complex partial seisure for some minutes long and interictal state, in which the consciousness did not return to normal. These states were periodically repeated in a few minutes and were effectivey controlled by intravenous diazepam.
Although double cortex syndrome is a rare migratory disorder, it has often been reported together with intractable epilepsy. As far as we know, this is the first case of double cortex syndrome accompanied by complex partial status epilepticus.
