• Annals of Clinical Neurophysiology
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• v.13 no.1
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• pp.26-30
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• 2011

Although the pathophysiologic mechanism is unknown, there has been long-running debate on whether periodic discharges such as periodic lateralized epileptiform discharges (PLEDs) and generalized periodic epileptiform discharges are an ictal or interictal EEG pattern. The goal of this review is to give evidence that such periodic discharges on EEG are not ictal phenomenon and just represent underlying acute brain damage. This review includes coma with epileptiform EEG pattern and its prognostic and therapeutic implications. Based on previous reports, rather than taking the view PLEDs represent either an underlying ictal process or an electrographic correlate of neuronal injury, it would be more reasonable that PLEDs are considered as a dynamic pathophysiological state in which unstable neurobiological processes create an ictal-interictal continuum.

• Journal of Genetic Medicine
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• v.19 no.2
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• pp.100-104
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• 2022

The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multidrug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous diffuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12). This is a rare case report of CS with DEE-SWAS in a Korean patient.

• Journal of Korean Neurosurgical Society
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• v.65 no.3
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• pp.385-396
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• 2022

Abusive head trauma (AHT) in infants, especially acute subdural hematoma, has an extremely poor outcome. The most decisive and important finding is the appearance of a widespread low-density area on head computed tomography. This phenomenon was traditionally thought to be caused by cerebral ischemia. However, many other pathophysiological abnormalities have been found to be intricately involved. Recent studies have found that status epilepticus and hyperperfusion injures are the major causes. Another serious problem associated with AHT is cardiopulmonary arrest (CPA). Many infants are reported to visit to the hospital with CPA, and its pathophysiology has not been fully elucidated. This paper examines the background of these pathological conditions and associated factors and elucidate the pathophysiological mechanisms resulting in poor outcomes in AHT. In addition to the intensity of assault on the head, the peculiar pathophysiological characteristics in infants, as well as the social background specific to child abuse, are found to be associated with poor outcome.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1097-1103
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• 2007

Purpose : Acute symptomatic seizure is defined as a temporary seizure together with acute systemic, metabolic, or toxic insult in association with an acute central nervous system insult. And unprovoked seizure is defined as seizure without provocating factors. We studied the risk factors of unprovoked seizures after acute symptomatic seizure in children. Methods : We retrospectively reviewed the records of one hundred and ten children with acute symptomatic seizures who were admitted to the pediatric department of Chungbuk National University Hospital between January, 1998 and December, 2003. We analyzed overall risk factors of unprovoked seizures after acute symptomatic seizures involving etiology, incidence, type of seizure, duration and neuroimaging. Results : We analyzed records of 110 children with acute symptomatic seizures aged from 1 month to 17 years. 24 children had unprovoked seizures (21.8%) after acute symptomatic seizures. Causes in order of frequency were encephalopathy, central nervous system infection, brain tumor, cerebrovascular disease. The risk of unprovoked seizure was significantly greater for those with status epilepticus (68.4%) than without status epilepticus, with partial seizure (64.7%) than generalized seizure. And the risk of unprovoked seizure was strongly associated with abnormal finding of electroencephalogram (79.1%) and neuroimaging (41.6%). Conclusion : In conclusion, the leading cause of subsequent unprovoked seizure in children with acute symptomatic seizure was encephalopathy and age specific incidence was high in the group aged 24-72 months. The risk for subsequent unprovoked seizure was greater for those with partial seizure, status epilepticus, abnormal finding of neuroimaging and electroencephalography.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.233-239
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• 2018

Purpose: Viral gastroenteritis is a common disease in infants and children. Seizures can be associated with viral gastroenteritis as benign convulsions with mild gastroenteritis (CwG). After the development of the rotavirus vaccination, norovirus has become inreasingly significant in children. We retrospectively analyzed the clinical features in a pediatric population presenting with seizures and confirmed enteral viral infections, especially norovirus infections. Methods: We retrospectively reviewed the medical records of pediatric patients aged <15 years admitted due to convulsions and gastroenteritis to the Department of Pediatrics of Myongji Hospital between July 2014 and June 2016. Results: A total of 46 patients (24 male and 22 female) were included. Norovirus was detected in 21 (45.7%) patients, adenovirus in three (6.5%), rotavirus in two (4.3%), astrovirus in one (2.2%), and none of agents were detected in 19 (41.3%) patients. Patients in the norovirus gastroenteritis (NGE) group had a higher incidence of diarrhea than that among in the non-norovirus gastroenteritis (NNGE) group (61.9% vs 28.0%; P<0.05). Twelve patients experienced status epilepticus, including five (23.8%) in the NGE group and seven (33.0%) in the NNGE group (P=0.837). Seizures were effectively terminated by intravenous benzodiazepines in 8 (66.7%) of 12 patients. Additional long-acting antiepileptic drugs such as fosphenytoin or levetiracetam were required in three (25%) of 12 patients. Conclusion: Patients with CwG with and without noroviurs infection did not differ in terms of clinical features. However, status epilepticus was not uncommon among patients with CwG by definition.

• BMB Reports
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• v.48 no.6
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• pp.348-353
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• 2015

In the present study, we investigated the characteristics of drug efflux transporter expressions following status epilepticus (SE). In the hippocampus and piriform cortex (PC), vasogenic edema peaked 3-4 days after SE. The expression of breast cancer resistance protein (BCRP), multidrug resistance protein-4 (MRP4), and p-glycoprotein (p-GP) were decreased 4 days after SE when vasogenic edema was peaked, but subsequently increased 4 weeks after SE. Multidrug resistance protein-1 (MRP1) expression gradually decreased in endothelial cells until 4 weeks after SE. These findings indicate that SE-induced vasogenic edema formation transiently reduced drug efflux pump expressions in endothelial cells. Subsequently, during recovery of vasogenic edema drug efflux pump expressions were differentially upregulated in astrocytes, neuropils, and endothelial cells. Therefore, we suggest that vasogenic edema formation may be a risk factor in pharmacoresistent epilepsy. [BMB Reports 2015; 48(6): 348-353]

• Clinical and Experimental Pediatrics
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• v.60 no.9
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• pp.302-306
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• 2017

Purpose: This study aimed to investigate whether serum neuron-specific enolase (NSE) was expressed in acute encephalitis syndrome (AES) that causes neuronal damage in children. Methods: This prospective observational study was conducted in the pediatric neurology ward of Soetomo Hospital. Cases of AES with ages ranging from 1 month to 12 years were included. Cases that were categorized as simple and complex febrile seizures constituted the non-AES group. Blood was collected for the measurement of NSE within 24 hours of hemodynamic stabilization. The median NSE values of both groups were compared by using the Mann-Whitney U test. All statistical analyses were performed with SPSS version 12 for Windows. Results: In the study period, 30 patients were enrolled. Glasgow Coma Scale mostly decreased in the AES group by about 40% in the level ${\leq}8$. All patients in the AES group suffered from status epilepticus and 46.67% of them had body temperature >$40^{\circ}C$. Most of the cases in the AES group had longer duration of stay in the hospital. The median serum NSE level in the AES group was 157.86 ng/mL, and this value was significantly higher than that of the non-AES group (10.96 ng/mL; P<0.05). Conclusion: AES cases showed higher levels of serum NSE. These results indicate that serum NSE is a good indicator of neuronal brain injury.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.913-917
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• 2003

Cardiac rhabdomyomas are relatively uncommon and associated with tuberous sclerosis in 40-50% cases. We report a 10-month-old infant with tuberous sclerosis who presented with ventricular arrythmias and status epilepticus. There were hypopigmented macules on the body, periventricular calcifications, renal cyst and cardiac rabdomyomas just below the aortic valve. The patient required resection of left ventricular subaortic masses due to sustained arrythmia in spite of intravenous amiodarone therapy. The pathologic examination confirmed the diagnosis of rhabdomyoma. The patient had no more arrythmia during the 14 month follow up period. Although cardiac rhabdomyomas may spontaneously regress, surgery is often necessary and frequently resolves the underlying arrythmia.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.4 no.1
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• pp.46-53
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• 2004

3-Methylcrotonyl-CoA carboxylase (MCC) is a biotin-dependent enzyme involved in leucine metabolism. We describe a patient with MCC deficiency who manifested with Reye syndrome-like illness with status epilepticus, metabolic acidosis, hypoglycemia, hyperammonemia, elevated liver enzymes and neurologic impairments after the viral gastroenteritis and then, has suffered from Lennox-Gastaut syndrome. Urinary organic acid analysis revealed increased excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. This patient was managed with leucine restriction diet and supplementation of biotin and carnitine but was not so effective. He has suffered from neurologic sequelae such as Lennox-Gastaut syndrome, motor and cognitive impairement.

• Korean Journal of Bronchoesophagology
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• v.8 no.1
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• pp.61-65
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• 2002

Background : Acquired tracheoesophageal fistula(TEF) results mostly from Prolonged tracheal intubation and insertion of nasogastric tube. Although the incidence has decreased since the usage of low pressure, high volume cuff of endotracheal tube, it is seldom cured spontaneously and needs surgical treatment. Material and Methods : We have retrospectively reviewed five cases of TEF who underwent surgical treatment for cure from March, 1990 to September, 2001 and analyzed the cause, treatment, postoperative complications and prognostic factors. Results : Majority were men(80% : 4 of 5 patients) and the mean age was 29.4 years old(range, 11-58). The most predominant etiology was prolonged intubation or tracheostomy(80% : 4 of 5 patients) and 3 of 5 patients were treated by tracheal resection and end to end anastomosis with primary closure of esophagus. Postoperative complications occurred in 4 patients the most common complications were wound infection(4 cases) and esophageal leakage(2 cases). Extubation was done on postoperative day 11.5(range, 1-33) days, and factors causing delayed extubation were status esophagus. epilepticus, edema, and tracheal stenosis. Conclusion : Spontaneous closure of TEF is seldom possible and the surgical treatment of choice is tracheal resection and end to end anastomosis with primary repair of the esophagus. preoperative pulmonary rehabilitation and early extubation postoperatively are important factors for success.