PURPOSE: Multiple sclerosis(MS) is an autoimmune demyelinating disorder causing significant neurological impairment in early adulthood. Since 1980, childhood MS has increased and the characteristics of childhood MS has been reported. For investigation of the clinical characteristics of childhood MS in Korea, we reviewed clinical presentations, paraclinical features, prognosis, and responses to interferone treatment of childhood MS patients. METHODS: We retrospectively reviewed the medical records of patients who have been diagnosed as MS according to McDonald criteria in Seoul National University Children's Hospital. 13 patients(M:F=6:7) were included in the study from 1988 to 2005. RESULTS: The age of onset was 1-12 years-old(mean 7.1) and the duration from initial presentation to 1st relapse was 3-27 months(mean 10.3). Initial presenting symptoms were motor disturbance, visual disturbance, bladder dysfunction, fever, brainstem dysfunction, headache and encephalitis-like-symptoms. All patients were treated with corticosteroids and 8 patients were on interferone-alpha treatment. EDSS score were 0-10(mean 1.8). CONCLUSION: The clinical characteristics of childhood MS were similar to the result from other studies in Korea, Japan, and eastern countries. Increasing number of childhood MS in Korea reflects the change of the environmental susceptibility to MS in Korean pediatric population. Interferone-alpha therapy can be considered as safe and effective treatment for modulating the clinical course of MS.
Adrenal Cortex Hormones ; Age of Onset ; Brain Stem ; Demyelinating Diseases ; Fever ; Headache ; Humans ; Interferons ; Japan ; Korea* ; Medical Records ; Multiple Sclerosis* ; Prognosis ; Recurrence ; Retrospective Studies ; Seoul ; Urinary Bladder

PURPOSE: The study was aimed to investigate immunogenetic peculiarities of neuroinflammatory CNS diseases in Korean children. METHODS: A total of 16 children with neuroinflammatory CNS diseases(9 males and 7 females; mean age 7.5+/-4.2 years) were consecutively recruited. Genomic typings were performed on their HLA DRB/HLA DQB genes using PCR-SSOP/SSP techniques with Gel immunoelectrophoresis. RESULTS: The frequencies of HLA-DRB1*14(38%), HLA-DRB1*15(25%), HLA-DRB3* 02(50%), HLA-DQB1*05(56%) and DQB1*06(44%) were significantly increased compared with a control group. The frequencies of HLA-DRB1*15(50%) and HLA-DQB1*06(63%) were significantly increased in children with ADEM and HLA-DRB3*0202(100%), HLA- DRB1*1302(67%), HLA-DRB3*0301(67%), and HLA-DQB1*0301(67%) in children with multiple sclerosis. HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 were found in children with acute necrotizing encephalopathy. CONCLUSION:HLA-DRB1*14, HLA-DRB1*15, HLA-DRB3*02, HLA-DQB1*05 and DQB1*06 may be associated with the susceptibility to neuroinflammatory CNS diseases in Korean children. The frequencies of HLA-DRB1*1501, HLA-DRB5*0101, HLA-DRB3* 0301, and HLA-DQB1*0602 in Korean children with multiple sclerosis were not as high as those in western children. However, HLA-DRB3*0202 was seen in all the children with multiple sclerosis. Our data may provide further evidence that the immunogenetic backgrounds of neuroinflammatory CNS diseases in Korean children are distinctly different from those in Westerns. However, further studies are needed.
Central Nervous System Diseases* ; Child* ; Encephalomyelitis, Acute Disseminated ; Female ; Humans ; Immunoelectrophoresis ; Immunogenetics ; Male ; Molecular Typing* ; Multiple Sclerosis

PURPOSE: Studies for handedness and footedness of patients with epilepsy are rare. Some studies suggest that left handedness is more common in children with injury in the left hemisphere, mental retardation and epilepsy. We analysed the correlation of handedness and footedness with epilepsy according to the cause of epilepsy and the site of brain lesion by comparing with a control group. METHODS: Subjects were 130 epileptic patients who visited the pediatric out-patient clinic of Pusan National University Hospital from June 2001 to August 2001. A control group was composed of 130 children without history of convulsion or neurologic problems. We let them carry out 10 items or answer the questions on the use of hands and feet. We defined handedness and footedness based on the number of the items carried out dominantly. We analyzed age, type of seizure, cause of epilepsy and site of brain lesions in the symptomatic group by reviewing their medical records. RESULTS: In 130 epileptic patients, left handedness and left footedness were 20.0% and 16.0%, respectively which are higher than 4.6% and 5.4% of the control group(P<0.05). There was statistically no difference between the idiopathic epilepsy group and the control group such as 8.9% and 8.9%. But left handedness in the symptomatic epilepsy group was 45.0% and left footedenss was 31.4%, which were significantly higher than those of the control group(P<0.05). According to the site of brain lesions in the symptomatic group, all the patients with abnormalities in the left hemisphere showed left handedness and 57.1% of the patients showed left footedenss. In cases of abnormalities in both hemispheres or diffuse brain lesions, left handedness was 25.9% and left footedness was 26.9%. The concordance rate of left handedness and left footedness was 66.7% (4/6) in the control group, 50.0%(4/8) in the idiopathic epilepsy group and 71.4%(10/14) in the symptomatic epilepsy group. CONCLUSION: Left handedness and footedness are more common in the epileptic patients than the control group. This is due to the high proportion of left handedness and left footedness in the symptomatic epileptic patients. Left handedness and footedness in children with epilepsy are much more related to the left side of brain lesions.
Brain ; Busan ; Child* ; Epilepsy* ; Foot ; Functional Laterality* ; Hand ; Humans ; Intellectual Disability ; Linear Energy Transfer ; Medical Records ; Outpatients ; Seizures

PURPOSE: Neonatal seizures are major morbidities of various neurological insults in neonates and have the predictive value for neurodevelopmental outcome. Nevertheless, it is difficult to detect seizures in this period and to verify underlying causes. In this study, we investigated the clinical features of neonatal seizures and proved the role of brain MRI in detection of underlying neurological insults. METHODS:Twenty-four patients(M:F=7:17) with neonatal seizures are recruited among 1,157 neonates born between Mar. 2004 and Jan. 2005. Seizure types are classified based upon Volpe's criteria. Retrospective reviews of medical records and interictal electroencephalograms are performed. RESULTS:Subtle seizure is the most common seizure type(9 patients:37.5%). In 4 patients, two types of seizures are noted. Among 20 patients with brain ultrasound, thirteen patients show normal findings. In contrast, out of 16 patients with brain MRI, fourteen patients have abnormal findings. Among 13 patients with normal brain ultrasound, seven patients have abnormal findings in brain MRI. Abnormal background activities of interictal electroencephalograms are noted in twenty one patients(87.5%). Subsequent seizures are detected in 3 patients. CONCLUSION:Clinical features of neonatal seizures are variable, and more than two types of seizures can be noted in one patient. Background activity of the electroencephalogram may help in diagnosis of neonatal seizures and Brain MRI should be considered in detection of underlying cause.
Brain ; Diagnosis ; Electroencephalography ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging ; Medical Records ; Retrospective Studies ; Seizures* ; Ultrasonography

PURPOSE: This study was undertaken to recognize the relapse rate and risk factors of relapse after discontinuation of antiepileptic drugs in patients with benign epilepsy of childhood with centro-temporal spikes(BECT). METHODS: The subjects were 57 patients with BECT, who visited our hospital from January 1990 to December 2004. They were followed up for more than 24 months after discontinuation of antiepileptic drugs. And they were analysed on the relapse rate and the factors that were presumed to influence the relapse. RESULTS: Seizures were relapsed in 7 of 57 patients(12.2%) after discontinuation of antiepileptic drugs. Relapses were more frequent in patients who took antiepileptic drugs for seizure control more than 12 months(15.0%) than those who didn't(10.8%). Moreover, more relapses occurred to those who were at higher age at onset and who took antiepileptic drugs less than 36 months(100%). Furthermore, those who had abnormalities in EEG experienced more relapses(33.3%) than others. The relapse rate was not significantly different in the aspect of and sex seizure frequency after discontinuation of medication between relapsed and non-relapsed patients. CONCLUSION: It is concluded that we should consider the risk factors of relapse in patients with BECT in order to avoid relapse when discontinuing antiepileptic drugs. Moreover, further studies are needed to clarify the risk factors of relapse.
Anticonvulsants* ; Electroencephalography ; Epilepsy* ; Humans ; Recurrence ; Risk Factors ; Seizures

PURPOSE: Infantile spam is a kind of epileptic syndrome causing delayed psychomotor development in children and one of the most common type of epileptic encephalopathy. Some cases of infantile spasm are known to be caused by focal abnormalities of cerebral cortex thus curable by operating them. Here we analyzed the results of surgical treatments on intractable epilepsy with infantile spasm. METHODS: We performed retrospective study on 22 patients with infantile spasm who received epileptic surgery since 1999. Surgical outcome, pathologic findings, localization of lesions, and types of surgery were reviewed. RESULTS: Sex ratio of our 22 cases was 1:1.4(M:F) and the age at surgery was quite variable. The mean duration from diagnosing epilepsy till operation was 6-10 years in 8(36%) cases, 1-2 years in 7(32%). In preoperative EEG, abnormalities implying possible focal lesion were seen in all 22 patients. 7(32%) out of 22 cases showed focal lesions in MRI while 12(92%) out of 13 cases in ictal SPECT, 8(62%) out of 13 cases in interictal SPECT, and 11(61%) out of 18 cases of PET examination showed abnormal findings. Concerning the types of surgery, single lobectomy was most commonly performed, in 13 (59%) cases in which 11 among them received frontal lobectomy. Also, multilobar resection was performed in 6(27%) cases and hemispherotomy in 2(9%) as well. 19(86%) patients were classified as Engel class I after operations. In pathological examination, cortical dysplasia was most commonly observed, in 11(50%) cases, microdysgenesis in 3(14%), gliosis in 2(9%), and tuberous sclerosis in 2(9%). CONCLUSION: In cases of children with infantile spam not controlled by medical treatment, epileptic surgery should be strongly considered when cortical pathology can be identified from various studies.
Cerebral Cortex ; Child ; Electroencephalography ; Epilepsy* ; Gliosis ; Humans ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Malformations of Cortical Development ; Pathology ; Retrospective Studies ; Sex Ratio ; Spasms, Infantile* ; Tomography, Emission-Computed, Single-Photon ; Tuberous Sclerosis

PURPOSE: This study sought to evaluate the clinical outcomes of various therapeutic modalities, including newly-developed anti-epileptic drugs (AEDs), prednisolone, ketogenic diet (KD) epilepsy surgery, and vagus nerve stimulation (VNS), in treating intractable childhood epilepsy. METHODS: Data of refractory epilepsy patients (n=297) treated from July 1995 through April 2003 at the epilepsy center of Sanggye Paik Hospital were retrospectively analyzed. RESULTS: Newly-developed AEDs were primarily prescribed to 290 patients, although only 20 patients (6.9%) maintained a seizure- free state. Of 138 patients for whom prednisolone was prescribed, 58 patients (42.0%) showed complete seizure controls while 41 patients experienced relapse. KD was attempted and evaluated at 12 months in 162 patients, at which time 74 (45.7%) remained on the diet and 68 (42.0%) showed seizure reduction of greater than 50%, including 37 (22.8%) who were completely seizure free. Epilepsy surgery was undergone in 38 patients, and Engel class I was identified in 25 (65.8%) patients. VNS was administered to five patients, only two of whom obtained a seizure reduction of more than 50%. RESULTS: Taken together, these findings suggest that considerable controls over intractable childhood epilepsy can be gained through KD and epilepsy surgery, whereas prednisolone treatment leads to somewhat more frequent relapses, and newly-developed AEDs are comparatively limited in their controls of refractory epilepsy.
Diet ; Epilepsy* ; Humans ; Ketogenic Diet ; Prednisolone ; Recurrence ; Retrospective Studies ; Seizures ; Vagus Nerve Stimulation

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: The goal of this study was to investigate the effect of tetraethylammonium (TEA) on the excitability of visual cortex, and observe the induction of epileptiform activity. Also, it was aimed to define the characteristics of spontaneous activity and observe the effect of GABAA antagonist, NMDA antagonist and non-NMDA antagonist on the TEA-induced epileptiform activity. METHODS: The visual cortex slices in this study were obtained from 19 to 23 day-old Sprague-Dawley rats. Extracellular cellular recording was performed to observe the induction of epileptiform discharge perfused by artificial CSF containing 1, 5 and 10 mM TEA and the effect of 10 ?M 6-cyano-7-nitroquinoxaline-dione disodium(CNQX) and 50 microM D-(-)-2-amino-5-phosphonopentanoic acid(D-AP5) on the 10 mM TEA-induced epileptiform activity. RESULTS: Spontaneous epileptiform activities were observed in 5 and 10 mM TEA groups. The addition of 5 ?M BIC blocked the TEA-induced spontaneous ictal epileptiform activity but didn't block the TEA-induced spontaneous interictal epileptiform activity. The addition of 10 ?M CNQX shortened duration, decreased frequency and amplitude of the TEA-induced spontaneous epileptiform activity. The addition of 50 microM D-AP5 blocked the TEA-induced spontaneous ictal and interictal epileptiform activity. CONCLUSION: TEA induced the increased excitability in the visual cortex and spontaneous epileptiform activity. 5 ?M BIC blocked the TEA-induced spontaneous ictal epileptiform activity and GABAA antagonist BIC plays a role in limiting the epileptiform discharge. The TEA-induced spontaneous epileptiform activity induction was decreased by CNQX and blocked by D-AP5. NMDA and non-NMDA are required to modify the TEA-induced spontaneous epileptiform activity.
6-Cyano-7-nitroquinoxaline-2,3-dione ; Animals ; GABA Antagonists ; N-Methylaspartate ; Rats* ; Rats, Sprague-Dawley ; Tea ; Tetraethylammonium ; Visual Cortex*

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