• Clinical and Experimental Pediatrics
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• v.57 no.1
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• pp.1-18
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• 2014

Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.

• The Journal of Churna Manual Medicine for Spine and Nerves
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• v.1 no.1
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• pp.27-33
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• 2006

Objectives : This report is about a case of a patient who improvement in general condition, Infection and dysphagia after poststroke seizure. Methods : We observed a patiet who received treatment in hospital because of stroke sequelae. Her symptoms were left hemiplegia, right hemiparesis, dysphagia, dyspnea, sputum and general weakness, and acupunture and herb medicine were applied. Results : Stroke is the most common cause of seizures in the elderly. The impact of late onset GTC(generalized tonic-clonic) seizures is associated with worse outcomes. Conclusion : The patient of this case was attacked by intracerebal hemorrhage, cerebaral infarction and GTC seizures. Since then her neurologic disorder was progressed. As the treatments of the patient, herb medication and acupuncture therapy were applied and she got a wide improvement of dysphagia, dyspnea, URI sign and general condition.

• Annals of Clinical Neurophysiology
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• v.20 no.1
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• pp.36-40
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• 2018

Lennox-Gastaut syndrome (LGS) is an electro-clinical syndrome composed of the triad of mental retardation, multiple seizure types, and the characteristic generalized slow spike-wave complexes in the electroencephalogram. In this article, we report on two patients with LGS whose brain magnetic resonance imaging showed dysgenesis of corpus callosum (CC). We discuss the role of CC in the genesis of secondary bilateral synchrony.

• Journal of The Korean Society of Clinical Toxicology
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• v.6 no.2
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• pp.142-145
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• 2008

Lamotrigine is a newer anti-epileptic drug for adjunctive treatment of refractory epilepsy, partial seizures, generalized tonic-clonic seizures, and bipolar disorder. Lamotrigine overdose causes serious central nervous and cardiovascular problems, but reports are uncommon. Few lamotrigine overdoses have been described because anti-epileptic drug use is limited and usually used with combination of other anti-epileptic drugs. In addition, most patients visit emergency departments with multi-drug overdoses, so few cases of lamotrigine poisoning alone exist. We had a female patient visit our emergency department a couple of hours after a lamotrigine overdose treated with intravenous hydration and urine alkalization by NaHCO3. She recovered successfully without any evidence of renal injury. However, she developed profound rhabdomyolysis, a previously unreported complication of this medication. We suggest that serial creatine kinase levels should be measured after lamotrigine poisoning.

• Journal of The Korean Society of Clinical Toxicology
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• v.6 no.2
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• pp.123-129
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• 2008

A 36-year-old female was transferred to our emergency medical center with decreased mental status after a 6.0 g propafenone overdose because of domestic disturbance. She had no previous history of epilepsy, diabetes mellitus, hypertension or psychiatric illness. Before presenting to our center, gastrointestinal decontamination, charcoal administration, and endotracheal intubation due to bradycardia and generalized seizure had been performed. Soon after hospital arrival, at 5 h after ingestion, she collapsed into shock and fatal arrhythmia. We successfully resuscitated the patient with amiodarone, sodium bicarbonate, a large volume of normal saline, calcium, and ventilator care. At 23 h after ingestion, she was fully recovered and had no subjective signs or symptoms. To our knowledge, this is the first case report of intentional propafenone overdose in Korea, which we report with reviews of the previous literature.

• Korean Journal of Psychosomatic Medicine
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• v.5 no.2
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• pp.205-213
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• 1997

The aim of this study is to find the relationship between interictal psychiatric symptoms and seizure-related variables such as structural changes, regional interictal perfusion changes, the number of interictal epileptic discharges and the presence of accompanying generalized tonic clonic seizure(GTC). The subjects were 75 patients (47 males : mean age $28.3{\pm}7.7$) with intractable epilepsy, Interictal psychiatric symptoms were rated by Symptom Checklist-90-R(SCL-90-R). Each of 4 seizure variables was measured by MRI, $^{99m}TC-HMPAO$ SPECT and the prolonged EEG monitoring. The mean SCL-90-R subscale T-scores and the ratio of the patients with definite psychiatric symptoms(T-score > 65 at any one subscale of SCL-90-R) were compared among the groups with different seizure variables. Demographic variables and clinical variables were not statistically different among the groups with different seizure variables. The patients with right mesial temporal sclerosis had higher mean SCL-90-R scores for obsessive compulsive symptom, interpersonal sensitivity, depression, hostility and psychotic symptoms than the patients with left mesial temporal sclerosis, and they also showed higher ratio of definite psychiatric symptoms. The presence of interictal epileptic discharges was related with higher T-scores of paranoid subscale. The areas of hypoperfusion, the presence of the GTC, and other clinical seizure variables showed no significant influences on the mean SCL-90-R subscale T-scores. from these results, the authors speculated that interictal subictal epileptic dischrages may be related with psychiatric symptoms of the intractable epileptic patients, especially with right mesial temporal sclerosis.

• Proceedings of the Plant Resources Society of Korea Conference
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• 2000.10a
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• pp.116-122
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• 2000

The molecular modification of antiepileptic drugs and direct synthesis of new drugs with the predetermined antiepileptic properties are perspective. New neurochemical attacking to solve the problem including prevention and inhibition of seizures seems to be related to ginsenosides and ginseng polypeptides. The main study based on the severity of febrile convulsions of rat pups has been done from the earlier investigations of antiepileptical action of ginsenosides between KGTRI and MSU (Chepurnov, Park et al., 1995) with different kinds of experimental models of epilepsy. From the cultured cell line DAN25 of ginseng root, the extracts of ginsenosides made in "BIOKHIMMASH" were studied by the project of preclinical anticonvulsant screening (Stables, Kupferberg, 1997). The inhibition of severity of convulsions, decrease of seizures threshold, decrease of audiogenic seizures in rats of different strains and normalization of cerebral blood flow (measured by hydrogen test) were demonstrated in rats after i.c.v., intraperitoneally and orally administration, respectively. The antiepileptical effects by the combination of compounds from ginseng; were compared with the iuluence of Rg1, Rb1, Rc and with the well known antiepileptical drugs such as carbamazepine, valproic acid. The base for the research is obtained by using the WAG/Rij strain (Luijtelaar, Coenen, Kuznetcova), an excellent genetic model for human generalized absence epilepsy. The improving action of gensinosides was effectively demonstrated on the model of electrical kindling of amygdala of WAG/Rij rats with genetically determined absences, and the influences of ginsenosides on the slow wave discharges have also been being investigated. The different characteristics of a kindling process exerted in the sex-different region of the amygdala and demonstrated that the level of sex steroids and content of neurosteroids in amygdaloid tissue can modify the development of seizures. The chemical structures of ginsenosides not only have some principal differences from well-known antiepileptical drugs but the Plant Pharmacology gives us unique possibility to develop new class of antiepileptic drugs and to improve its biological activity.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.22-25
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• 2018

Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies of childhood onset. It is characterized by multiple types of generalized seizures, slowness of intellectual growth, and specific electroencephalogram pattern. It is one of the most difficult epilepsy syndromes to treat and is frequently resistant to treatment. In this case report, we report a case of dental treatment under general anesthesia in a child with LGS. A 4-year-6-month old boy with LGS visited Seoul National University Dental Hospital for dental treatment. Dental treatment was planned under general anesthesia because of his medical condition. Treatment was successfully done without any postoperative complications. If a LGS patient requires dental treatment, a treatment plan should be established to take into account the degree of seizure, the medication being used, and the cooperation of the patient. If the degree of seizure is severe and the patient's cooperation is poor, dental treatment under general anesthesia would be advantageous.

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.484-488
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• 2007

Purpose : The purpose of this study was to determine the incidence and potential predictors of weight gain in older children and teens treated with valproate (VPA) for epilepsy. Methods : Sixty-five subjects aged 8 to 17 years of age, who began VPA treatment between January 1, 2001, and December 31, 2004, and who had documented weight and height measurements at medication initiation and at least one follow-up visit were retrospectively identified. Exclusion criteria were follow-up <6 months, discontinuation of VPA within 6 months, and concurrent therapy with medication known to affect weight (such as topiramate, carbamazepin). Body mass index (BMI) was calculated at initiation and either discontinuation of VPA or last follow-up and stratified into four categories: group 1, underweight <5%; group 2, appropriate 5-85%; group 3, potentially overweight 85-95%; group 4, overweight >95%. Results : Twenty-eight subjects (77.8%) remained within their same category and eight (22.2%) moved up at least one category. Weight gain (increase in BMI difference) was observed in 72.2% of the 36 subjects treated with VPA. Three factors, neurocognitive status (P=0.017), seizure type (P=0.001) and duration of VPA treatment (P=0.035) were identified to be significant predictors of BMI difference. Conclusion : VPA induces weight gain in children and teens with epilepsy. These factors which are normal neurocognitive status, primary generalized type and duration of VPA treatment over the 12 months were predictors for an increase of weight gain. Therefore potential weight gain should be discussed with patients before the initiation of therapy and BMI should be monitored closely.

• Journal of Korean Neurosurgical Society
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• v.46 no.3
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• pp.252-256
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• 2009

Meningioangiomatosis (MA) is a rare congenital tumor that occurs mostly in 5-15 year old children. There have been only 5 cases previously reported that described the cystic nature within these tumors. We present a case of a MA accompanied by a separate macrocyst. A normally developed 2 year-old female patient presented with partial and generalized seizures. The brain computerized tomogram and magnetic resonance imaging revealed the presence of a calcified mass accompanied by a cyst in the right parietal area, surrounded by low density and high attenuation edema and hemorrhage. Upon right parietal craniotomy, a $1.6cm{\times}1.2cm{\times}0.5cm$ sized plate-like, gray-white, slightly hard mass was seen and it was completely excised. Approximately 1 cm from the mass in the anterior lateral direction, a cyst was found and subsequent biopsy of the cyst wall revealed no tumor tissue, and therefore the cyst was not removed. Pathologic report demonstrated the meningioangiomatosis. Follow up examination 2 years later showed no recurrence of the tumor, and there was no evidence of neurological deficits. Authors suggest that cysts that arise in the surrounding tissues of tumors may not be tumor cysts, and do not require surgical removal.