John Libbey Eurotext

Epileptic Disorders

The Educational Journal of the International League Against Epilepsy

Occipital ulegyria causing epilepsy and visual impairment: an easily overlooked epilepsy syndrome Volume 19, numéro 4, December 2017

Illustrations

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Auteurs
Department of Neurology, Cork University Hospital, Cork, Ireland
* Correspondence: Antoinette O’Connor Department of Neurology, Cork University Hospital, Cork, Ireland
  • Mots-clés : ulegyria, cortical blindness, drug-resistant epilepsy
  • DOI : 10.1684/epd.2017.0948
  • Page(s) : 486-90
  • Année de parution : 2017

Ulegyria refers to scarring of the cerebral cortex usually arising from perinatal ischaemia. The scarring has a specific configuration in which small atrophic circumvolutions at the bottom of a sulcus underlie an intact spared gyral apex. This disconnection of overlying cortex may allow an “epileptogenic” island of cortex to generate seizures. Ulegyria is often associated with epilepsy and developmental delay, however, the syndromic association of visual impairment with epilepsy due to occipital ulegyria may not be recognised as a specific entity. Here, we report a series of five patients with occipital ulegyria who presented with widely variable seizure semiology and an array of visual deficits. In some patients, the link between the epilepsy and the visual impairment was not appreciated until they attended an epilepsy clinic.