Abstracts

Rationale: Early Infantile Epileptic Encephalopathy (EIEE) has a bad prognosis. Seizures almost never respond to medical treatment, psychomotor development is severely impaired, mortality is high. There are a few case reports of surgical treatment only after months of medical treatment failure. Seizure control and development improved after these surgeries. Realizing that medical treatment has failed to produce a good outcome, we recommended and performed surgery as soon as possible for infants with unilateral developmental cortical abnormalities and EIEE. We report our experience with 3 infants who have undergone early surgical treatment. Methods: Two girls, 1 boy, all had tonic spasms in clusters, 150 to over 300 / day, who met diagnostic criteria for EIEE underwent early surgery. While being evaluated for surgery, they received medical treatment with little benefit (table 1). All 3 infants had a modified hemispherectomy with removal of the pre-M1, subcollosal gyri and the insula. Results: All 3 are seizure free since surgery and have improved development. Patients 1 and 3 have fraternal twins. Parents report that they are now behaving similar to their twin and development has almost "caught up". All three have hemianopsia and prefer to use the hand contralateral to the good hemisphere. Surprisingly, all three have finger flexion-extension grip and release, and independent movement of fingers in the hand contralateral to the hemispherectomy. Postoperative EEG shows a normal wake and sleep pattern over the good hemisphere and a less well organized suppression-burst pattern over the remaining hemispherectomy side. Pathology shows cortical dysplasia in all three. Conclusions: Early surgical treatment of EIEE in selected cases has been effective for seizure control, improvement in alertness, and development. Although it is too soon and these infants are too young to predict ultimate developmental outcome, they appear to be much better than when they were having 200 or more clinical seizures per day and suffering from continuous electrographic epileptic encephalopathy. The use of the fingers on the hand contralateral to the hemispherectomy is an unexpected benefit.