Epileptic Disorders
MENUStatus 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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Carlo Di Bonaventura, Francesco Mari, Alberto Pierallini, Oriano Mecarelli, Franco Randi, Mario Manfredi, Massimiliano Prencipe, Anna Teresa Giallonardo
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.