• Journal of Korean Neurosurgical Society
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• v.60 no.3
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• pp.294-300
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• 2017

Hypothalamic hamartoma (HH) is a benign indolent lesion despite the presentation of refractory epilepsy. Behavioral disturbances and endocrine problems are additional critical symptoms that arise along with HHs. Due to its nature of generating epileptiform discharge and spreading to cortical region, various management strategies have been proposed and combined. Surgical approaches with open craniotomy or endoscopy, stereotactic approaches with radiosurgery and gamma knife surgery or radiofrequency thermos-coagulation, and laser ablation have been introduced. Topographical dimension and the surgeon's preference are key factors for treatment modalities. Endoscopic disconnection has been one of the most favorable options performed in treating HHs. Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications.

• The Journal of the Korean life insurance medical association
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• v.16
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• pp.59-72
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• 1997

• Clinical and Experimental Pediatrics
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• v.63 no.8
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• pp.310-311
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• 2020

• Journal of Korean Neurosurgical Society
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• v.62 no.3
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• pp.263-264
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• 2019

• Clinical and Experimental Pediatrics
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• v.62 no.7
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• pp.259-260
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• 2019

• 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.

• Clinical and Experimental Pediatrics
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• v.58 no.9
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• pp.354-357
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• 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.571-578
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• 2010

Purpose : To evaluate the efficacy and safety of levetiracetam adjunctive therapy for reducing the rate of seizure frequency in children with intractable pediatric epilepsy. Methods : We reviewed the medical records of 86 patients with intractable pediatric epilepsy who visited our hospital between March 1989 and February 2009. Levetiracetam was included in the previous anticonvulsant regimen for at least 6 months and the reduction in the rate of seizure frequency was determined in follow-up examinations. We analyzed demographic data, seizure types, antiepileptic drug history, levetiracetam dose, adverse effects of levetiracetam therapy, treatment outcome, electroencephalogram findings, etc. Results : More than 50% reduction in the seizure frequency was observed in 62 of the 86 (72.1%) patients; 44 patients (51.1%) became seizure free, while the seizure frequency increased in 5.8% patients. The associations between seizure reduction rate and age, associated diseases, seizure types, and seizure frequency before treatment were not significant. However, the duration of disease, dose of levetiracetam, duration and frequency of anticonvulsant administration before levetiracetam therapy were significantly correlated. Electroencephalogram findings and the cause of epilepsy showed partial correlation. Forty (46%) patients showed adverse symptoms; the symptoms in the order of their frequency were somnolence, hyperactivity, irritability, aggressiveness, tiredness, etc. Conclusion : The findings of our study provide the evidence that levetiracetam adjunctive therapy is efficacious and well tolerated in various refractory childhood epilepsy cases.

• Clinical and Experimental Pediatrics
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• v.64 no.8
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• pp.384-392
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• 2021

Paroxysmal nonepileptic events (PNE) comprise of a variety of nonepileptic behaviors and are divided into various types. A more accurate diagnosis is possible by examining the video clip provided by the caregiver. In infants, physiologic PNE accounts for the majority of the PNE. It is important to exclude epilepsy, for which blood tests, electroencephalography, and imaging tests can facilitate differential diagnosis. Since most PNE have a benign progress, symptoms often improve with age and without special treatment. Therefore, it is important to reassure the caregivers after making an accurate diagnosis.

• Clinical and Experimental Pediatrics
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• v.53 no.8
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• pp.786-789
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• 2010

Induced pluripotent stem (iPS) cells are generated by epigenetic reprogramming of somatic cells through the exogenous expression of transcription factors. Recently, the generation of iPS cells from patients with a variety of genetic diseases was found to likely have a major impact on regenerative medicine, because these cells self-renew indefinitely in culture while retaining the capacity to differentiate into any cell type in the body, thereby enabling disease investigation and drug development. This review focuses on the current state of iPS cell technology and discusses the potential applications of these cells for disease modeling; drug discovery; and eventually, cell replacement therapy.