• Clinical and Experimental Pediatrics
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• v.55 no.9
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• pp.316-321
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• 2012

The ketogenic diet has been widely used and proved to be effective for intractable epilepsy. Although the mechanisms underlying its antiepileptic effects remain to be proven, there are increasing experimental evidences for its neuroprotective effects along with many researches about expanding use of the diet in other neurologic disorders. The first success was reported in glucose transporter type 1 deficiency syndrome, in which the diet served as an alternative metabolic source. Many neurologic disorders share some of the common pathologic mechanisms such as mitochondrial dysfunction, altered neurotransmitter function and synaptic transmission, or abnormal regulation of reactive oxygen species, and the role of the ketogenic diet has been postulated in these mechanisms. In this article, we introduce an overview about the expanding use and emerging trials of the ketogenic diet in various neurologic disorders excluding intractable epilepsy and provide explanations of the mechanisms in that usage.

• Journal of Korean Neurosurgical Society
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• v.62 no.3
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• pp.321-327
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• 2019

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 after surgery and the long-term seizure freedom remain relatively stable. Seizure outcome after surgery depends on a various factors such as pathologic etiologies, extent of lesion, and types of surgery. Therefore, seizure outcome after surgery for FCD should be analyzed carefully considering cohorts' characteristics. Studies of pediatric epilepsy surgery emphasize the early surgical intervention for a better cognition. Early surgical intervention and cessation of seizure activity are important for children with intractable epilepsy. However, there are limited data on the cognitive outcome after surgery in pediatric FCD, requiring further investigation. This paper reviews the seizure and cognitive outcomes of epilepsy surgery for FCD in children. Several prognostic factors influencing seizure outcome after surgery will be discussed in detail.

• Journal of Korean Neurosurgical Society
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• v.62 no.3
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• pp.344-352
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• 2019

Epilepsy is one of the major chronic neurological diseases affecting many patients. Resection surgery is the most effective therapy for medically intractable epilepsy, but it is not feasible in all patients. Vagus nerve stimulation (VNS) is an adjunctive neuromodulation therapy that was approved in 1997 for the alleviation of seizures; however, efforts to control epilepsy by stimulating the vagus nerve have been studied for over 100 years. Although its exact mechanism is still under investigation, VNS is thought to affect various brain areas. Hence, VNS has a wide indication for various intractable epileptic syndromes and epilepsy-related comorbidities. Moreover, recent studies have shown anti-inflammatory effects of VNS, and the indication is expanding beyond epilepsy to rheumatoid arthritis, chronic headaches, and depression. VNS yields a more than 50% reduction in seizures in approximately 60% of recipients, with an increase in reduction rates as the follow-up duration increases. The complication rate of VNS is 3-6%, and infection is the most important complication to consider. However, revision surgery was reported to be feasible and safe with appropriate measures. Recently, noninvasive VNS (nVNS) has been introduced, which can be performed transcutaneously without implantation surgery. Although more clinical trials are being conducted, nVNS can reduce the risk of infection and subsequent device failure. In conclusion, VNS has been demonstrated to be beneficial and effective in the treatment of epilepsy and various diseases, and more development is expected in the future.

• Clinical and Experimental Pediatrics
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• v.56 no.7
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• pp.275-281
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• 2013

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 in the case of adults, TLE in infants and young children often has etiologies other than mesial temporal sclerosis, such as tumors, cortical dysplasia, trauma, and vascular malformations. Differences in seizure semiology have also been reported. Motor manifestations are prominent in infants and young children, but they become less obvious with increasing age. Further, automatisms tend to become increasingly complex with age. However, in childhood and especially in adolescence, the clinical manifestations are similar to those of the adult population. Selective amygdalohippocampectomy can lead to excellent postoperative seizure outcome in adults, but favorable results have been seen in children as well. Anterior temporal lobectomy may prove to be a more successful surgery than amygdalohippocampectomy in children with intractable TLE. The presence of a focal brain lesion on magnetic resonance imaging is one of the most reliable independent predictors of a good postoperative seizure outcome. Seizure-free status is the most important predictor of improved psychosocial outcome with advanced quality of life and a lower proportion of disability among adults and children. Since the brain is more plastic during infancy and early childhood, recovery is promoted. In contrast, long epilepsy duration is an important risk factor for surgically refractory seizures. Therefore, patients with medically intractable TLE should undergo surgery as early as possible.

• Journal of Korean Neurosurgical Society
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• v.45 no.6
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• pp.329-335
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• 2009

Objective : Determining language lateralization is important for the presurgical evaluation of patients with medically intractable epilepsy. The Wada test has been the gold standard for lateralization of language dominance before epilepsy surgery. However, it is an invasive test with risk, and have some limitations. Methods : We compared the volumetric analysis with Wada test, and studied the clinical potential of volumetric analysis to assess language laterality in large surgical candidates with temporal lobe epilepsy (TLE). To examine the efficacy of volumetric analysis to determine language lateralization during presurgical evaluation, we compared the volumetric analysis of the bilateral planum temporale with the results of Wada test in 59 patients with chronic intractable TLE (rTLE, n=32; lTLE, n=27) who underwent epilepsy surgery. We measured the gray matter volumes of planum temporale (PT) of each patients using the VoxelPlus2 program (Mevisys, Daejeon, Korea). Results : Overall congruence of the volumetric analysis with the Wada test was 97.75% in rTLE patients and 81.5% in lTLE patients. There were more significant leftward asymmetry of the PT in rTLE patients than lTLE patients. In lTLE patients, relatively high proportion (37%) of the patients showed bilateral or right hemispheric language dominance. Conclusion : These results provide evidence that the volumetric analysis of the PT could be used as an alternatives in language lateralization. Also, the results of the Wada test suggested that there was considerable plasticity of language representation in the brains of patients with intractable TLE and it was associated with an earlier age of brain injury.

• Journal of Korean Neurosurgical Society
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• v.62 no.3
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• pp.272-287
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• 2019

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 pathway regulates fundamental biological processes associated with neural development and neurodegeneration. Intriguingly, genes that constitute the mTOR pathway have been found to be germline and somatic mutation from patients with various epileptic disorders. Hyperactivation of the mTOR pathway due to said mutations has garnered increasing attention as culprits of these conditions : somatic mutations, in particular, in epileptic foci have recently been identified as a major genetic cause of intractable focal epilepsy, such as focal cortical dysplasia. Meanwhile, epilepsy models with aberrant activation of the mTOR pathway have helped elucidate the role of the mTOR pathway in epileptogenesis, and evidence from epilepsy models of human mutations recapitulating the features of epileptic patients has indicated that mTOR inhibitors may be of use in treating epilepsy associated with mutations in mTOR pathway genes. Here, we review recent advances in the molecular and genetic understanding of mTOR signaling in epileptic disorders. In particular, we focus on the development of and limitations to therapies targeting the mTOR pathway to treat epileptic seizures. We also discuss future perspectives on mTOR inhibition therapies and special diagnostic methods for intractable epilepsies caused by brain somatic mutations.

• Korean Journal of Biological Psychiatry
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• v.5 no.2
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• pp.175-183
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• 1998

To consider current concepts of epilepsy further, the brief review begins with a discussion of what is epilepsy, discribes multifactorial nature of epileptic disorders, and ends with a presentation of current classifications. A combination of the standard antiepielptic drugs(AEDs) may be necessary to treat intractable seizures, but no studies have been done to indicate an optimal combination. The new AEDs provide alternative choices, but questions remain about the optimal timing and manner of administration. AEDs selection must individualized, no drug of choice can be named for all patients.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.204-207
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• 2005

Ketogenic diet is a high-fat, low-carbohydrate, low-protein diet used in the treatment of epilepsy since 1920's. Recently, it's use for intractable epilepsy in childhood has increased. Complications of ketogenic diet are known to include dehydration, vomiting, diarrhea, renal stones, metabolic derangement, hypercholesterolemia and refusal to eat. We experienced two cases of renal stones in children with intractable epilepsy during ketogenic diet.

• Child Health Nursing Research
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• v.10 no.2
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• pp.225-232
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• 2004

The purpose of this article is to describe the overview of current medical treatments of childhood epilepsy in Korea and to review several recent nursing researches related to quality of life problems, especially psychological functioning in children with epilepsy and the stress of the family. The prognosis of childhood epilepsy has been improved considerably and about 80％ of patients can now be expected to achieve complete seizure control by the antiepileptic drug treatment. Even for the intractable epilepsy, with the combination of ketogenic diet program and antiepileptic drug therapy or surgical treatment, the prognosis became very much better than before. The majority of research has reported that children with epilepsy were experiencing quality of life problems. They are at risk for impaired functioning, compared to either general population controls or to other chronic illness groups such as asthma and diabetes. The ultimate goal of providing care to children with epilepsy is to control seizures while facilitating an optimal quality of life for the child as well as the family. Recommendations are included for future research and intervention programs for children, parents and our society.

• Childhood Kidney Diseases
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• v.23 no.1
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• pp.48-52
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• 2019

The ketogenic diet (KD) has been used as an effective antiepileptic therapy for intractable childhood epilepsy. However, various adverse effects have been reported with use of the KD. We report a case of a child who developed acute tubular necrosis subsequent to therapy with KD. A 5-year-old girl had myoclonic epilepsy with developmental delay. She was under the treatment with antiepileptic drugs since the age of 3 months and on the KD during the past 18 months. Proteinuria persisted intermittently with the initiation of the KD and subsequently increased in the past 2 months. She was admitted with intermittent mild fever, vomiting, and lethargy for the past 3-4 weeks. At the time of admission, she presented with hypertriglyceridemia, heavy proteinuria, renal Fanconi syndrome, and acute kidney injury. Renal sonography showed a marked increase in the size and parenchymal echogenicity of both kidneys. A renal biopsy revealed acute tubular necrosis accompanied by early interstitial fibrosis. After the withdrawal of the KD and supportive therapy, without changing other anticonvulsants and their dosages, improvement of renal function was observed. Proteinuria had disappeared after 1 month and kidney size returned to normal after 8 months. It is hypothesized that the KD can induce and/or aggravate the renal tubulointerstitial injury in some patients who are under the treatment with anticonvulsants.