PURPOSE: Studies gave conflicting results as to the association between febrile seizures(FSs) and IL1B promoter polymorphisms. In the present study, to determine whether or not the function-related two single nucleotide base C/T biallelic polymorphisms in the promoter region at positions -31 and -511 of the IL1B gene are associated with susceptibility to FSs, the frequencies of the polymorphisms were investigated in children with FSs and GEFS+, and normal control subjects. METHODS: 72 FSs, 23 GEFS+ and 174 healthy control subjects were selected throughout a collaborative study of Catholic Child Neurology Research Group. IL1B promoter -31 C/T and -511 C/T genotyping was performed by means of PCR-restriction fragment length polymorphism. RESULTS: The distribution of IL1B -31 genotypes and the frequencies of allele in children with FSs and GEFS+, and healthy control subjects were not significantly different. The distributions of IL1B -31 genotypes(CC, CT, TT) are 22.2%, 50%, and 27.8% in children with FSs, 21.7%, 43.5% and 34.8% in children with GEFS+, and 27.6%, 49.3% and 24.1% in healthy control subjects. The distribution of IL1B -511 genotypes and the frequencies of allele in children with FSs and GEFS+, and healthy control subjects were not significantly different. The distributions of IL1B -511 genotypes(CC, CT, TT) are 23.6%, 47.2%, and 29.2% in children with FSs, 26.1%, 39.1% and 34.8% in children with GEFS+, and 27.6%, 49.3% and 24.1% in healthy control subjects. CONCLUSION: Theses data suggest that genomic variations of IL1B promoter might not be one of the susceptibility factors for FSs in the Korean population.
Alleles ; Child ; Genotype ; Humans ; Interleukin-1beta* ; Neurology ; Promoter Regions, Genetic ; Seizures, Febrile*

PURPOSE: Hyponatremia may be common in febrile convulsions and lower the threshold for febrile convulsions. We evaluated the association between hyponatremia and febrile convulsions and also examined the effect of hyponatremia on the recurrence of convulsions during the same febrile illness. METHODS: Serum sodium levels were measured from 98 children with febrile convulsions, among whom there were 21 recurrent and 77 non-recurrent patients during the same febrile illness. Also, as a control group, we selected 32 febrile and 48 non-febrile children, who did not have febrile convulsions. Results were analyzed by Student's t-test and logistic regression. RESULTS: The average serum sodium level in febrile convulsions was 135.5+/-3.7 mEq/ L, which was significantly lower than 138.7+/-3.2 mEq/L of febrile children and 138.0+/-3.0 mEq/L of non-febrile children in the control group(P<0.05). The average serum sodium level in recurrent febrile convulsions during the same febrile illness was 133.1+/-4.1 mEq/ L, which was significantly lower than 136.1+/-3.3 mEq/L in non-recurrent febrile convulsions(P<0.05). CONCLUSION: The serum sodium levels of the patients with febrile convulsions were significantly lower than those of the children in the control group. Also, the lower the sodium levels were, the higher recurrent febrile convulsions during the same febrile illness occurred.
Child ; Humans ; Hyponatremia ; Logistic Models ; Recurrence ; Seizures ; Seizures, Febrile* ; Sodium*

PURPOSE: Recently, many studies on febrile convulsions again suggest that the degree of pyrexia may be related to the recurrence of febrile convulsions. In a previous study, we advocated that a low body temperature during the initial febrile convulsions is associated with an increase of recurrent febrile convulsions. Therefore, we have expanded the study by including 246 febrile convulsions during 6 years and investigated risk factors and especially the relationship between pyrexia and the recurrence rates. METHODS: Children with febrile convulsions were divided into three groups according to the degree of fever. Group I showed body temperatures higher than 39.5 degrees, group II from 38.5 to 39.4 degrees, and group III lower than 38.4 degrees. Then, we analyzed the recurrence rates of febrile convulsions. RESULTS: There occurred recurrent febrile convulsions in 19(41.3%) children with family history of febrile convulsion and 5(35.7%) children whose first-degree relatives diagnosed epilepsy. In group I, 5(13.5%) infants aged 6-18 months and 5(19.2%) aged 19-30 months had recurrent febrile convulsions. In group II, 22(36.1%) infants aged 6-18 months and 8(24.2%) aged 19-30 months had recurrent febrile convulsions. In group III, 21(42.0%) infants aged 6-18 months and 8(38.1%) aged 19-30 months had recurrent febrile convulsions. CONCLUSION: Children with a lower degree of pyrexia and also younger age at the onset of the first febrile convulsion were more susceptible to recurrent febrile convulsios than otherwise.
Body Temperature ; Child ; Epilepsy ; Fever* ; Humans ; Infant ; Recurrence* ; Risk Factors ; Seizures, Febrile*

The prevalent ages at onset for Kawasaki Disease (KD) and Epstein-Barr virus (EBV) infection are known to be similar in Korea and Japan. We evaluated the correlation between EBV infection and KD. The antibodies to EBV such as anti-viral capsid antigen (VCA) IgG and IgM, anti-diffuse and restricted early antigen IgG (anti-EADR IgG), and the anti-EBV determined nuclear antigen IgG (anti-EBNA IgG) were examined in 29KD patients at five separate times sequentially during a period of one year, and also in 14 other children with a past history of KD. The results of each group were compared with those of age-matched controls. The positive rates of anti-VCA IgG and IgM at presentation in the KD patients were 41.4% (12/29) and 0% (0/29), respectively. Only one patient was found to be anti-VCA IgM-positive within two months. There were no cases of anti-VCA IgG except one, anti-EADR IgG and anti-EBNA IgG positive to negative seroconversion during the year. The children with a past history of KD showed higher anti-EBNA IgG-positive rates than the controls (p=0.04). There was no difference in the seropositive rates of the antibodies to EBV, cytomegalovirus, herpes simplex virus and herpes zoster virus. In conclusion, children with KD were noted to have normal immune responses to EBV infection. Children with a past history of KD seemed to be infected with EBV at a later age than children with no history of KD.
Mucocutaneous Lymph Node Syndrome/*virology ; Male ; Korea ; Infant ; Immunoglobulins/metabolism ; Immunoglobulin M/chemistry ; Immunoglobulin G/chemistry ; Humans ; Herpesvirus 4, Human/*metabolism ; Female ; Epstein-Barr Virus Infections/*complications ; Child, Preschool ; Antibodies, Viral/*chemistry ; Age of Onset

PURPOSE: The goal of the present study was to investigate the effects of 4-aminopyridine(4-AP) on the excitability of visual cortex, observe the induction of epileptiform activity and define the characteristics of spontaneous activity. METHODS: We divided 19 to 23-day-old Sprague-Dawley rats into 3 groups by the concentration of 4-AP:5(n=10), 50(n=11), and 100(n=12) microM. The slices from their brains were incubated in artificial CSF for 1 hour, and then extracellular recordings were performed. RESULTS: Spontaneous epileptiform activities were observed in 50 and 100 microM 4-AP groups. The latencies of interictal epileptiform activity were 7.8+/-1.1 and 5.8+/-0.9 min, the frequencies 1.8+/-0.2 and 24.1+/-6.6 min-1, the amplitudes 0.7+/-0.1 and 2.8+/-0.5 mV, and the durations 238.0+/-57.8 and 242.2+/-70.0 ms in 50 and 100 microM 4-AP groups respectively. The latencies of ictal epileptiform activity were 21.0+/-9.8 and 6.7+/-2.3 min, the frequencies 116.2+/-46.7 and 193.7+/-26.4/event, the amplitudes 3.1+/-0.8 and 2.8+/-0.9 mV, and the durations 26.9+/-27.6, 35.2+/-12.6 s in 50 and 100 microM 4-AP groups respectively. CONCLUSION: 4-AP showed increased excitability in the visual cortex and induced interictal and ictal spontaneous epileptiform activity. This induction was decreased by D- AP5 or CNQX. Those results suggest that both types of inotropic excitatory amino acid receptors are overactivated and contribute to seizure initiation and propagation.
4-Aminopyridine* ; 6-Cyano-7-nitroquinoxaline-2,3-dione ; Brain ; Rats, Sprague-Dawley ; Receptors, Glutamate ; Seizures ; Visual Cortex

PURPOSE: Febrile seizures are characterized by a heterogenous phenotype segregating as an autosomal dominant trait with incomplete penetrance. Mutations in GABRG2 gene were identified in two families with generalized epilepsy and febrile seizures plus (GEFS+) and with absence epilepsy and febrile seizures(FSs). The present study assessed the role of GABRG2 gene in FSs and GEFS+ of the Korean population. METHODS: 66 FSs, 20 GEFS+ and 94 healthy control subjects were selected throughout a collaborative study of Catholic Child Neurology Research Group. The SNP211037 of GABRG2 was screened by DHPLC. DNA fragments showing variant chromatograms were subsequently sequenced. Genotypes and allelic frequencies for GABRG2 gene polymorphism in three groups were compared. RESULTS: The number of individuals with the GABRG2(SNP211037)-C/C genotype in patients with FSs was significantly greater compared with that in healthy control subjects and the GABRG2(SNP211037)-C allele frequency in patients with FSs was significantly higher than that in healthy control subjects. The odds ratio for developing FSs in individuals with the GABRG2(SNP211037)-CC genotype was 5.96 compard with individuals with the GABRG2(SNP211037)-T/T genotype. In contrast, the GABRG2 (SNP211037) gene in GEFS+ and control groups was not significantly different. CONCLUSION: Theses data suggest that genomic variations of GABRG2 might be one of the susceptibility factors for FSs in the Korean population.
Child ; DNA ; Epilepsy, Absence ; Epilepsy, Generalized ; Gene Frequency ; Genotype ; Humans ; Neurology ; Odds Ratio ; Penetrance ; Phenotype ; Polymorphism, Single Nucleotide* ; Seizures, Febrile*

PURPOSE: In order to elucidate the actual mechanism and the optimal concentration of Lamotrigine(LTG) that suppresses epileptiform discharges, we observed epileptiform discharges from hippocampal slices of immature rat in 4-aminopyridine(4-AP) added Mg2+ - free medium of artificial cerebrospinal fluid(aCSF) with various LTG concentrations. METHODS: We divided 19-23 day-old Sprague-Dawley rats into 4 groups; control group(n=12) and 3 LTG groups depending on the concentrations of LTG such as 400 (n=9), 800(n=7), and 1,000(n=8) microM. The rats were anesthetized and their brains were taken, soaked in aCSF(NaCl 125 mM, KCl 2.5 mM, NaH2PO4 2 mM, MgSO4 1.25 mM NaHCO3 25 mM, CaCl2 2 mM, Glucose 10 mM, pH 7.3-7.4). And then the brains were cut into 400 microm hippocampal slices by a vibratome. The slices of control group were soaked in 200 microM 4-AP added Mg2+ -free medium of aCSF for 1 hour, and then extracellular recordings were performed in hippocampal CA1 pyramidal region. The slices of LTG groups were soaked in the solution containing 400, 800, and 1,000 microM LTG, then extracellular recordings were performed. RESULTS: Interictal discharges were observed in all the control and the LTG groups. The latency to the first interictal discharges after 4-AP addition was 52.7+/-26.9 sec in control group, but was 225.0+/-28.2 sec in 800 microM and 322.1+/-116.4 sec in 1,000 microM group of LTG(P<0.05). The duration of interictal discharges was 64.6+/-35.6 sec in control group, but was the shortest in 800 microM group of LTG at 39.3+/-12.6 sec. Ictal discharges were observed in all of control and 400 microM group, but the frequency was decreased as the concentration of LTG increases, 57.1% in 800 microM, 12.5% in 1,000 microM group. The latency to ictal discharge after 4-AP addition was 142.1+/-52.6 sec in control group, but increased as the concentration of LTG increases, 304.4+/-84.5 sec in 400 microM group and 689.8+/-213.1 sec in 800 microM group(P<0.05). The duration of ictal discharges was 1,534.7/-339.3 sec in control group, but decreased as the concentration of LTG increases, it was 126.5+/-76.1 sec in 800 microM group(P <0.05) and 42 sec in 1,000 microM group. CONCLUSION: The antiepileptic effects of LTG were most significant when the concentration, inhibiting epileptiform discharges induced by 4-AP and Mg2+ -free medium in hippocampal slices of immature rats, was 800 microM or higher. Although the basic pharmacologic mechanism of LTG is the inhibition of sodium channel, it may also work on potassium channel at higher concentrations.
4-Aminopyridine* ; Animals ; Brain ; Glucose ; Hydrogen-Ion Concentration ; Potassium Channels ; Rats* ; Rats, Sprague-Dawley ; Sodium Channels

PURPOSE: Treatment efficacy for children with speech and language delay has been the subject of considerable debate in recent years. We evaluated the clinical features of children with delayed speech and language and their prognoses according to their etiologies after 6 months of speech and language therapy. METHODS: From January, 2000 to March, 2004, we retrospectively reviewed 56 children with speech and language delay who were administered speech and language therapy for 6 months in Uijongbu St. Mary's Hospital. RESULTS: Of 56 cases, the proportion of developmental language disorder was 66.1 percent, structural malformation 19.6 percent, mental retardation 12.5 percent, hearing defect 1.8 percent. The ratio of male to female was 4.6: 1 and the most frequent age group was over 47 months. The mean age of first spontaneous words with useful meaning was 15.9 months. The mean gestational age of the subjects was 39.8 weeks. The proportion of full-term infants was 96.4 percent and of premature infants was 3.6 percent. As for the birth order, the proportion of the first baby was 51.8 percent, the one of second babies it was 42.9 percent, and percent of third babies it was 7.1 percent. After 6 months of language intervention, 32.4 percent of patients with developmental language disorder showed normal linguistic development. All the patients with mental retardation showed sustained language and speech delay. As for the patients with structural malformations, five out of 11 patients showed normal linguistic development. CONCLUSION: The relatively advanced old age of majority of participants in this study suggests the necessity of screening test for language delay in this local community.
Birth Order ; Child* ; Female ; Gestational Age ; Hearing ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intellectual Disability ; Language Development Disorders* ; Language Therapy* ; Linguistics ; Male ; Mass Screening ; Prognosis ; Retrospective Studies ; Treatment Outcome

PURPOSE: To study the availability of the radiological classification for incongruent hallux valgus deformities, which has been used as the key to the algorithm for selecting surgical options. MATERIALS AND METHODS: To determine radiological severity, 257 cases of incongruent hallux valgus deformities were studied. The hallux valgus angle (HVA) and the 1/2 intermetatarsal angle (IMA) were measured in each case. Following Mann's radiological classification system, the HVA and IMA were compared to determine any similarity in severity. The distal metatarsal articular angle (DMAA) was also measured to observe the extent of its effect on the incongruent deformity. RESULTS: The HVA was 36.1degrees on average (range, 16-60degrees) and the 1/2 IMA was 15.8degrees on average (range, 5-30degrees). In 126 cases (49.1%), the severity between the 1/2 IMA and HVA coincided with the index classification, whereas. 131 cases (50.9%) did not correspond. Overall, the severity of approximately half the cases, could not be graded. The DMAA was 15.6degrees on average (range, 0-40degrees) and 133 cases (51.8%) demonstrated a value above normal value (>15degrees). CONCLUSION: The radiological classification system used as a guide for surgical decision-making needs to be revised, due to the overt limits of correspondence between the severity of the 1/2 IMA and HVA. Because the HVA would be influenced by both the lateral subluxation and bony deformity around the first metatarsophalangeal joint, it is reasonable to consider these factors on the HVA as elements to be corrected individually, instead of the HVA alone.
Classification* ; Congenital Abnormalities* ; Hallux Valgus* ; Hallux* ; Metatarsal Bones ; Metatarsophalangeal Joint ; Reference Values

PURPOSE: The greatest concern for children with febrile seizures is not only the possibility of epilepsy, but also unprovoked seizures. The present study examined the risk and predictors of unprovoked seizures. Study factors include three identified factors of unclear significance-family history of febrile seizures and the number of recurrent febrile seizures and two new factors that are important predictors - the height of temperature and the duration of fever prior to the initial febrile seizure. METHODS: Children(n=333) between 6 months and 5 years of age with first febrile seizures were reviewed to determine the risk and predictors of unprovoked seizures for 10 years. Children with the central nervous system infections(meningitis or encephalitis), past history of febrile seizures or epilepsies were excluded. RESULTS: 10(43.5%) of 23 children with neurodevelopmental abnormalities had epilepsies. 12(10%) of 120 children with complex febrile seizures had epilepsies. 17(6.6%) of 256 children without family history of febrile seizures in 1 degrees relative and 8(10.4%) of 77 children with family history of febrile seizures in 1 degrees relative had epilepsies. CONCLUSION: In our results, there exists a strong association between unprovoked seizures and complex features in neurodevelopmentally abnormal children compared with normal children.
Central Nervous System ; Child* ; Epilepsy ; Fever ; Humans ; Seizures* ; Seizures, Febrile*