ABSTRACT

In this case report, we presented a patient with Sturge-Weber syndrome manifesting atonic seizures. A thirty four year old man was admitted to our hospital with frequent seizures. His neurological examination revealed moderate cognitive decline, right sided hemangioma on ophthalmic division of trigeminal nerve, and telangiectasias at lower extremities. Glaucoma, optic atrophy and blindness in the right eye, decrease vision in the left eye and right sided central facial paralyze were detected. The observed seizures were often atonic, sometimes absence. On magnetic resonance imaging (MRI), there were atrophy and gliosis on the right parieto-occipital region, and angiography revealed multiple venous angiomas in the deep part of the left side frontal lobe, electroencephalography showed an epileptic activity in the right posterior-central temporal region. Seizures were partially controlled with valproic acid and topiramate politherapy.