Surgical Treatment of an Early Epileptic Encephalopathy with Suppression‐Bursts and Focal Cortical Dysplasia
- 1 January 1995
- Vol. 36 (1) , 37-40
- https://doi.org/10.1111/j.1528-1157.1995.tb01662.x
Abstract
A case of early epileptic encephalopathy (EIEE) with suppression-bursts or Ohtahara's syndrome, associated with focal cortical dysplasia is reported. Infantile spasms and brief tonic unilateral seizures began on the fifth day of life. Interictal EEG demonstrated an asymmetrical "suppression-burst" pattern with no wake or sleep cycling. Seizures were refractory to all antiepileptic drug (AED) and steroid therapy. Magnetic resonance imaging (MRI) showed right frontotemporal cortical thickening. After three weeks of an ineffective medical treatment a preoperative evaluation with single photon emission computed tomography (SPECT) and electrocorticography (ECoG) was performed to characterize epileptogenic focus. Surgical resection of the precentral area was performed at age 1 month. Neuropathologic examinations confirmed diagnosis of focal cortical dysplasia by identifying cytoarchitectural disarray and ectopic neurons located deep in subcortical white matter. During follow-up, 1-year postoperative the child had a single febrile seizure. Neurologic examination showed minor developmental delay and slight left-sided weakness.Keywords
This publication has 16 references indexed in Scilit:
- Facilitation of Infantile Spasms by Partial SeizuresEpilepsia, 1993
- Neuropathologic findings in cortical resections (including hemispherectomies) performed for the treatment of intractable childhood epilepsyActa Neuropathologica, 1992
- Topical Review Article: The Age-Dependent Epileptic EncephalopathiesJournal of Child Neurology, 1992
- Focal neuronal migration disorders and intractable partial epilepsy: Results of surgical treatmentAnnals of Neurology, 1991
- Focal neuronal migration disorders and intractable partial epilepsy: A study of 30 patientsAnnals of Neurology, 1991
- Focal resection for malignant partial seizures in infancyNeurology, 1990
- Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatmentAnnals of Neurology, 1990
- Intracranial tumors and West syndromePediatric Neurology, 1989
- Lesions focales dans les spasmes infantilesNeurophysiologie Clinique, 1988
- EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY WITH SUPPRESSION BURST: OHTAHARA SYNDROMEDevelopmental Medicine and Child Neurology, 1987