John Libbey Eurotext

Status epilepticus in a patient with fragile X syndrome: electro-clinical features and peri-ictal neuroimaging Volume 8, issue 3, September 2006


See all figures

Department of Neurological Sciences, Neuromed Institute of Pozzilli (IS), University of Rome “La Sapienza”, San Raffaele IRCCS, Rome, Italy

Fragile X syndrome (Fra-X) is a common cause of mental retardation that can be associated with partial epilepsy characterized by a variety of electro-clinical features. A wide spectrum of interictal activities are reported, although no data regarding ictal EEG activity have as yet been published. Drug-resistant seizures are uncommon, and the occurrence of clustering seizures or status epilepticus has only been reported anecdotally. We describe a Fra-X patient with refractory partial epilepsy related to a malformation of cortical development who experienced a partial status epilepticus that was well documented by video-EEG and MRI. We report the electro-clinical features and peri-ictal neuroimaging data.