Seok-Ho Hong

Surgical treatment for localization-related infantile spasms: excellent long-term outcomes.

OBJECTIVE Focal epileptogenic lesions can manifest as infantile spasms, a catastrophic type of epilepsy. Although early surgery for catastrophic epilepsies has shown positive effects, little is known regarding long-term outcomes. The present study examined long-term outcomes in patients with localization-related infantile spasms treated surgically. METHODS Data from localization-related infantile spasm cases treated surgically between 1998 and 2002 at the Asan Medical Center were retrospectively reviewed. Presurgical evaluation, surgery, postoperative seizure frequency and developmental outcome data were analyzed. RESULTS Five patients met the inclusion criteria, and had etiologies of tuberous sclerosis, fetal infection, encephalomalacia, malformation of cortical development and low-grade astrocytoma, respectively. The mean seizure onset age was 5.4 months (range, 3 days to 11 months), and the mean age at surgery was 19 months (range, 9-29 months). Two patients underwent a functional hemispherectomy, and the remaining three underwent lesionectomy or temporal lobectomy. The follow-up duration was 6-9 years. We found that following surgery, 4 of the 5 patients were seizure-free at the final follow-up. The 2 patients with low grade astrocytoma and cortical dysplasia, respectively showed relatively good developmental outcomes. CONCLUSION Surgery may be an excellent option for treating selected patients with infantile spasms due to unilateral or focal congenital or early-acquired cortical lesions. However, developmental outcomes appear to be strongly linked to etiology and the pre-operative developmental level.

Long-term outcomes of hemispheric disconnection in pediatric patients with intractable epilepsy.

Background and Purpose Hemispherectomy reportedly produces remarkable results in terms of seizure outcome and quality of life for medically intractable hemispheric epilepsy in children. We reviewed the neuroradiologic findings, pathologic findings, epilepsy characteristics, and clinical long-term outcomes in pediatric patients following a hemispheric disconnection. Methods We retrospectively studied 12 children (8 males) who underwent a hemispherectomy at Asan Medical Center between 1997 and 2005. Clinical, EEG, neuroradiological, and surgical data were collected. Long-term outcomes for seizure, motor functions, and cognitive functions were evaluated at a mean follow-up of 12.7 years (range, 7.6-16.2 years) after surgery. Results The mean age at epilepsy onset was 3.0 years (range, 0-7.6 years). The following epilepsy syndromes were identified in our cohort: focal symptomatic epilepsy (n=8), West syndrome (n=3), and Rasmussens syndrome (n=1). Postoperative histopathology of our study patients revealed malformation of cortical development (n=7), encephalomalacia as a sequela of infarction or trauma (n=3), Sturge-Weber syndrome (n=1), and Rasmussens encephalitis (n=1). The mean age at surgery was 6.5 years (range, 0.8-12.3 years). Anatomical or functional hemispherectomy was performed in 8 patients, and hemispherotomy was performed in 4 patients. Eight of our 12 children (66.7%) were seizure-free, but 3 patients with perioperative complications showed persistent seizure. Although all patients had preoperative hemiparesis and developmental delay, none had additional motor or cognitive deficits after surgery, and most achieved independent walking and improvement in daily activities. Conclusions The long-term clinical outcomes of hemispherectomy in children with intractable hemispheric epilepsy are good when careful patient selection and skilled surgical approaches are applied.

A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.

Staged Total Callosotomy for Lennox-Gastaut Syndrome: A Case Report

We report a case with Lennox-Gastaut syndrome (LGS) who underwent staged total callosotomy with a favorable outcome. A 6-year-old boy began having myoclonic seizures at the age of 8 months and was diagnosed with LGS when he was 27 months old. Various antiepileptic drugs and a ketogenic diet failed to control his multiple types of seizures. At the age of 36 months, he underwent corpus callosotomy and achieved an immediate, post-operative seizure free state. However, 3 months later, various seizures relapsed and were refractory to additional vagus nerve stimulation. Remaining callosal fibers in the splenium noted on post-operative diffusion tensor imaging made us to perform a second operation, total callosotomy. The patient finally achieved a seizure-free state with electroencephalography (EEG) normalization noted after the staged total callosotomy.