Occipital lobe epilepsy secondary to ulegyria

Abstract

To analyse clinical and therapeutic aspects of epilepsy secondary to ulegyria in adults. Out of 1,020 consecutive patients studied at a tertiary care epilepsy centre, eight cases of ulegyria were identified. All patients had comprehensive clinical evaluation, neuropsychological testing, interictal EEG, and brain magnetic resonance imaging (MRI). In addition, five patients had video-EEG monitoring. Ulegyria was confirmed by histological analysis in two patients who had successful epilepsy surgery. All patients had a history of perinatal asphyxia. In four of them there was psychomotor developmental delay. Mean age at onset of seizures was 5.8 years (range first week to 21 years). Brain MRI demonstrated predominant involvement of occipito-parietal cortical and subcortical areas. This posterior distribution of lesions was also supported by the presence of auras with occipital and parietal semiology in six patients, and signs of visuospatial dysfunction in five. Four patients had medically refractory epilepsy and two of them had significant improvement with surgical treatment. In this group of adult epileptic patients with ulegyria brain MRI, ictal semiology, and neurological examination are consistent with occipital lobe epilepsy. Most patients have severe epilepsy, but in some of them epilepsy can be controlled with antiepileptic drugs, while in others surgical treatment can be effective. Brain MRI criteria of ulegyria are well established, and in two cases it was possible to confirm their diagnosis with histological analysis.