The Developmental Outcomes of Infantile Spasm / 대한소아신경학회지

PURPOSE:Infantile spasm is the most important epilepsy syndrome that brings catastrophic results in childhood. Persistent spasms and hypsarrhythmia have been known to regress the brain maturation and development. Therefore, it is very important in these patients to find a way to achieve developmental progress as good as possible. The objective of this study was to compare the influence of various etiology on developemental outcome and to determine which therapy has a more favorable development outcome. METHODS:We reviewed 95 children diagnosed as infantile spasm between 1991 and 2005 at College of Medicine of Yonsei University and Sang-gye Paik Hospital. We compared possible factors to predict the developmental outcomes in terms of patient characteristics, etiology, seizure duration and seizure outcomes along with various treatment modalities such as antiepileptic drugs, steroid, ketogenic diet, and surgery. RESULTS:Mental retardation occured in 81(85.3%) of the patients with infantile spasms and 38(40.0%) suffered from propound mental retardation. In predicting the developmental outcomes, the most important factor was found to be the etiology. While only 13(56.5%) patients with cryptogenic infantile spasms had severe to profound mental retardation, 53(73.6%) patients with symptomatic etiologies did. Other factors shown to be associated with a good progress included high developmental scores at the onset, a short duration of spasms, an early effective control of spasms, early consideration of non-pharmacologic treatment such as ketogenic diet and surgery when the seizures filed to respond to antiepileptic drugs. CONCLUSION:This retrospective review suggests that it is possible to improve the developmental outcomes of infantile spasms by making correct etiologic diagnosis and providing early appropriate therapy chosen from variable treatment modalities.

The Cases of Surgical Treatment of Intractable Childhood Epilepsy with Tuberous Sclerosis / 대한소아신경학회지

Tuberous sclerosis(TS) is a disorder of aberrant neuronal differentiation and proliferation manifesting as multiple central nervous system(CNS) hamartomas. Seizures in patients with TS are often intractable to anticonvulsant therapy. Although TS shows various lesions including multiple tubers, many patients with well localized epileptic focus to some tubers may have a benefit from epileptic surgery. We experienced three cases who had clinical improvement after epileptic surgery in intractable epilepsy complicated by TS. We report three cases and the brief review of literatures.

An Analysis of Results of Surgical Treatment of Epilepsy at National Epilepsy Center in Japan / 대한간질학회지

PURPOSE: The neurosurgical group of the National Epilepsy Center in Japan has been operating on epileptic patients for 17years. In recent 10 years, The 387 patients that have been operated on have enjoyed positive results. in this study, the authors will attempts to make a scheme for surgical treatment of epilepsy based upon the analysis of the patients who were treated with surgery METHODS: THe authors investugated the influenced of advanced in diagnostic procedures. In order to study the relationship between the previous disease and the histologic results, we divided the 152 temporal lobe epilepsy patients who received a resection of both medial temporal structure and lateral neocortex two groups : those with hippocampal sclerosis and those with some other histologic finding. For analysis, patients were divided into 3 groups : first, a lesional group : second, a skip group that had no chronic intractanial recording : third, an intracranial recording group. The results at 2 years, 5 years, 8 years, and 10 years after surgery were studied based on Engel's classification RESULTS & CONCLUSION: With the development of better diagnostic equipment, the incidences of epilepsy surgery have increased, while the percentage of the cases requiring invasive diagnostic procedure has been decreased overall. Not only have the cases of surgery for temporal lobe epilepsy increased but also for extratemporal epilepsy as well. Mesial temporal sclerosis and cortical migration disorder were the most common lesions requiring operative treatment. Febrile status epilepticus in childhood was the most common etiology of epilepsy found. Eighteen children among the twenty-three who had been operated were lesional cases. Longterm follow up studies up studies up to 10 years after surgery shows no worsening in patients condition based on Ecgel's grade of temporal lobe epilepsy.

Epileptogenic Zones in Intractable Epilepsy Patients with Dysembryoplastic neuroepithelial tumor

BACKGROUNDS: A dysembryoplastic neuroepithelial tumor (DNT) is an important cause of the intractable epilepsy. The prognosis after epileptic surgery was excellent when the epileptogenic zones were completely excised. But, the exact epileptogenic zones in epilepsy patients with DNT are not well known. The purpose of this study was to investigate where the epileptogenic zones were in epilepsy patients with DNT. That results might be helpful in having good results of epileptic surgery of DNT. METHODS: Six patients with medically determined intractable epilepsy due to DNT were studied retrospectively. They had undergone epileptic surgery after video-EEG monitoring not only with scalp electrodes but also with invasive subdural electrodes. DNTs were located in the temporal lobe and pathologically proven in all patients. We analyzed distributions of ictal onset zones and irritative zones with non-invasive and invasive Video-EEG monitoring. Also, we reviewed the clinical features, neuroimaging features, neuropsychological tests, Wada tests and pathological findings. The operative strategy included complete resection of DNT, ictal onset and irritative zones in all patients. In two patients, modified operative strategy including amygdalohippocampectomy was done due to mesial involvement. A modified Engel's classification was used to determine surgical outcomes. RESULTS: The irritative zones (included only more than 10% of total interictal discharges during the monitoring) showed various patterns from multifocal to unobserved. Ictal onset zones were multiple in 5 patients (dual: 3 patients, triple: 2 patients). In 1 patient, contralateral hemispheric mirror focus was observed. Mesial temporal involvement was seen in 2 patients from EEG and in 1 patient from pathology. There were discrepancies between ictal onset zones and irritative zones in 4 patients. The EEG seizures without clinical events were recorded in 1 patient. Postoperatively 5 patients were free of seizure, 1 patient had rare seizures. CONCLUSION: Not only the wide or multiple distribution of epileptogenic zones around or in the lesion but also dual pathology of hippocampus are possible in epilepsy patients with DNT. The operative strategy including the epileptogenic zones can yield a good surgical outcome.