PURPOSE: Our study was aimed to investigate the characteristics of seizures as well as to determine whether the expression of neuronal nitric oxide synthase expression(nNOS) of hippocampus has an affect in the hyperthermic seizure in developing rat. METHODS: Hyperthermic seizures were repeatedly induced twice a week for four weeks in 20-day old Spraque-Dowley rats. Fifty two rats were used as a hyperthermic group and 30 rats used as a normothermic control group. Hyperthermic seizures were induced in a water bath at 45degreesC+/-1 for 4 min. The characteristics of seizures were recorded. Using western blot, hippocampal nNOS expression was measured in normothermic control, hyperthermic non-seizure, and hyperthermic seizure groups, respectively. RESULTS:Eighty seven percent of hyperthermia exposed rats showed generalized tonic-clonic seizure most frequently. The duration of seizure was ranged from 12 to 145 sec(mean 55 sec) and the latency to seizure ranged from 158 to 240 sec(mean 204 sec). The duration of seizure was prolonged but there was no significant difference in the seizure latency as the rat exposed more number of hyperthermia. Interestingly, the expression level of hippocampal nNOS in hyperthermic seizure and hyperthermic non-seizure groups was not different from each other, however, the expression in these groups was lower than that of the control group. CONCLUSION: Our results indicate that nNOS do not have an affect in this repeated hyperthermic seizures. Further studies are required to clarify a role of nNOS in hyperthermic seizure.
Animals ; Baths ; Blotting, Western ; Fever ; Hippocampus* ; Neurons* ; Nitric Oxide Synthase Type I* ; Rats* ; Seizures* ; Water

PURPOSE: In order to determine the effect of neuronal nitric oxide synthase(nNOS) in seizure-related neuronal vulnerability of the hippocampus, the expression patterns of nNOS were examined in pentylenetetrazole(PTZ)-induced seizure groups and in PTZ seizure groups which were pretreated with nNOS inhibitors. METHODS: Male Sprague-Dawley rats weighing 200-300 g were used in PTZ(40 mg/kg)-induced seizure experiments. A specific inhibitor, 50 mg/kg 7-nitroindazole(7-NI), and a non-specific inhibitor, 50 mg/kg nitro-L-arginine(L-NA) were treated 30 min before the administration of PTZ to block nNOS. nNOS expression was evaluated by using immunohistochemical staining in the hippocampus of each group. RESULTS: The onset time of the first myoclonic jerk was markedly delayed in the 7-NI and the L- NA pretreated groups in comparison to the PTZ group. In addition, 7-NI markedly suppressed the severity of PTZ-induced seizures. The expression of nNOS in the hippocampal CA3 area was higher than that in CA1 area in the PTZ treated groups. In the L-NA pretreated groups, the expression levels in the CA3 and CA1 areas were lower than those of the PTZ treated groups. Interestingly, in the 7-NI pretreated groups, the nNOS expression levels in CA1 and CA3 areas were makedly lower than those of PTZ and L-NA pretreated groups. There was no expression in CA2 area of all groups. CONCLUSION: These results suggest that the hippocampal neurons expressing nNOS may be vulnerable to PTZ-induced seizures and that nNOS may play an important role in seizure-related neuronal vulnerability.
Animals ; Hippocampus* ; Humans ; Immunohistochemistry ; Male ; Myoclonus ; Neurons* ; Nitric Oxide ; Nitric Oxide Synthase Type I* ; Pentylenetetrazole ; Rats* ; Rats, Sprague-Dawley ; Seizures*

PURPOSE: This study was aimed to elucidate the effect of hyperthermia on neuronal nitric oxide synthase(nNOS) expression in both cerebral hemispheres after left common carotid artery occlusion in gerbils. METHODS: Using Mongolian gerbils, cerebral ischemia was produced by occluding carotid artery for 1-4 hours. Rectal temperature was maintained at 36degrees C for normothermia and 40degrees C for hyperthermia by heating pad. Western blot and RT-PCR was used to examine the nNOS and the mRNA expression. Neuronal damages were observed by histological study. RESULTS: After cerebral ischemia, mRNA of nNOS was expressed more abundantly in ischemic hemisphere than control in both normothermia and hyperthermia. Hyperthermia reduced nNOS protein expression markedly. In pathological study, neurons of hippocampal region were degenerated by ischemia. Hyperthermia by itself induced neuronal degeneration in both control and ischemic region. In immunohistochemistry of brain, there was no significant difference of nNOS expression between normothermia and hyperthermia. CONCLUSION: These findings suggest that increase in body temperature might enhance nNOS mRNA expression but reduce nNOS protein, and that hyperthermia aggravates neuronal damage by ischemia, independent of nNOS gene expression.
Blotting, Western ; Body Temperature ; Brain ; Brain Ischemia* ; Carotid Arteries ; Carotid Artery, Common ; Cerebrum ; Fever* ; Gene Expression ; Gerbillinae* ; Heating ; Hot Temperature ; Immunohistochemistry ; Ischemia ; Neurons* ; Nitric Oxide ; Nitric Oxide Synthase Type I* ; RNA, Messenger

The immature brain differs from the adult brain in its susceptibility to seizures, seizure characteristics, and responses to antiepileptic drugs. Gamma(gamma)-aminobutyric acid(GABA) is the predominant inhibitory neurotransmitter in the adult brain. GABA exerts its main action through GABAA receptors, which are coupled to a ligand-gated chloride channel. The receptor protein is a pentameric structure composed of multiple subunits of different families and isoforms. The subunit combinations vary in different brain regions and cell types and determine the functional and pharmacological properties of the receptor. The molecular composition of GABAA receptors is developmentally regulated with associated changes in functional properties. In the developing brain, the delayed onset of functional GABAergic inhibition, and the overabundance of excitatory receptors make the immature brain quite susceptible to seizures. Recurrent seizures in early-life may contribute to seizure susceptibility and epileptogenesis, although the pattern of seizure-induced injury is age-related. The changes of GABAA receptors expression in tetanus toxin-induced seizure model in immature rat hippocampus are different from human temporal lobe epilpsy and experimental model in adult rat. These changes in the immature brain may reflect the induction of compensatory or homeostatic neuronal processes.
Adult ; Animals ; Anticonvulsants ; Brain ; Chloride Channels ; gamma-Aminobutyric Acid ; Hippocampus* ; Humans ; Models, Theoretical ; Neurons ; Neurotransmitter Agents ; Protein Isoforms ; Rats ; Seizures* ; Temporal Lobe ; Tetanus

No abstract available.

This study aimed to determine the long-term change of seizure susceptibility and the role of nNOS on brain development following recurrent early-life seizures in rats. Video-EEG recordings were conducted between postnatal days 50 and 60. Alterations in seizure susceptibility were assayed on day 22 or 50 using the flurothyl method. Changes in nNOS expression were determined by quantitative immunoblotting on day 50. On average, rats had 8.4+/-2.7 seizures during 10 daily 1 hr behavioral monitoring sessions. As adults (days 50-60), all rats displayed interictal spikes in the hippocampus and/or overlying cortex. Brief electrographic seizures were recorded in only one of five animals. Rats appeared to progress from a period of marked seizure susceptibility (day 22) to one of lessened seizure susceptibility (day 50). Up-regulation of nNOS expression following early-life recurrent seizures was observed on day 50. In conclusion, these data suggested that recurrent early-life seizures had the long-term effects on seizure susceptibility late in life and up-regulatory nNOS expression on the hippocampus during brain development, and nNOS appeared to contribute to the persistent changes in seizure susceptibility, and epileptogenesis.

Alexander disease (ALXD) is a rare demyelinating disease of the white matter of the brain that is caused by a mutation in the glial fibrillary acidic protein (GFAP) gene. The overexpression of GFAP in astrocytes induces a failure in the developmental growth of the myelin sheath. The neurodegenerative destruction of the myelin sheath of the white matter is accompanied by an accumulation of abnormal deposits of Rosenthal fibers in astrocytes, which is the hallmark of ALXD. The disease can be divided into four groups based on the onset age of the patients: neonatal, infantile, juvenile, or adult. Early-onset disease is more severe, progresses rapidly, and results in a shorter life span than late-onset cases. Magnetic resonance imaging and genetic tests are mostly used for diagnostic purposes. Pathological tests of brain tissue for Rosenthal fibers are definitive diagnostic methods. Therapeutic strategies are being investigated. Ceftriaxone, which is an enhancer of glial glutamate transporter (GLT-1) expression, is currently in clinical trials for the treatment of patients with ALXD. To date, there are no clinically available treatments. The cause, pathology, pathophysiology, inheritance, clinical features, diagnosis, and treatment of ALXD will be reviewed comprehensively.
Adult ; Age of Onset ; Alexander Disease* ; Amino Acid Transport System X-AG ; Astrocytes ; Brain ; Ceftriaxone ; Demyelinating Diseases ; Diagnosis ; Glial Fibrillary Acidic Protein ; Humans ; Magnetic Resonance Imaging ; Methods ; Myelin Sheath ; Pathology ; Wills

PURPOSE: This study is for being aware of clinical manifestations in neonates with tachypnea; discussing the early clinical characteristics that would lead to prolonged symptoms; and investigating the predictive factors that would cause more morbid and progressive diseases other than transient tachypnea of the newborn (TTN). METHODS: Based on the medical records, we retrospectively investigated 44 neonates who were admitted to the neonatal intensive care unit in Dongguk University Hospital of Pohang for tachypnea, from March 1, 2003 to December 31, 2005. RESULTS: The patient characteristics showed male predominance of 2.6:1. Among the 44 neonates with tachypnea, TTN (24 cases, 54.5%) showed the highest incidence, followed by pneumonia (6 cases, 13.6%), idiopathic respiratory distress syndrome (5 cases, 11.4%), meconium aspiration syndrome (4 cases, 9.1%), cardiac disease (2 cases, 4.5%), sepsis (2 cases, 4.5%) and neonatal asphyxia (1 case, 2.3%). We compared TTN group with other groups including pneumonia, idiopathic respiratory distress syndrome, and meconium aspiration syndrome. We observed less incidence of premature rupture of membrane (PROM) (P<0.05); less likely to develop tachypneic symptom after 12 hours postpartum (P<0.05); shorter duration of tachypneic symptom (P<0.05); and less incidence of nasal flaring (P<0.05) in TTN group. CONCLUSION: The higher chance of pulmonary complications was observed in tachypneic neonates with either history of PROM or onset of tachypneic symptom after 12 hours postpartum. The prolonged tachypneic symptom and combined pulmonary complications were seemed to be accompanied with neonates showing nasal flaring.
Asphyxia ; Gyeongsangbuk-do ; Heart Diseases ; Humans ; Incidence ; Infant, Newborn* ; Intensive Care, Neonatal ; Male ; Meconium Aspiration Syndrome ; Medical Records ; Membranes ; Pneumonia ; Postpartum Period ; Retrospective Studies ; Rupture ; Sepsis ; Tachypnea* ; Transient Tachypnea of the Newborn

Crohn disease is a chronic transmural inflammatory disease that may involve any portion of the gastrointestinal tract. An increased incidence of Crohn disease in the general population has been reported, along with a greater than threefold increase of Crohn disease in children under the age of 16 years noted in a recent study. Crohn disease may be seen as early as infancy, but the most common pediatric age of onset is during the teenage period. We experienced a case of Crohn disease in 6 year old male child complained abdominal pain, oral aphthous ulcers, arthralgia, anorexia, and growth failure. A brief review of related literature is also presented.
Abdominal Pain ; Age of Onset ; Anorexia ; Arthralgia ; Child ; Crohn Disease* ; Gastrointestinal Tract ; Humans ; Incidence ; Male ; Stomatitis, Aphthous

PURPOSE: Intravenous gamma globulin(IVGG) treatment has reduced symptoms and complications in Kawasaki disease(KD). However, fever persisted in 20-30% of the patients, and there are no reliable data on the indication and dosage of IVGG re-treatment. Therefore, we tried to reveal the effectiveness of IVGG re-treatment and to find risk factors in predicting the re-treatment. METHODS: Among 57 patients with typical KD, 47(82.5%) patients were put into group A, which improved after the treatment with standard 2g/kg of IVIG. 10(17.5%) patients were put into group B, which retreated with 1-2g/kg of IVIG due to persistent fever for at least 3 days after standard IVIG therapy. We compared clinical symptoms, laboratory findings and echocardiograms between group A and B, retrospectively. RESULTS: All patients in group B responded IVGG re-treatment and no considerable side effects. The total duration of the fever was significantly longer(P<0.001) and the initial and peak levels of CRP and the peak levels of ESR were significantly higher(P<0.01) in group B compared to group A. Even though leukocytosis, cervical lymphadenitis and coronary artery aneurysm were more frequent, and the levels of serum lipids at admission were lower in group B, without significance. CONCLUSION: IVGG re-treatment appeared to be effective in the treatment of refractory KD, but could not reduce the incidence of coronary artery aneurysm. We concluded it was difficult to predict risk factors for IVGG re-treatment from these data. Further studies are needed to determine the indication and appropriate dosage of IVGG re-treatment.
Aneurysm ; Coronary Vessels ; Fever ; gamma-Globulins* ; Humans ; Immunoglobulins, Intravenous ; Incidence ; Leukocytosis ; Lymphadenitis ; Mucocutaneous Lymph Node Syndrome* ; Retrospective Studies ; Risk Factors