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Focal Cortical Dysplasia in Pediatric Epilepsy

Choi SA, Kim KJ

Focal cortical dysplasia (FCD) is the most common cause of intractable focal epilepsy in children undergoing epilepsy surgery. In these patients, seizures usually develop in early childhood and are often...
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Treatment Outcomes of Vagus Nerve Stimulation in Lennox-Gastaut Syndrome

Kang BS, Woo YS, Lee J, Yi YY, Koo BS, Kang JW

PURPOSE: Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies and frequently patients with this syndrome respond poorly to antiepileptic drugs. The aim of this study was to...
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Treatment Outcomes of Vagus Nerve Stimulation in Lennox-Gastaut Syndrome

Kang BS, Woo YS, Lee J, Yi YY, Koo BS, Kang JW

PURPOSE: Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies and frequently patients with this syndrome respond poorly to antiepileptic drugs. The aim of this study was to...
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A Primer on Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Medically Refractory Epilepsy

Lee EJ, Kalia SK, Hong SH

Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial...
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The Surgical and Cognitive Outcomes of Focal Cortical Dysplasia

Choi SA, Kim KJ

Focal cortical dysplasia (FCD) is the major cause of intractable focal epilepsy in childhood leading to epilepsy surgery. The overall seizure freedom after surgery ranges between 50–75% at 2 years...
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Pathological Classification of Focal Cortical Dysplasia (FCD) : Personal Comments for Well Understanding FCD Classification

Kim SH, Choi J

In 2011, the International League against Epilepsy (ILAE) proposed a first international consensus of the classification of focal cortical dysplasia (FCD). This FCD classification had been widely used in worldwide....
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Mechanistic Target of Rapamycin Pathway in Epileptic Disorders

Kim JK, Lee JH

The mechanistic target of rapamycin (mTOR) pathway coordinates the metabolic activity of eukaryotic cells through environmental signals, including nutrients, energy, growth factors, and oxygen. In the nervous system, the mTOR...
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Normal and Disordered Formation of the Cerebral Cortex : Normal Embryology, Related Molecules, Types of Migration, Migration Disorders

Lee JY

The expansion and folding of the cerebral cortex occur during brain development and are critical factors that influence cognitive ability and sensorimotor skills. The disruption of cortical growth and folding...
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Antiepileptic Drug Withdrawal after Surgery in Children with Focal Cortical Dysplasia: Seizure Recurrence and Its Predictors

Choi SA, Kim SY, Kim WJ, Shim YK, Kim H, Hwang H, Choi JE, Lim BC, Chae JH, Chong S, Lee JY, Phi JH, Kim SK, Wang KC, Kim KJ

BACKGROUND AND PURPOSE: This study investigated the seizure recurrence rate and potential predictors of seizure recurrence following antiepileptic drug (AED) withdrawal after resective epilepsy surgery in children with focal cortical...
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Transient Abnormalities on Magnetic Resonance Imaging after Absence Seizures

Yoo HW, Yoon L, Kim HY, Kwak MJ, Park KH, Bae MH, Lee Y, Nam SO, Kim YM

Magnetic resonance imaging (MRI) is recommended for patients with epileptic seizures to rule out an underlying focal lesion. However, abnormalities in idiopathic generalized epilepsy, including childhood absence epilepsy, cannot usually...
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Discontinuing Antiepileptic Drugs after Pediatric Epilepsy Surgery for Focal Cortical Dysplasia

Seo SE, Kim SH, Kim DS, Kang HC, Lee JS, Kim HD

PURPOSE: Antiepileptic drugs (AEDs) can be discontinued in a subset of patients after surgery. We aimed to identify the factors related to successful AED withdrawal after surgery in pediatric patients...
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Malformations of cortical development: genetic mechanisms and diagnostic approach

Lee J

Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous...
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Epilepsy and Other Neuropsychiatric Manifestations in Children and Adolescents with 22q11.2 Deletion Syndrome

Kim EH, Yum MS, Lee BH, Kim HW, Lee HJ, Kim GH, Lee YJ, Yoo HW, Ko TS

BACKGROUND AND PURPOSE: 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome. Epilepsy and other neuropsychiatric (NP) manifestations of this genetic syndrome are not uncommon, but they are also...
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Megalencephaly-capillary Malformation Syndrome

Park SM, Kim GW, Cho HH, Kim WJ, Mun JH, Song M, Kim HS, Kim BS, Kim MB, Ko HC

  • KMID: 2046266
  • Korean J Dermatol.
  • 2015 Aug;53(7):546-551.
Megalencephaly-capillary malformation syndrome is a disorder characterized by megalencephaly or hemimegalencephaly, focal or generalized somatic overgrowth, and vascular malformations. Other characteristic features are neonatal hypotonia, hydrocephalus, developmental delay, syndactyly/polydactyly, frontal...
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Determining the Cause of Natural Death: A Case of Previously Unknown Sturge-Weber Syndrome

Kim MY, Kim H, Park S, Yang K, Park SH, Lee SD

In some cases, it is difficult to determine a single cause of death even after conducting full autopsy and additional tests. A 49-year-old man, reportedly having diabetes mellitus, was found...
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Molecular genetic decoding of malformations of cortical development

Lim JS, Lee JH

Malformations of cortical development (MCD) cover a broad spectrum of developmental disorders which cause the various clinical manifestations including epilepsy, developmental delay, and intellectual disability. MCD have been clinically classified...
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Emerging Surgical Strategies of Intractable Frontal Lobe Epilepsy with Cortical Dysplasia in Terms of Extent of Resection

Shin JH, Jung NY, Kim SP, Son EI

OBJECTIVE: Cortical dysplasia (CD) is one of the common causes of epilepsy surgery. However, surgical outcome still remains poor, especially with frontal lobe epilepsy (FLE), despite the advancement of neuroimaging...
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Happle-Tinschert Syndrome: Report of a Case with Hemimegalencephaly

Ozgur A, Cabuk G, Arpaci R, Baz K, Katar D

Happle-Tinschert syndrome is a disorder causing unilateral segmentally arranged basaloid follicular hamartomas of the skin associated with ipsilateral osseous, dental and cerebral abnormalities including tumors. Although a case with hemimegalencephaly...
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Disorganized Foliation of Unilateral Cerebellar Hemisphere as Cerebellar Cortical Dysplasia in Patients with Recurrent Seizures: A Case Report

Baek HJ

We present a rare case of abnormal foliation for one cerebellar hemisphere on MR imaging, showing vertically-oriented folia. Foliation of contralateral cerebellar hemisphere and other structures in the posterior fossa...
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Temporal lobe epilepsy surgery in children versus adults: from etiologies to outcomes

Lee YJ, Lee JS

Temporal lobe epilepsy (TLE) is the most common type of medically intractable epilepsy in adults and children, and mesial temporal sclerosis is the most common underlying cause of TLE. Unlike...
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