John Libbey Eurotext

Epileptic Disorders

The Educational Journal of the

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

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Authors
Department of Neurological Sciences, Neuromed Institute of Pozzilli (IS), University of Rome “La Sapienza”, San Raffaele IRCCS, Rome, Italy
  • Key words: fragile X syndrome, status epilepticus, partial epilepsy, peri-ictal neuroimaging, cortical dysplasia
  • Page(s) : 195-9
  • Published in: 2006

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.