• Pediatric Infection and Vaccine
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• v.31 no.1
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• pp.147-152
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• 2024

There have been several case reports of neurological manifestations in pediatrics as severe acute respiratory syndrome coronavirus-2 infection in children is being increased. We report a case of a 4-year-old boy who presented febrile seizure during follow-up in a negative pressure isolation room after confirmed coronavirus disease 2019, which has not yet been reported in Korea. He has no symptoms other than fever. The seizure was controlled after one dose of intravenous lorazepam, and there was no respiratory support during the hospitalization. He was discharged 12 days later without neurological sequelae.

• Clinical Nutrition Research
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• v.12 no.3
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• pp.169-176
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• 2023

Glucose transporter type 1 (GLUT1) deficiency syndrome (DS) is a metabolic brain disorder caused by a deficiency resulting from SLC2A1 gene mutation and is characterized by abnormal brain metabolism and associated metabolic encephalopathy. Reduced glucose supply to the brain leads to brain damage, resulting in delayed neurodevelopment in infancy and symptoms such as eye abnormalities, microcephaly, ataxia, and rigidity. Treatment options for GLUT1 DS include ketogenic diet (KD), pharmacotherapy, and rehabilitation therapy. Of these, KD is an essential and the most important treatment method as it promotes brain neurodevelopment by generating ketone bodies to produce energy. This case is a focused study on intensive KD nutritional intervention for an infant diagnosed with GLUT1 DS at Gangnam Severance Hospital from May 2022 to January 2023. During the initial hospitalization, nutritional intervention was performed to address poor intake via the use of concentrated formula and an attempt was made to introduce complementary feeding. After the second hospitalization and diagnosis of GLUT1 DS, positive effects on the infant's growth and development, nutritional status, and seizure control were achieved with minimal side effects by implementing KD nutritional intervention and adjusting the type and dosage of anticonvulsant medications. In conclusion, for patients with GLUT1 DS, it is important to implement a KD with an appropriate ratio of ketogenic to nonketogenic components to supply adequate energy. Furthermore, individualized and intensive nutritional management is necessary to improve growth, development, and nutritional status.

• Clinical and Experimental Pediatrics
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• v.51 no.2
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• pp.162-169
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• 2008

Purpose : Oxcabarzepine (OXC), newly recommended antiepileptic drug, has been prescribed for patients with partial seizures and generalized tonic clonic seizures in Korea from 1999. There are limited reports about an efficacy of OXC therapy in epileptic children in Korea. This study evaluated the efficacy and safety of OXC in the light of our experience. Methods : The patients, who had visited the pediatric neurology clinic of Korea University Guro Hospital from January 2001 to December 2006, were included. The data of 144 patients who were administrated OXC as monotherapy or polytherapy, was summarized retrospectively and we evaluated the efficacy and safety of OXC. Results : After 6 months of OXC therapy, 77 patients (53.5%, n=144) achieved seizure freedom, 48 patients (33.3%) experienced >50% improvement. After 12 months of OXC therapy, cessation of seizure was observed in 88 patients (61.1%, n=133), and 27 patients (18.8%) manifested an improvement. Monotherapy group showed superior efficacy to polytherapy one. The frequent side effects of OXC were drowsiness (20.1%), headache (12.5%), dizziness (9.7%) and rash (8.3%). They did not related to patient's age or sex, and dosage of OXC. Twenty four patients (16.7%) experienced hyponatremia, but which were neither symptomatic nor significant one. Conclusion : The efficacy and safety of OXC in our patients were excellent and had less significant side effects than established international one. We expect this report contributes toward OXC therapy in epileptic children.

• The Korean Journal of Nuclear Medicine
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• v.35 no.1
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• pp.12-22
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• 2001

Purpose: The ictal perfusion patterns of cerebellum and basal ganglia have not been systematically investigated in patients with temporal lobe epilepsy (TLE). Their ictal perfusion patterns were analyzed in relation with temporal lobe and frontal lobe hyperperfusion during TLE seizures using SPECT subtraction. Materials and Methods: Thirty-three TLE patients had interictal and ictal SPECT, video-EEG monitoring, SPGR MRI, and SPECT subtraction with MRI co-registration. Results: The vermian cerebellar hyperperfusion (CH) was observed in 26 patients (78.8%) and hemispheric CH in 25 (75.8%). Compared to the side of epileptogenic temporal lobe, there were seven ipsilateral hemispheric CH (28.0%), fifteen contralateral hemispheric CH (60.0%) and three bilateral hemispheric CH (12.0%). CH was more frequently observed in patients with additional frontal hyperperfusion (14/15, 93.3%) than in patients without frontal hyperperfusion (11/18, 61.1%). The basal ganglia hyperperfusion (BGH) was seen in 11 of the 15 patients with frontotemporal hyperperfusion (73.3%) and 11 of the 18 with temporal hyperperfusion only (61.1%). In 17 patients with unilateral BGH, contralateral CH to the BGH was observed in 14 (82.5%) and ipsilateral CH to BGH in 2 (11.8%) and bilateral CH in 1 (5.9%). Conclusion: The cerebellar hyperperfusion and basal ganglia hyperperfusion during seizures of TLE can be contralateral, ipsilateral or bilateral to the seizure focus. The presence of additional frontal or basal ganglia hyperperfusion was more frequently associated with contralateral hemispheric CH to their sides. However, temporal lobe hyperperfusion appears to be related with both ipsilateral and contralateral hemispheric CH.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.891-895
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• 2007

Purpose : Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. Methods : The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital. We grouped the patients according to the MRI findings as follows. Group I (14 cases): Multi- or unifocal lesions only in the cerebral white matter. Group II (7 cases): lesions in the gray matter with or without white matter involvement. Results : 1. Preceding events were as follows: no defined prodrome (38.1%), upper respiratory tract infection (28.6%), nonspecific febrile illness (19.0%), gastointestinal disturbance and vaccination. 2. Presenting symptoms were as follows: seizures (76.2%), headache/vomiting (47.6%), altered consciousness (38.1%), hemiparesis, cerebellar ataxia, visual disturbance and facial nerve palsy. 3. Laboratory findings were as follows: CSF pleocytosis (76.2%), leucocytosis (38.1%) and elevated CSF protein (28.6%). 4. Fifteen patients were recovered completely without neurological sequelae. Three patients in group I and 1 patient in group II had intractable seizures. Two patients in group I and 2 patients in group II had motor disturbance. Conclusion : There were no statistically significant differences in preceding events, presenting symptoms, and neurological outcomes according to the distribution of the lesions on brain MRI. However, the ADEM have quite diverse clinical manifestations and neuroimage findings. MRI plays an important role in making diagnosis of the patients who are suspected of ADEM.

• Journal of agricultural medicine and community health
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• v.4 no.1
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• pp.41-61
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• 1979

Two interview surveys (1976 for 800 patients, 1978 for 200 patients) and an inventory survey through medical records(1978) for epileptic patients who have registered with the Korean Epilepsy Association (Rose Club) since 1971 were carried out by trained health workers in advance of survey. The data obtained from the analysis showed as follows: 1) 35.2% of patients were born in Seoul and 70. 6% of patients born elsewhere have lived in Seoul. 2) 50-60% of patients were 15-30 years cid. 3) 33.4%, 24,6 and 24.6 of all pupils and students went to elementary, junior and senior high schools respectively. 4) 21.2% of all pupils and students had dropped out of school and 51.4% of them were away from school because of epilepsy. 5) 3.1% of all patients had no job at all and students comprised 20.9% of patients followed by clerical work, commercial business and farming with about 6% in each group.6) Reasons given for unemployment such as dismissal (4.3%), quit (27.7%), hesitation to employ (42.5%)and discontinuance of job (25.5%) were basically due to epilepsy. 7) About half(46.2%) of all patients have become Christian since the Rose Club was a voluntary agency which has been sponsored by Christians. 8) 82. 6% of patients were diagnosed as having grand mal as the most. 9) 29.4% of patients explained aura with psychomotor disturbances and 13.8% with sensory disorders. 10) 46.3% of patients were attacked with seizures when they were tired and others(11.6% and 4.9%) after excessive eating and hunger. 11) Patients suffered more seizures in spring and summer rather than in autumn and winter and most patients had attacks 1-5 times a month. 12) For etiologic reasons of epilepsy, 35.5% of patients considered it was caused by psychological stress and 11.5% by trauma. Only 1.1% of patients considered it as having hereditary components. 13) 51% of patients were slow in caring for their own illnesses. They started to reat epilepsy after spending 5 years of time from the initial seizure. Only 5.4% of patients had received the modern anti-epileptic therapy right after the nitial seizure. 14) 62.1% of patients had no therapy or irregular or incomplete treatment before registration at the Rose Club Clinic. 15) Before registration at the Rose Club, 42.4% of patients received medical care. On the other hand, 25.6% went to herb doctors and 12.5% used to go to the drugstore in order to get anti-epileptic drugs. 16) 41. 6% of patients who took anti-epileptic drugs had more or less side-effects. Indigestion was the most common. 17) For continuation of treatment, 30.3% have received treatment for more than 5 years and the evident showed that epilepsy took a longer time to be cured. 18) Regarding the medical care received 44.2% of patients were very satisfied with effective care and 26.5% felt as good. 19) For attitudes toward epilepsy. 27.0% of patients and 68.2% of patients family were pessimistic. 20) 65.9% of patients had optimistic attitudes toward effectiveness of medical care of epilepsy. 21) 64.8% of wives and husbands had better understanding and cooperative for their spouses who had epilepsy. 22) 33.3% of patients were under-treated at the place of work. 23) 70.2% of patients wished to marry when they reach childbearing age and 63% wished to have children. Through the above results it is recommended for nation-wide epilepsy control that the sound and correct health education not only from health aspect but also from welfare aspect should be planned and implemented as soon as possible.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.2
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• pp.183-193
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• 2009

Purpose: Rotaviruses, noroviruses, astroviruses, and enteric adenoviruses cause acute gastroenteritis (AGE) in children. Some children with AGE have afebrile convulsions associated with viral gastroenteritis. The purpose of this study was to detect and genotype viruses from children with AGE or benign infantile seizures associated with mild gastroenteritis (BIS-MG). Methods: Between August 2004 and June 2005, 311 children with AGE were included. Four viral agents, including rotavirus, norovirus, astrovirus, and adenovirus, were analyzed from stool specimens of each patient using the latex agglutination method, enzyme immunoassay, and reverse transcriptase polymerase chain reaction. Genotyping of each virus was performed in 217 of the 311 children. Results: Among 217 children (male, 121; female, 96; mean age, 20.6${\pm}$15.4 months), rotavirus was detected in 109 (50.2%), norovirus in 28 (12.9%), adenovirus in 13 (6.0%), and astrovirus in 2 children (0.9%). Genotyping of rotavirus revealed positive results in 97 children; P[8]G3 in 36, P[4]G2 in 21, P[6]G4 in 10, P[4]G4 in 9, P[8]G9 in 6, P[8]G1 in 6, P[4]G3 in 4, P[4]G9 in 3, and P[6]G2 in 2. Genotyping of norovirus showed GII-4 in 27 of 28 children and GII-6 in 1 child. Sixteen children were diagnosed with BIS-MG. Rotavirus was detected in 13 of 16 children with BIS-MG, and norovirus in 2 children. Genotyping of rotavirus detected in children with BIS-MG revealed P[8]G3 in 6 children, P[4]G2 in 2 children, and P[4]G9 in 1 child. Conclusion: Analysis of viruses from stool specimens indicates that both rotavirus and norovirus are the main viruses related to BIS-MG in children.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.23-34
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• 2012

Purpose: Acute symptomatic seizures are caused by structural changes, inflammation or metabolic changes of brain, such as tumor, stroke, meningitis, encephalitis and metabolic disorders. Inherited metabolic disorders that can cause seizures are organic acidopathies, lysosomal storage disorders, peroxisomal disorders and mineral disorders. We have done this study to find out the importance of organic acidopathies causing seizure disorders in Korean childhood and adolescent patients. Method: Retrograde analysis for 1,306 patients with seizure disorders whose clinical informations are available and have done urine organic acid analysis for 5 years period, between Jan. 1st 2007 to Dec. 31th 2011. Statistical analysis was done with Student's t test using SPSS. Result: Out of 1,306 patients, 665 patients (51%) showed abnormalities on urine organic acid analysis. The most frequent disease was mitochondrial respiratory chain disorders (394, 30.1%), followed by mandelic aciduria (127, 9.7%), ketolytic defects (81, 6.2%), 3-hydroxyisobutyric aciduria (19, 1.4%), glutaric aciduria type II (10, 0.8%), ethylmalonic aciduria (4), propionic aciduria (4), methylmalonic aciduria (3), glutaric aciduria type I (3), pyruvate dehydrogenase deficiency (3), pyruvate carboxylase deficiency (3), isovaleric aciduria (2), HMG-CoA lyase deficiency (2), 3-methylcrotonylglycinuria (2), fatty acid oxidation disorders (2), fumaric aciduria (1), citrullinemia (1), CPS deficiency (1), MCAD deficiency (1). Conclusion: On neonatal period, mandelic aciduria due to infection was found relatively frequently. Mitochondrial disorders are most frequent etiologic disease on all age group, followed by ketolytic defects and various organic acidopathies. The number and diversities of organic acidopathies emphasize meticulous evaluation of basic routine laboratory examinations and organic acid analysis with initial sample on every seizure patient.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.205-209
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• 2018

Purpose: Several studies have shown that magnesium plays an important role in modulating N-methyl-D-aspartate (NMDA)-related seizures by blocking NMDA ion channel receptors. Clinicians usually measure total serum magnesium levels instead of biologically active ionized magnesium levels. We compared the serum ionized magnesium ($iMg^{2+}$) level between epileptic children with and without a history of fever-triggered seizure (FTS). Methods: All epileptic children who visited the outpatient clinic or pediatric emergency department at Korea University Guro Hospital between January 2015 and July 2017 were included. Only epileptic children aged 1-8 years who were newly diagnosed within 2 years were included. Results: There were 12 children with FTS and 16 without FTS. Median serum $iMg^{2+}$ level was 0.93 (0.85-1.14, quartile) mEq/L. Serum $iMg^{2+}$ level was significantly lower in epileptic children with FTS (0.86 mEq/L) compared to those without FTS (1.10 mEq/L) (P=0.005). No difference was noted in clinical variables between the two groups. Lower serum $iMg^{2+}$ level significantly increased the risk of having FTS in epileptic children based on multivariable logistic regression analysis (odds ratio [OR]=0.028). Conclusion: Serum $iMg^{2+}$ level was significantly lower in epileptic children with FTS than in those without FTS. Measurement of biologically active serum $iMg^{2+}$ level could be considered in epileptic children with recurrent FTS. A large-scale prospective study is warranted.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.210-214
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• 2018

Purpose: A relationship between Febrile seizure (FS) and iron deficiency anemia (IDA) has been found in several studies. However, few studies have focused on the role of IDA in complex febrile seizures (CFS) and simple febrile seizures (SFS) and there is no report on whether IDA is a risk factor for recurrence. The aim of this study was to investigate the role of IDA in SFS and CFS and to examine the effect of IDA on recurrence. Methods: Patients (n=166) who had been diagnosed with FS were enrolled in our study. Subjects were divided into the following groups for analysis: the SFS and CFS groups, recurrence and non-recurrence groups. The onset age was compared in each group of patients and laboratory test results based on IDA were compared. Results: Between the SFS and the CFS groups, there was no significant difference in laboratory test results based on IDA. There was a significant difference in onset age between the two groups and the onset age tended to be lower in the CFS group (24.00 vs. 16.49 months) (P=0.004). Comparing recurrence and non-recurrence groups, the mean corpuscular volume was significantly different (P=0.043) with the recurrence group having a lower mean corpuscular volume level (78.92 vs. 77.48). The onset age in the recurrence group was lower (26.02 vs. 19.68 months). Conclusion: This study suggests that onset age could be a risk factor for CFS, and IDA may not contribute to elevating the risk of CFS. However, IDA may play an important role in the recurrence of FS.