• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.458-463
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• 2009

Purpose : Schizencephaly is a uncommon congenital brain anomaly characterized by congenital clefts spanning the cerebral hemispheres from pial surface to lateral ventricles and lined by gray matter. In this study, we investigated the clinical manifestation and radiologic findings of pediatric schizencephaly. Methods : The data of 13 patients who were diagnosed with schizencephaly in Severance Childrens Hospital and Yongdong Severance Hospital from January 2005 to December 2007 were analyzed retrospectively. Results : The mean age at diagnosis was $9.08{\pm}2.67$ months old and ranged from 1 to 30 months. The ratio of male to female patients was 3.33:1. Five (38.5%) patients had bilateral clefts, while 8 (61.5%) had unilateral clefts. Five (38.5%) patients had closed lip clefts, and 4 (30.8%) had opened lip clefts. Four (30.8%) patients had multiple clefts. Associated anomalies showed in all cases. The clinical features consisted of mild unilateral weakness in 7 (53.8%) cases and a hemiparesis was present in 3 (23.1%) patients. A tetraparesis was in 3 (23.1%) patients. There was no difference in motor deficit between unilateral and bilateral clefts. Delayed development was observed in all cases. Epilepsy was present in 7 (53.8%) patients, 5 patients with unilateral clefts and 2 patients with bilateral clefts. Three (42.8%) patients showed intractable seizures. Conclusion : Schizencephaly showed variable clinical manifestations and radiologic findings in association with the types and locations of the clefts. It is necessary to diagnose schizencephaly early and to detect the development of epilepsy. Intensive and large studies of the correlation of clinical outcomes and radiologic findings should be continued for more effective treatment.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.62-66
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• 2008

Purpose : In addition to epileptic seizures (ES), a variety of physiologic, organic and psychogenic disorders can manifest as paroxysmal behavioral events. Paroxysmal nonepileptic events (PNEs) are quite encountered in infants, young children, and adolescents. In a substantial proportion of cases, a careful history and examination will elucidate their nature. However, in other cases, it is necessary to differentiate PNEs from ES by video-electroencephalographic (EEG) monitoring. We report our experiences with PNEs in a group of children and adolescents who underwent video-EEG monitoring. Methods : From September, 2004 to June, 2006, one hundred thirty patients were monitored in the Pediatric Epilepsy Monitoring Units of Korea University Guro and Ansan hospitals. Their hospital charts were reviewed and video records of these events were analyzed. We observed all patients after video-EEG monitoring for more than 3 months. Results : Typical spells occurred during monitoring in 33 patients, not associated with a seizure pattern on EEG recordings. Two patients were diagnosed as frontal lobe epilepsy on basis of typical semiology and clinical characteristics, so 31 patients were documented to have PNEs finally. The mean age of patients was $7.2{\pm}5.8\;years$. The male to female ratio was 15 (48.4%) to 16 (51.6%). Among 31 patients, fifteen patients had associated disorders such as epilepsy, developmental delay, cerebral palsy, gastric ulcer, attention deficit hyperactivity disorder or depressive disorder. Somatoform disorder and factitious disorder was frequently seen in children more than 5 years old (P<0.05). Psychogenic disorder was more frequent in female (n=6) than in male (n=2) but there was no statistical significance (P>0.05). Conclusion : Our study suggests that video-EEG monitoring is an important diagnostic tool in the evaluation of paroxysmal behavioral events. With correct diagnosis of the PNEs, several unnecessary treatment could be avoided.

• Applied Microscopy
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• v.30 no.4
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• pp.347-355
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• 2000

Zinc is one of the most abundant oligoelements in the living cell. It appears tightly bound to some metalloproteins and nucleic acids, loosely bound to some metallothioneins or even as free ion. Small amounts of zinc ions (in the nanomolar range) regulate a plentitude of enzymatic proteins, receptors and transcription factors, thus rolls need accurate homeostasis of zinc ions. Zinc is an essential catalytic or structural element of many proteins, and a signaling messenger that is released by neural activity at many central excitatory synapses. Growing evidences suggest that zinc may also be a key mediator and modulator of the neuronal death associated with transient global ischemia and sustained seizures, as well as perhaps other neurological disease stoles. Some neurons have developed mechanisms to accumulate zinc in specific membrane compartment ('vesicular zinc') which can be evidenced using histochemical techniques. Substances giving a bright colour or emitting fluorescence when in contact with divalent metal ions are currently used to detect them inside cells; their use leads to the so called 'direct' methods. The fixation and precipitation of metal ions as insoluble salt precipitates, their maintenance along the histological process and, finally, their demonstration after autometallographic development are essential steps for other methods, the so called 'indirect methods'. This study is a short report on the autometallograhical approaches for zinc detection in the central nervous system (CNS) by means of a modified selenium method.

• Korean Journal of Biological Psychiatry
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• v.6 no.1
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• pp.74-80
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• 1999

This study was performed to investigate the mechanism of the clozapine-induced seizures in partially restrained rats by concomitant treatment with drugs affecting monoaminergic systems. Partially restrained rats treated with acute single doses of 10mg/kg clozapine exhibited myoclonic jerks (MJs). Drugs affecting the monoaminergic systems, including 2mg/kg haloperidol, 5mg/kg propranolol, 2mg/kg ritanserin, 20mg/kg fluoxetine, and 20mg/kg imipramine, were concomitantly treated with clozapine to observe the effects of these drugs on the MJs. The drugs were given intraperitoneally either as acute single doses(haloperidol, propranolol, ritanserin, and fluoxetine) or as chronic doses for 21days(haloperidol, imipramine, ritanserin, and fluoxetine). The effects of the concomitant treatment of other drugs on the clozapine-induced MJs were evaluated by comparison of the total numbers of the MJs between the clozapine-treated and concomitantly treated groups. The results were as follows. 1) Concomitant treatment with acute single doses of haloperidol, propranolol, and fluoxetine reduced the total numbers of the clozapine-induced MJs, while concomitant treatment with ritanserin did not. 2) Concomitant treatment with chronic doses of imipramine and ritanserin increased the total numbers of the MJs, while concomitant treatment with fluoxetine reduced them. Concomitant chronic treatment with haloperidol did not affect the numbers of the MJs. These results suggest that dopamine and serotonin, not noradrenalin may be involved in the clozapine-induced MJs in partially restrained rats. Future research needs to study the function of each subtype of monoaminergic receptors on the mechanism of the clozapine-induced seizure.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.659-664
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• 2006

Purpose : To evaluate the morbidity and mortality of children with status epilepticus(SE) as an initial seizure and to compare these according to age groups. Methods : The 78 cases(38 cases <2 years and 38 cases ${\geq}2$ years) with SE as an initial seizure admitted to the Chonnam national university hospital from Jan. 2000 to Jan. 2004 were reviewed. Developmental profiles, laboratory findings, etiologies and seizure types of SE and outcomes were compared in between two age groups, under and over 2 years. Results : SE occurred predominantly in less than 5 years old. Febrile causes were the most common, which is significantly more in those under 2 years than over 2 years(P<0.05). whereas idiopathic and acute symptomatic causes were more common in those over 2 years(P<0.05). Generalized tonic-clonic seizures was the most common type. The mortality rate was 6.4 percent(5 cases : 1 case <2 years and 4 cases ${\geq}2$ years). The estimated occurrence of epilepsy after SE was 24.4 percent(19 cases : 8 cases <2 years and 11 cases ${\geq}2$ years). The neurologic sequelae after SE in cases that had developed normally before SE(62 cases : 32 cases <2 years and 30 cases ${\geq}2$ years) were observed in 20 cases(32.3 percent), and were more frequent over 2 years(21.9 percent vs. 43.3 percent, P<0.05). Conclusion : In this study death was less common and the neurologic sequelaes of SE as an initial seizure were less severe in children under 2 years of age. The reason seemed to be the difference in the etiology of SE with age.

• Neonatal Medicine
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• v.16 no.1
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• pp.55-63
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• 2009

Purpose: This study was conducted to investigate the incidence and clinical manifestations of rotaviral infections in the neonatal intensive care unit (NICU). We also investigated whether neonates referred from other hospitals cause outbreaks of rotaviral infections in the NICU. Methods: Neonates diagnosed wit rotaviral infections in the NICU at Korea University Ansan Hospital between January 2002 and December 2006 were evaluated retrospectively. Results: Of 1,501 neonates admitted to the NICU, the number of rotaviral infections was 94 (6.3%) and the prevalence was 4.9 cases per 1,000-hospital days. Of 223 neonates referred from other hospitals to our NICU, 24 (10.8%) were confirmed to have rotaviral infections at the time of referral. Common symptoms of rotaviral infections were jaundice diarrhea or loose stools, vomiting, fever, apnea, irritability, seizures, and moaning sounds. Necrotizing enterocolitis of stage II or more was diagnosed in 3 premature neonates, accounting for 3.2% of total infected neonates and 6.8% of premature infected patients. There were no statistically significant differences in the prevalence of symptoms between term and preterm neonates, except for jaundice that is more frequent in premature infants. In time series analysis, the admission of neonates who were born at other hospitals and diagnosed with rotaviral infections were associated 1 month later by increased cases of neonates with rotaviral infections who were born at our hospital, whereas new cases of rotaviral infections among inborn neonates were decreased 2 and 4 months later (P<0.05). Conclusion: Rotaviral infections in NICU were not rare and showed vague symptoms. Three cases (3.2%) were diagnosed with stage II or more necrotizing enterocolitis. The referred neonates with rotaviral infections may induce outbreaks in the NICU, therefore awareness and strict surveillance for rotaviral infections should be performed at the time of referral.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.286-292
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• 2008

Purpose : We performed EEG and PET on children with epilepsy concomitantly in order to evaluate the effects of epileptiform and non-epileptiform discharge of EEG on glucose metabolism. Methods : Seventy three children with epilepsy who had PET and EEG simultaneously were included in our study. The subjects were classified in two ways: (1) based on the frequency of epileptiform discharge and (2) the severity of non-epileptiform discharge. We evaluated the clinical aspects of their seizures, the severity of focal slow waves during the interictal period with the frequency of spikes or sharp waves in order to compare with the PET results. Results : The subjects were divided by the frequency of epileptiform discharge, with 13 in the no/rare group, 7 in the occasional group, and 53 children in the frequent group. The concordant rates with PET in each group were 0%, 42.9%, and 67.9%, respectively, showing high correlations with the frequency of epileptiform discharge (P<0.05, r=0.491). The subjects as divided by the severity of non-epileptiform discharge were 15 in the no group, 25 in the infrequent group, 17 in the intermediate group, and 16 in the continuous group. The concurrence rates with PET for each group were 13.3%, 52.0%, 64.7%, and 68.8%, respectively, also showing a high correlation with the severity of non-epileptiform discharge (P<0.05, r= 0.365). Conclusion : Epileptiform discharge and non-epileptiform discharge in EEG showed a certain association with hypometabolism in PET. We recommend EEG to reduce false lateralization and to localize lesions in cases of high frequency and severity.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.566-571
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• 2003

Purpose : Leukoencephalopathy(LE) is one of the most serious complications in children with hematologic malignancies during the course of treatment. Early recognition is important to reduce the impact and sequelae from LE. We therefore investigated the clinical features of LE following central nervous system(CNS) prophylaxis in children with hematologic malignancies and evaluated the significance of regular check-ups of brain MRI. Methods : We retrospectively reviewed children with hematologic malignancies who had CNS prophylaxis including intrathecal(IT) methotrexate(MTX) and/or cranial irradiation at the Department of Pediatrics, Kyungpook National University Hospital from Oct. 1995 to May 2002. Fifteen cases of acute leukemia and one case of lymphoma who experienced LE following CNS prophylaxis were included in the study. Clinical data were analyzed from the medical records and brain MRIs were reviewed by neuroradiologists. Results : The ages ranged from 1 to 13 years(median age=5.2 years), and the male to female ratio was 3 : 1. The time interval from the beginning of chemotherapy to the time of diagnosis of LE ranged from 2 to 17 months. They all had IT MTX two to 15 times and ten underwent cranial irradiation(1,800 rads). At the time of diagnosis, ten of them had neuropsychiatric symptoms including seizures, personality changes, headache, etc. After the change of treatment modality, four cases showed significant improvement on follow-up MRIs, six cases had no significant changes and two had worsening of LE. Four patients died of infection and bone marrow relapse. Conclusion : CNS prophylaxis with IT therapy and cranial irradiation may cause leukoencephalopathy during the course of treatment. As a result, regular brain MRI check-up is recommended for the early detection and reducing the incidence of LE, along with changes in the treatment modality.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.199-204
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• 2009

Purpose : Seizure associated with fever may indicate the presence of underlying inherited metabolic diseases. The present study was performed to investigate the presence of underlying metabolic diseases in patients with complex febrile seizures, using analyses of urine organic acids. Method : We retrospectively analyzed and compared the results of urine organic acid analysis with routine laboratory findings in 278 patients referred for complex febrile seizure. Results : Of 278 patients, 132 had no abnormal laboratory findings, and 146 patients had at least one of the following abnormal laboratory findings: acidosis (n=58), hyperammonemia (n=55), hypoglycemia (n=21), ketosis (n=12). Twenty-six (19.7 %) of the 132 patients with no abnormal findings and 104 (71.2%) of the 146 patients with statistically significant abnormalities showed abnormalities on the organic acid analysis (P<0.05). Mitochondrial respiratory chain disorders (n=23) were the most common diseases found in the normal routine laboratory group, followed by PDH deficiency (n=2) and ketolytic defect (n=1). In the abnormal routine laboratory group, mitochondrial respiratory chain disorder (n=29) was the most common disease, followed by ketolytic defects (n=27), PDH deficiency (n=9), glutaric aciduria type II (n=9), 3-methylglutaconic aciduria type III (n=6), biotinidase deficiency (n=5), propionic acidemia (n=4), methylmalonic acidemia (n=2), 3-hydroxyisobutyric aciduria (n=2), orotic aciduria (n=2), fatty acid oxidation disorders (n=2), 2-methylbranched chain acyl CoA dehydrogenase deficiency (n=2), 3-methylglutaconic aciduria type I (n=1), maple syrup urine disease (n=1), isovaleric acidemia (n=1), HMG-CoA lyase deficiency (n=1), L-2-hydroxyglutaric aciduria (n=1), and pyruvate carboxylase deficiency (n=1). Conclusion : These findings suggest that urine organic acid analysis should be performed in all patients with complex febrile seizure and other risk factors for early detection of inherited metabolic diseases.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1118-1123
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• 2003

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous purpura, abdominal pain, arthralgia and renal involvement. The clinical features of HSP have been reasonably well documented but there are still many gaps in our understanding of HSP. The aim of this study was to present the clinical features of 125 children with HSP and compare them with previous reports, placing particular emphasis on clinical information. Methods : We collected the clinical data of 125 patients with acute HSP who visited Chungbuk National University Hospital from March 1992 to April 2002. Data were expressed as the mean or $mean{\pm}SD$ and statistical analysis was performed using Chi-square approximation. P<0.05 was considered as significant. Results : The patient population consisted of 87 boys and 38 girls ranging in age from one to 14 years. HSP occurs throughout the year, but this study shows seasonal skewing, with most patients presenting from fall through spring and a paucity of cases in summer. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities. Purpuric lesions were also scattered on the arms and occaisionally on the face and ears, but the trunk was largely spared. A recurrence of purpura was defined as the reappearance of a rash or other symptoms following resolution of disease for at least two weeks. The mean number of recurrences was 0.51. Eighty eight patients(70.4%), 18 patients(14.4%) and 67 patients(53.6%) complained of abdomianl pain, gastrointestinal bleeding and arthralgia, respectively. Nephritis occurred in 48(38.4%) patients. Fifteen boys (17.2%) developed epididymitis. Neurologic features occurred in 13(10.4%) and two(15%) of these were seizures. Conclusion : HSP all showing purpura as defined is characterized by various clinical features, including abdominal pain, arthralgia, epididymitis and nephritis which could occur before the appearance of purpura. Therefore, we suggest that the possibility of HSP should be considered in children before invasive procedures, even if the above symptoms and signs present without purpura.