The Effect of Intrauterine Irradiation on Cortical Neurons and Glial Cells

PURPOSE: Our study was an experimental model for the mechanism of cortical dysplasia. We examined the changes of neuronal cells and glial cells by intrauterine irradiation. This paper will elucidate the effect of these changes on the development of cortical dysplasia. METHODS: The cytotoxic effect of irradiation was examined by viability and numbers in cerebral cortical neurons and glial cells, which were derived from a mouse exposed to 225 cGy of gamma-irradiation on embryonic day 17. In addition, the protective effect of an inhibitor of intracellular calcium release, dantrolene sodium(DS), on irradiation-induced neurotoxicity was examined after DS(10 mg/kg) was administrated via intraperitoneal injection after intrauterine irradiation. RESULTS: 1) Irradiation induced the decrement of the cell number and cell viability of cerebral cortical neurons in the developing stages. 2) The number of glial cells in the mouse treated with intrauterine irradiation was increased in E20-P4 stages compared with the control group, but there was no difference in cell viability. 3) The glial fibrillary acidic protein(GFAP)-positive cells were seen in developing stages (E20-P4). 4) In the protective effect from neuronal cell death by intrauterine irradiation, DS attenuated cell death by an increase of neuronal cells. CONCLUSION: From these results, it is suggested that intrauterine irradiation has the neurotoxic effect as neuronal cell death and induced glial cell proliferation. A selective inhibitor of intracellular calcium release such as DS is effective in protecting neuronal cell death induced by irradiation of the intrauterine period. Cortical dysplasia induced by intrauterine irradiation may be involved in neuronal cell death and the hyperproliferation of glial cells. Intracellular calcium influx may contribute to the pathogenesis of irradiation-induced neuronal cell death.

The Effects of Antiepileptic Drugs on Hematologic Parameters and Liver Enzymes According to Antiepileptics Plasma Concentrations in Epileptic Children

PURPOSE: Successful management of epileptic patients requires complete control of seizures without adverse effect. The purpose of this study is to evaluate the hematologic effect and hepatic enzyme change of antiepileptic drugs in epileptic children and compare the changes of these values according to serum drug level. METHODS: The study included 89 epileptic children with antiepileptic drugs such as phenobarbital, valproate, and carbamazepine from May 1990 to July 1999. We classified these patients into 3 groups according to the drug they had taken; group 1 : patients treated by phenobarbital, group 2 : valproate, group 3 : carbamazepine. Baseline screening tests before the start of therapy for all patients included complete blood count(CBC) and differential, platelet count, serum alanine aminotransferase(ALT), aspartate aminotransferase(AST). The tests wee repeated at follow-up visits in 2nd week, 4th week, 6th week, 12th month on the new drug. We compared their mean hematologic and liver enzyme values, which were examined before and after taken the medications, such as white blood cell counts(WBC), red blood cell(RBC), platelets, hemoglobin(Hgb), hamatocrit(Hct), mean corpuscular volume(MCA), mean corpuscular hemoglobin(MCH), mean corpuscular hemoglobin concentration(MCHC), AST, and ALT. Statistically significant change of each value was observed according to drug blood levels. RESULTS: No significant differences were found between before and after medication on AST, ALT, Hgb, MCHC in all the groups. The WBC count diminished after medication of carbamazepine, significantly. But the correlation between WBC count and serum carbamazepine level was no statistically significant. The mean platelet count diminished significantly after medication of phenobarbital and valproate, and the correlation of maximum serum valproate level with the degree of platelets count was statistically significant. Statistically significant changes were found on MCV and MCH values before and after the medication in 3rd group. But it did not depend on carbamazepine blood level. CONCLUSION: Statistically significant correlations was found between the platelet count and the plasma valproate level. Significant increase of MCV and MCH, and decrease WBC count was observed after the medication of carbamazepine.

Malignant Neurocutaneous Melanosis: A Case Report

No abstract available.

The Diagnostic Significance of Clinical Characteristics and Neuroimaging Findings for Diagnosis of Children with Developmental Delay

PURPOSE: In order to identify developmental disability and help the patients earlier, the diagnostic significance of clinical characteristics and neuroimaging findings for diagnosis of children with developmental delay was studied. METHODS: The clinical records and diagnostic procedures including head size, gestational age, Denver developmental screening test, brain MRI, EEG, brainstem auditory evoked potential (BAEP), chromosomal karyotyping, and final diagnosis of 96 patients with developmental delay were retrospectively reviewed. RESULTS: The final diagnosis of 96 children with developmental disability were cerebral palsy (41.7%), minimal cerebral dysfunction (17.7%), developmental language disorder (14.6%), autism (13.5%), and mental retardation with or without hearing impairment (12.5%), in order of frequency. Of the 40 cases with cerebral palsy, 36 cases (90%) had abnormal MRI. There was a significant difference between cerebral palsy and the others of developmental disabilities in MRI abnormality. Of the 55 EEGs, abnormal findings was shown in 26 cases (68.9%). All of the 5 patients with developmental language disorders had normal EEG, and 20 cases (68.9%) of the 29 patients with cerebral palsy showed abnormal EEG. There was a significant difference between cerebral palsy and developmental language disorder in EEG. Prematurity was in 22 cases (22.9%) of 96 patients, 16 cases (40%) in cerebral palsy, and none in autism. There was a significant difference between cerebral palsy and autism in gestational age. Microcephaly was present in 42 cases (43.7%) among 96 patients, and 2 cases (14.3%) in developmental language disorder, 24 cases (60%) in cerebral palsy. There was a significant difference between cerebral palsy and developmental language disorder in head circumference. CONCLUSION: There was a diagnostic significant difference of head circumference and EEG, gestational age, and brain MRI for comparison of patients with cerebral palsy and developmental language disorder, cerebral palsy and autism, and cerebral palsy and the other developmental disabilities, respectively.

Clinical Study of Differential Diagnosis between Childhood Convulsive Disorders and Nonepileptic Paroxysmal Disorders

PURPOSE: Many neurologic and psychiatric behaviors can mimic epileptic seizures. Making a diagnosis of these nonepileptic paroxysmal disorders is very important to prevent inappropriate use of antiepileptic drugs. We studied the clinical characteristics of nonepileptic paroxysmal events in order to help differentiate them from true epileptic seizures. METHODS: We retrospectively reviewed 77 patients aged 2 months-15 years who visited the Pediatric Neurology clinic from January, 1990 to January, 1999 for evaluation of seizure disorders who were diagnosed as having nonepileptic paroxysmal disorders. RESULTS: Nonepileptic paroxysmal disorders were classified into 6 major groups as follows : movement disorders 19 cases (23.5%), respiratory disorders 16 (19.8%), loss of consciousness or tone 15 (18.5%), drug-induced dystonia 14 (17.3%), behavioral disorders 12 (14.8%), and perceptual disturbances 5 (6.1%). The final diagnoses of the 6 major groups were as follows : breath holding spell 16 cases (19.8%), syncope 14 (17.3%), drug-induced dystonia 14 (17.3%), pseudoseizure 6 (7.4%), sleep disorders 5 (6.1%), tics 4 (4.9%), headache 4 (4.9%), temper tantrum 4 (4.9%), sleep myoclonus 3 (3.7%), paroxysmal choreoathetosis 2 (2.5%), and masturbation 2 (2.5%). Five of the 77 patients also had idiopathic epilepsy. Breath holding spell is common in 2 months-6 years of age, syncope in 2-15 years, and pseudoseizure in 6-15 years. Monitoring in patients with pseudoseizure and abnormal eye movement with video EEG is helpful for diagnosis. CONCLUSION: Nonepileptic neurologic disorders are common and show a different incidence according to age group. The exact diagnosis of nonepileptic paroxysmal disorders using video EEG, is very important for preventing misdiagnosis and inappropriate use of antiepileptic drugs.

The Correlation of Clinical Findings of Neuronal Migration Disorders with Brain Magnetic Resonance Imaging Characteristics

PURPOSE: Beginning in the eighth week of fetal life the neuroblasts migrate from the midline to the periphery to form the gray matter of the cerebral cortex. Abnormalities of cell migration are characterized by ectopic location of neurons in the cerebral cortex. This broad group of anomalies include lissencephaly, schizencephaly, cortical dysplasia, gray matter heterotopia, and unilateral hemimegalencephaly. The purpose of this study was to correlate clinical data with anatomic data, which was evaluated by brain magnetic resonance imaging characteristics that are most useful in predicting clinical METHODS: The clinical records, EEG, and MRI findings of 20 patients with neuronal migration disorders were retrospectively reviewed. RESULTS: The 20 patients with neuronal migration disorders consisted of 11 with lissencephaly, 7 with cortical dysplasia, 2 with heterotopia, and 2 with schizencephaly. Clinically, seizure was the most common symptom in 85%, next developmental delay in 50%, and then delayed speech in 25%, motor deficit 15% in order. The main associated brain anomalies included absence of septum pellucidum in 20%, periventricular leukomalacia in 15%, and corpus callosal agenesis in 15% of Patients. Bilateral involvement of lesion in MRI was 60%, comparing to unilateral lesion in 40% of the patients. The most common involved lobes was frontoparietal region. An abnormality of EEG examination was showed in 11 cases of patients(68.7%). Patients with diffuse, bilateral lesion in MRI findings of neuronal migration disorders had significantly developmental delay than those with unilateral lesion(p=0.0007). Patients with unilateral lesion had significantly motor deficit than those with bilateral lesion(p=0.04). CONCLUSION: Seizures were the most common symptoms among neurological manifestations of neuronal migration disorders. Statistically significant correlations of delayed developement with bilateral lesion and motor deficit with unilateral lesion were found.

Immunohistochemical Study on Cytokeratin Expression in Tracheal Epithelium of Human Fetus / 체질인류학회지

Trachea is lined by a pseudostratified epithelium which usually expresses a complex mixture of stratified as well as simple epithelial-type cytokeratins. In the present work, the cytokeratin expressions was studied immunohistochemically in the tracheal epithelium and gland of human fetus at 14, 26 and 32 weeks of gestation. The primary antibodies used were CK7, 8, 10, 14, 18, AE8, 5D3, MNFl16 and AE3. In PAS-hematoxylin stain, the tracheal eithelium was composed of pseudostratified ciliated columnar type and consisted of surface, intermediate and basal layers regardless of gestational ages. The PAS positive cells, however, were decreased in number in proportion to gestational ages. The tracheal gland was not fully differentiated at 14 weeks of gestation, and had well differentiated secretory portions consisting mucous and serous cells at 26 and 32 weeks of gestation. The mucous cells and luminal border of the duct were positive for PAS stain. The tracheal eithelium showed different immunoreactivity between cartilageous and membranous portions. In general, CK7 and 5D3 were expressed in surface cells, AE8 in intermediate cells, and MNFl16 and AE3 in the cells of all layers. At 14 weeks of gestation, the tracheal epithelium immunoreacted for CK7, AE8, 5D3, MNFl16 and AE3. The premordium of tracheal gland was positive for 5D3, MNFl16 and AE3. The tracheal epithelium at 26 and 32 weeks of gestation showed same staining properties to those at 14 weeks of gestation. The duct cells at 26 weeks of gestation were immunoreactive for CK7, 8, 14, 18, AE8, 5D3, MNFl16 and AE3, and those at 32 weeks of gestation were immunoreactive for CK7, 14, 5D3, MNFl16 and AE3. The acinar cells at 26 and 32 weeks of gestation were positively stained for CK7, 8, 18, 5D3, MNFl16 and AE3. These results suggest that CK7 and 5D3 may serve as useful markers for mature cilated cells, AE8 (CKl3) for immature ciliated cells, and CKl4 for duct cells in tracheal epithelium and gland.

A case of bilateral congenital choanal atresia associated with multiple cardiac anomalies / 대한주산의학회잡지

No abstract available.

The relationship between spontaneous diuresis and changes of pulmonary function in infants with respiratory distress syndrome

To evaluate the relationship between spontancous diuresis and improvement of pulmonary function in respiratory distress syndrome, 15 premature neonates requiring mechanical ventilation for RDS, who admitted in NICU of Wonkwang University Hospital from January 1990 to June 1992, were studied. The results were as follows (results are mean+/-SEM) 1) Mean birth weight of study infants was 1.82+/-0.08Kg, and mean gestational age was 32.80+/-0.54 weeks. 2) The onset of diuresis occured at a mean age of 29.1+/-2.48 hours with maximum diuresis at a mean age of 41.9+/-1.8 hours 3) Mean urine output increased from 2.25+/-0.43ml/Kg/hr at the stage of pre 12 hours-onset of diuresis (p<0.001), then decreased 3.5+/-0.2ml/Kg/hr at the stage of after maximum diuresis (p<0.05). 4) After maximum diuresis, inspired oxygen gradient, peak inflating pressure, peak end expiratory pressure, mean airway pressure, and respiratory rate were decreased significantly (p<0.05), and alveolar-arterial oxygen gradient was decreased more significantly (p<0,01). So, We conclude that the pulmonary function was rapidly improving after maximum diuresis and these changes reflect the general principle used in ventilatory management of infants with RDS.

The risk of seizure recurrence of pediatric epileptic patients while receiving anticonvulsant drugs treatment

To evaluate the risk and factors associated with seizure recurrence in children with epilepsy while receiving the adequate anticonvulsant treament, we studied 58 patients with newly diagnosed epilepsy who were followed prospectively for a median of 26 months (range 7 to 54). The results were as follows: 1) Forty-four of the 58 patients (75.9%) had recurrence of seizure. 2) The rate of recurrence according to type of seizure was observed to be 22 patients (68.8%) in generalized tonic-clonic seizure, 6 patients (85.7%) in simple partial seizure, 5 patients (83.3%) in complex partial seizure, 3 patients (100%) in mixed seizure, 2 patients (100%) in absence, 3 patients (100%) in infantile spasm, 1 patient (100%) in atonic seizure, 2 patients (50%) in secondary generalized seizure. There was no significant difference in the risk of recurrence observed among these seizure types. 3) The risk of recurrence varied according to the history of seizure, seizure recurrence was observed in 100% of the cases with history of neonatal seizure, 72.7% of the cases with febrile convulsion, and 73.3% of the cases with non-specific history. No significant difference was observed among these past history of seizure. 4) The rate of seizure recurrence according to electroencephalographic abnormalities did not differ significantly. Seizure recurrence was noted in 13 of the 18 patients with mildly disordered tracings (72.2%), 15 of the 20 patients with moderate abnormality (75.0%), and 12 of the 16 patients with severe abnormality (75.0%). 5) Recurrence rate according to cause of seizure was more significantly frequent in those with symptomatic epilepsy than in those with idiopathic type (100% vs 70.2%, p<0.05). 6) The frequency percentage of seizure recurrence by age groups of below 1 year, 1 to 3 years, 4 to 6 years, and above 6 years at onset of seizure were 100, 66.7, 57.1, and 72.7, respectively. The rate of seizure recurrence was significantly highest in patients aged below 1 year at onset of seizure. 7) There was significant difference in seizure recurrence between those with and without abnormalities as shown by neurologic examination (100% vs 70.8%, p<0.05). 8) There was no consistent difference in valproic acid serum levels between those who had a recurrence and those who did not. The patients receiving phenobarbital had significantly high serum levels of the phenobarbital in recurrent groups than those who had no recurrence. In conclusion, factors associated with an increased risk of seizure recurrence were early age at onset of epilepsy, symptomatic epilepsy, and neurologic abnormalities. We found no associations between risks of recurrence and types of epilepsy, or electroencephalographic abnormalities.

A Case of Cor Triatriatum with Atrial Septal Defect

Cor triatriatum is a rare cardiac malformation in which the left atrium is subdivided into two chambers by a fibromuscular septum, one locates posterosuperiorly, which is connects with the pulmonary veins, and the other locates anteroinferiorly connecting with the mitral valves and left ventricie. It is often lethal in children with cor triatriatum due to congestive heart faliure which develops abruptly and rapidly. So, the most important thing is early detection of the disease. We experienced a case of cor triatriatum in 20 month-old female. She had severe symptoms related to congestive heart faliure, and the conditions showed dangerous. Echocardiography was used for correct diagnosis. The patients was improved dramatically after proper surgery. So, We reported this case and review literatures briefly.

The left parasternal movement in children with heart diseases

No abstract available.

The value of computerized axial tomography of the brain in children with central nervous system disorders

No abstract available.

The value of computerized axial tomography of the brain in children with central nervous system disorders

No abstract available.

A Case of Blue Rubber Bleb Nevus Syndrome

No abstract available.

A Case of Blue Rubber Bleb Nevus Syndrome

No abstract available.