Effects of phenobarbital, potassium bromide and topiramate on intractable epilepsies in two cases of holoprosencephaly

We describe the courses of treatment for epilepsies in two patients with semilobar type holoprosencephaly (HPE); a 5-year-old girl and 30-year-old man. Seizure semiology included eye fixation, upward or lateral gaze, horizontal vertical nystagmus, eyelid flutter, grinning facial distortion, which frequently evolved to generalized tonic convulsions. In both patients, ictal electroencephalography revealed seizure onset rhythmic activity bilateral frontopolar areas. Administration phenobarbital subsequent elevation blood levels 30–40 μg/ml alleviated clustered seizures. This allowed dosage reduction other antiepileptic drugs that had sedative and/or respiratory adverse effects causing significant distress these patients. Potassium bromide 20–50 mg/kg/day topiramate 1–3 were also beneficial A combination regimen three could be promising option intractable epilepsy HPE.