Status epilepticus in a patient with fragile X syndrome: electro-clinical features and peri-ictal neuroimaging.
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.
Di Bonaventura
Carlo
C
Department of Neurological Sciences, Rome, Italy. c_dibonaventura@yahoo.it
Mari
Francesco
F
Pierallini
Alberto
A
Mecarelli
Oriano
O
Randi
Franco
F
Manfredi
Mario
M
Prencipe
Massimiliano
M
Giallonardo
Anna Teresa
AT
D000293:Adolescent; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology; D002552:Cerebral Ventricles / Q000002:abnormalities / Q000473:pathology; D004351:Drug Resistance; D004569:Electroencephalography; D005600:Fragile X Syndrome / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013226:Status Epilepticus / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology