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Jong Geun KIM ; Yoon Kyung LEE ; Byung Joon CHOI ; In Goo LEE ; Kyung Tai WHANG

Journal of the Korean Child Neurology Society.1999;6(2):400-403.

The septo-optic dysplasia, or de Morsier syndrome is a developmental anomaly characterized by involvement of the septum pellucidum, optic system and hypothalamic-pituitary axis. We had experienced a case of septo-optic dysplasia in 8 month-old female. A magnetic resonance imaging of the brain showed isolated absent septum pellucidum. And ophthalmoscopic examination showed right optic nerve hypoplasia, exotropia of right eye.
Axis, Cervical Vertebra ; Brain ; Exotropia ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Septo-Optic Dysplasia* ; Septum Pellucidum

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The Effect and Factors Affecting on Lipid Status of Valproate Therapy in Children with Epilepsy.

Eung Seok KIM ; Chang Bum KOH ; Eun Joo BAE ; Hong Jin LEE ; Won Il PARK ; Kyoung Ja LEE

Journal of the Korean Child Neurology Society.2003;11(2):249-255.

PURPOSE: Antiepileptic drugs may alter serum lipid status in epileptic patients. We conducted this study to assess the effect of valproate on serum levels of total cholesterol(TC), triglycerides(TG), low-density lipoprotein(LDL), high-density lipoprotein(HDL), and TC/HDL ratio, and to investigate the factors affecting serum lipid status in children with epilepsy who had been receiving valproate therapy. METHODS: Thirty epileptic children(16 males, 14 females, mean age 7.4+/-3.3 years) were evaluated for serum lipid status at the onset and the 6, 12 and 24 months of valproate therapy, and were analysed changes and potential factors of affecting changes such as sex, body mass index, valproate concentration, in serum lipid levels during valproate therapy. RESULTS: TC were significantly lowered during first 12 months of valproate theraphy (P<0.05). LDL were lowered during first 12 months. HDL and TC/HDL ratio were not changed and TG were increased during valproate theraphy but not reach to statistical significance. TC, TG, LDL, and HDL return to pretreatment levels after 24 months of valproate theraphy. TC, LDL, HDL, and TC/HDL ratio changes were not significantly different by sex and initial body mass index, but TG were significantly increased in group of BMI below 20(P<0.05). LDL levels were significantly decreased correlation to serum valproate concentration(r=-0.2915. P<0.05). CONCLUSION: Our results suggest that valproate therapy would not increase a risk for atherosclerotic disorders in adulthood, but weight gain with a metabolic consequence of obesity would increase risk for atherosclerotic disorders in adulthood.
Anticonvulsants ; Body Mass Index ; Child* ; Epilepsy* ; Female ; Humans ; Male ; Obesity ; Valproic Acid* ; Weight Gain

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The Role of ATP on Reactive Oxygen Species Generation and Cellular Proliferation in BV-2 Cells.

Young Hoon KIM ; Jeung Ki CHO ; Pil Woo HUH ; Young LIM ; Hyoung Kyun RHA ; Kyung Tae WHANG

Journal of the Korean Child Neurology Society.2003;11(2):238-248.

PURPOSE: Adenosine triphosphate (ATP) evokes several cellular responses in microglia including propagation. However, the role of the purinoceptor on ROS generation in microglia is unclear. In order to determine the action of the purinoceptor in microglia, the effects of ATP on ROS generation and cellular proliferation in BV-2 murine microglial cells were evaluated. An additional aim of this study was to investigate signal transduction pathways using several inhibitors. METHODS: The [Ca2+] was measured using Ca2+ sensitive indicator, Fura-2/AM. ROS production was observed by fluorescence-confocal microscope and cell proliferation was evaluated by counting cell number. RESULTS: ATP increased the intracellular calcium levels ([Ca2+]i) in BV-2 cells in a dose-dependent manner. This increase was attenuated by pretreatment with a calcium chelator (EGTA) and a phospholipase C (PLC) inhibitor (U-73122) while the protein tyrosine kinase (PTK) inhibitor (genistein) had no inhibitory effects. To identify the effects of the nucleotides, ROS generation was observed in the nucleotide-stimulated BV-2 cells. The treatment with 100 M ATP induced ROS generation, but 100 M adenosine and 100 M UTP did not. To investigate the signal transduction pathway in ATP-induced ROS generation, several inhibitors were pretreated before adding ATP. ATP- induced ROS production was blocked by pretreatment with either 0.5 mM EGTA or 10 M U73122 while 40 M genistein had an inhibitory effect on ATP action. Correspondingly, 40 M KN62 (CaM kinase II inhibitor), 1 M sphingosine (protein kinase C inhibitor), 1 nM DPI (NADPH oxidase inhibitor) and 50 M mepacrine (phospholipase A2 inhibitor) could suppress ATP-induced ROS generation. The effects of ATP on cell proliferation was observed 3 days after ATP treatment and its peak velocity after 4 days. NF-kB activation was observed after the cells were incubated with 0.1 mM ATP. The maximal level of NF-kB activation was obtained with 0.3 mM ATP while higher concentrations were less effective. CONCLUSION: Overall, we conclude that ATP in BV-2 cells induces ROS generation and cell propagation. The signal transduction pathways including calcium, CaM kinase II, PLC, protein kinase C, phospholipase A2 and NADPH oxidase are involved in ATP-induced ROS generation.
Adenosine ; Adenosine Triphosphate* ; Calcium ; Calcium-Calmodulin-Dependent Protein Kinase Type 2 ; Cell Count ; Cell Proliferation* ; Egtazic Acid ; Genistein ; Microglia ; NADPH Oxidase ; NF-kappa B ; Nucleotides ; Oxidoreductases ; Phospholipases A2 ; Phosphotransferases ; Protein Kinase C ; Protein-Tyrosine Kinases ; Quinacrine ; Reactive Oxygen Species* ; Receptors, Purinergic ; Signal Transduction ; Sphingosine ; Type C Phospholipases ; Uridine Triphosphate

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The Protective Effect of Chlorpromazine on Flurothyl-Induced Seizure.

Dong Wook KIM

Journal of the Korean Child Neurology Society.2003;11(2):234-237.

PURPOSE: Chlorpromazine(CPZ) is known to inhibit glutamate ehydrogenase(GDH). Reductive amination of alpha-ketoglutarate is catalyzed by GDH and forms glutamate, a major excitatory neurotransmitter. Thus, I hypothesized that CPZ might have a seizure- protective effect by inhibiting glutamate release from the excitatory presynaptic nerve terminal. The present study was designed to investigate the protective effect of CPZ on flurothyl-induced seizure in rats. METHODS: Twenty-eight male Sprague-Dawley rats were equally divided into 2 groups. CPZ(20 mg/kg) was administered to experimental animals by subcutaneous injection, while normal saline to control animals. Twenty minutes later, seizures were chemically induced by flurothyl infusion(40 microL/min). Seizure susceptibility was defined as the latency from the start of flurothyl infusion to the onset of a generalized seizure(loss of posture with bilateral hindlimb tonic extension). Shorter latency reflects greater seizure susceptibility. RESULTS: The mean(+/-SEM) seizure latency in the experimental group was 539.2 (+/-17.5) seconds, and it was significantly longer than 432.4(+/-21.9) seconds in the control group(P<0.001). CONCLUSION: This study demonstrates that CPZ decrease flurothyl-induced seizure susceptibility in rats. This result suggests that CPZ may have a seizure-protective effect. I hope that further studies on this issue should be performed in a near future.
Amination ; Animals ; Chlorpromazine* ; Flurothyl ; Glutamate Dehydrogenase ; Glutamic Acid ; Hindlimb ; Hope ; Humans ; Injections, Subcutaneous ; Male ; Neurotransmitter Agents ; Posture ; Rats ; Rats, Sprague-Dawley ; Seizures*

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c-Jun Protein Expression and Seizure-induced Neuronal Damage on Hippocampal Explant Cultures of Rat in Different Maturation Stage.

Soo Ahn CHAE ; Kwang In LEE ; Eung Sang CHOI

Journal of the Korean Child Neurology Society.2003;11(2):225-233.

PURPOSE: Immaure brain is more resistant to seizure-induced neuronal damage than the adult brain in animal study. Immediate early genes such as c-jun play a critical role in neuronal damage. Therefore, we hypothesized that the difference of constitutive and electrically stimulated c-Jun expression would explain the difference in neuronal damage from seizures between immature and mature explant culture. METHODS: Seven and 14 days-in-vitro(DIV) hippocampal explant cultures derived from 8-day-old rat pups were used. Extracellular field recording was done in cultures. A 1-sec stimulus train(60 Hz, 0.1 msec rectangular pulses) was applied to the Schaffer collaterals, and the afterdischarge was recorded in CA1 pyramidal layer. Cultures were returned to the incubator and observed serially. Intensity of propidium iodide fluorescence indicative of neuronal damage was quantitated as percent of total damage induced by 2 mM NMDA. Proteins extracted from individual cultures were analyzed by Western blot. RESULTS: Constitutive c-Jun protein expression at 7 DIV was higher than that at 14 DIV. There was not a significant difference of c-Jun expression between the 7 DIV and 14 DIV cultures after electrical stimulation. Neuronal damage after electrical stimulation in the hippocampus at 7 DIV was significantly lower than at 14 DIV. CONCLUSION: The results show reduced neuronal injury from seizures in more immature culture. However, constitutive expression of c-Jun protein was higher. Higher constitutive expression may inhibit further induction of c-Jun from seizures and thus result in less severe neuronal injury.
Adult ; Animals ; Blotting, Western ; Brain ; Electric Stimulation ; Fluorescence ; Genes, Immediate-Early ; Hippocampus ; Humans ; Incubators ; N-Methylaspartate ; Neurons* ; Propidium ; Rats* ; Seizures

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Recent Studies and Clinical Application in Pediatric Neurology: A PET Study.

Joon Soo LEE ; Chang Jun COE

Journal of the Korean Child Neurology Society.2003;11(2):214-224.

No abstract available.
Neurology*

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Treatment for Intractable Childhood Epilepsy.

Heung Dong KIM ; Hoon Chul KANG ; Chan Jun COE

Journal of the Korean Child Neurology Society.2003;11(2):205-213.

No abstract available.
Epilepsy*

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Development Disabilities in Children: Recognition and Evaluation.

Hee Jung CHUNG ; Chang Jun COE

Journal of the Korean Child Neurology Society.2003;11(2):194-204.

No abstract available.
Child* ; Humans

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Three Cases of Symptomatic Huge Arachnoid Cysts.

Ki Hyun JEON ; Hyo Geun JU ; Tae Hyung CHO ; Keun Mo KIM ; Young Jong WOO ; Jung Kil LEE ; Jae Hyu KIM

Journal of the Korean Child Neurology Society.1998;6(1):142-148.

Congenital arachnoid cysts are commonly located at sylvian cistern or middle cranial fossa which are usually asymptomatic and incidentally found. Posterior fossa cysts, however, are usually large when diagnosed, and symptomatic. Three cases of large posterior fossa cysts were recognized on the diagnostic MRI investigation for infantile spasm, developmental delay, and the precocious puberty. Surgical decompression of the cysts by craniectomy, cyst excision and fenestration were performed successfully in two patients with arachnoid cysts in the cerebellopontine cistern and the suprasellar, right cerebellopontine, and prepontine cisterns, but an additional cystoperitoneal shunt was needed in a case with the cyst in the quadrigemial cistern with obstructive hydrocephalus. Infantile spasm was treated with vigabatrin and pyridoxine, and the true precocious puberty was managed with LHRH analogue(Decapeptyl ).
Arachnoid Cysts* ; Arachnoid* ; Cranial Fossa, Middle ; Decompression, Surgical ; Gonadotropin-Releasing Hormone ; Humans ; Hydrocephalus ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Puberty, Precocious ; Pyridoxine ; Spasms, Infantile ; Vigabatrin

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A Case of Enterobacter sakazakii Epidural Abscess in Neonate.

Jung A SEO ; Se Wook OH ; Yong Won PARK ; Sang Woo KIM ; Ki Jae LEE

Journal of the Korean Child Neurology Society.1998;6(1):138-141.

Enterobacter sakazakii previously known as a 'yellow-pigmented Enterobacter cloacae', was designated as a unique species in 1980. This reclassification was based on differences from E. cloacae in DNA relatedness, pigment production and biochemical reactions. In 1961, Urmenyi and Franklin reported the first two known cases of neonatal meningitis and septicemia caused by E. sakazakii, although at that time it was still considered as an E. cloacae strain. Since 1961, a number of cases of neonatal infection caused by E. sakazakii have been reported worldwide. Usually newborn with E. sakazakii meningitis have a poor prognosis, many of them dying within days of infection. The reported case-fatality rate vary from 40-80%. We diagnosed and experienced a case of epidural abscess caused by E. sakazakii in a 1 month old male patient, who was asymptomatic with brain mass detected on brain ultrasound examination. We presented this case with a brief review of literatures.
Brain ; Cloaca ; Cronobacter sakazakii* ; DNA ; Enterobacter* ; Epidural Abscess* ; Humans ; Infant, Newborn* ; Male ; Meningitis ; Prognosis ; Sepsis ; Ultrasonography

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