Spontaneous remission of childhood epilepsy in two patients with focal extraopercular cortical dysplasia.
Childhood-onset partial epilepsy caused by focal cortical dysplastic lesions (FCDLs) is often severe. A few patients reported with a favorable outcome had a normal neuropsychological examination, and FCDLs were always localized around the opercular region, suggesting that extent and location of the lesion may account for the favorable outcome. We report two patients with extraopercular FCDLs, who had a spontaneous remission of their childhood-onset epilepsy, despite a severe neurological deficit. A 22-year-old girl (patient 1) and a 16-year-old boy (patient 2), began to have partial seizures at the age of 9 years and 1 year respectively. On neurological examination, patient 1 had left hemiparesis and patient 2 had low IQ. Interictal EEG recordings revealed repetitive epileptiform discharges involving the right temporo-parietal or frontal areas in patients 1 and 2 respectively. MRI study showed focal cortical thickening or abnormal gyration located over the right parietal and frontal region respectively in patients 1 and 2, but failed to evidence T2 prolongation in the white matter beneath the dysplastic cortex. Optimal antiepileptic regimen always stopped seizures. Their long-term course was favorable, with remission of the seizures and normalization of EEG recordings, even 4-5 years after medication withdrawal. In conclusion, FCDLs may cause epilepsy with a benign course even in patient with mental retardation or neurological abnormalities. This may be related to a morphologically milder dysplastic lesion than found in patients with FCDLs and severe epilepsy.
Gambardella
A
A
Institute of Neurology, School of Medicine of Catanzaro, Italy.
Le Piane
E
E
Oliveri
R L
RL
Zappia
M
M
Pardatscher
K
K
Quattrone
A
A
Aguglia
U
U
D000293:Adolescent; D000328:Adult; D002540:Cerebral Cortex / Q000002:abnormalities*; D004569:Electroencephalography; D004827:Epilepsy / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000523:psychology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011859:Radiography