Autosomal dominant nocturnal frontal lobe epilepsy(ADNFLE) is a recently identified partial epilepsy. This disorder is characterized by a variable age of onset(mostly in childhood), autosomal dominant inheritance, clusters of brief frontal nocturnal seizures, negative findings on neuroimaging, and fairly good prognosis. Carbamazepine is known to be the most useful drug in this type of epilepsy. We experienced a case of 5-year-old boy who had unusual arousal and irritability every night for 3 years and had sharp & wave discharges from frontal lobe region in long-term video-EEG monitoring, who was suspected as an autosomal dominant nocturnal frontal lobe epilepsy. We report a case with a brief review of literatures.
Arousal ; Carbamazepine ; Child, Preschool ; Epilepsies, Partial ; Epilepsy ; Epilepsy, Frontal Lobe* ; Frontal Lobe* ; Humans ; Male ; Neuroimaging ; Prognosis ; Seizures ; Wills

BACKGROUND: Leptin, the product of ob gene, is an important circulating hormone for the regulation of homeostasis of body weight and enegy expenditure. There was a previous reports that thyroid hormone is one of regulating factors of leptin gene expression in vitro. The aim of this study was designed to evaluate the role of thyroid hormone levels in the regulation of circulating leptin concentrations in human. METHODS: A total 16S subjects were studied; 76 patients with Graves disease, 49 patients with Hashimoto disease and 43 control sujjects. The correlation between thryoid hormone and leptin levels were analyzed and serum leptin levels were compared among the groups which was classified by thyroid functional status. Serum leptin concentratios were measured by radioimmunoassay. RESULTS: There were no significant differences in serum leptin levels between the groups of control, Graves disease and Hashimoto disease. The hypothyroid groups of Graves disease which was induced by excessive antithyroid drug treatment showed significant low levels(5.6 +/-2.8 ng/mL) compared to control(9.6 +/- 5.2 ng/ml) and thyrotoxic groups(10.0 +/- 5,0 ng/mL) CONCLUSION: The hypothyroid patients showed low levels of serum leptin concentrations it may indicate that thyroid horrnone play a role in the appropriate secretion of leptin in human.
Body Weight ; Gene Expression ; Graves Disease ; Hashimoto Disease ; Health Expenditures ; Homeostasis ; Humans ; Leptin* ; Radioimmunoassay ; Thyroid Gland*

The Changes of soluble Fas levels in Patients with Autoimmune Thyroid Diseases BACKGROUD: Apoptosis was observed in thyroid tissue from Hashimoto disease but not those from Graves disease. Recently Fas and Fas ligand interactions among thyrocytes were suggested to development of clinical hypothyroidism in Hashimoto disease.Soluble Fas produced as the form lacking the tranmembrane domain due to alternative splicing, is supposed to inhibit Fas-Fas ligand interaction and blocks Fas mediated apoptosis. METHODS: In tbis study, we measured serum soluble Fas to determine the possible involvement of this molecule in the autoimmune thyroid disease by enzyme linked immunosorbant assay in 29 patients with Graves disease, 30 patients with Hashimotos disease and 19 normal controls. RESULTS: Compared with normal subjeets (4.26 +/- 1.00 U/mL), soluble Fas was not increased in patients with Graves disease (4.23 +/- 1.14 U/mL, p>0.05) but it was increased in throtoxic Graves patients (4.70 +/- 1.26 U/mL, p<0.05) compared to euthyroid Graves (3.72 +/- 0.73 U/mL, p<0.05) and normal subjects (4.26 +/- 1.00 U/mL, p<0.05). The euthyroid and hypothyroid patients with Hashimoto disease showed low soluble Fas levels, 2.94 +/- 0.54 U/mL and 2.74 U/mL, respectively compare to the patients with Graves disease and normal subjects. The thyroid hormone levels to (T3 T4 and free T4) showed positive correlation with the serum titers of antithyroid autoantibodies, antithyroglobuin antibodies, antiperoxidase antibodies and thyrotropin binding inhibitor immunoglobulins. CONCLUSION: We found that the patients with thyrotoxic Graves disease had increased level of serum soluble Fas and the patients with Hashimoto disease showed low levels of soluble Fas compared to normal controls. Increased soluble Fas in Graves disease suggests increased expression of alternatively spliced Fas mRNA variant and decreased soluble Fas in Hashimoto disease suggests decreased Fas mRNA variant and increased full length membrane Fas, so these findings are related to the promotion of apoptosis of thyroid cells during autoimmune reaction in Hashimotos disease.
Alternative Splicing ; Antibodies ; Apoptosis ; Autoantibodies ; Fas Ligand Protein ; Graves Disease ; Hashimoto Disease ; Humans ; Hypothyroidism ; Immunoglobulins ; Membranes ; RNA, Messenger ; Thyroid Diseases* ; Thyroid Gland* ; Thyrotropin

The mortality of patients with brain abscess has decreased significaltly. This has been attributed to improved diagnostic imaging, the evolution of neurosurgical techniques and understanding of intracranial pressure pathophysiology, greater critical care understanding, and newer antibiotics. However, the mortality associated with intraventricular rupture of brain abscess remained consistently high at or above 80% once identified. A case of intraventicular rupture of thalamic abscess with good quality of survival is presented based on aggressive 4-component therapeutic plan used. The four components are 1) extraventricular drainage for 6 weeks, 2) lavage of the ventricular system using closed irrigation system, 3) intravenous antibiotics, 4) intraventricular gentamicin and vancomycin, twice and once daily, respectively.
Abscess* ; Anti-Bacterial Agents ; Brain Abscess ; Critical Care ; Diagnostic Imaging ; Drainage ; Gentamicins ; Humans ; Intracranial Pressure ; Mortality ; Rupture* ; Thalamus ; Therapeutic Irrigation ; Vancomycin

Peri-vater divertieula may interfere with the normal flow of biliary and pancreatic secretion, thus leading to pancreatobiliary diseasa and the very presence of juxtapapillary diverticulum makes cannulation technically difficult and contributes to the low success rate. In this present series, the relationship between duodenal diverticula and age, sex, and biliary-pancreatic pathology was stud.ied in 1400 patients examined with ERCP. The results are as follows. 1) Duodenal diverticula was demonstrated in 73 of 1400 patients(5, 2%). 2) The incidence of duodenal divertieular increased with age and they were more frequent in men. 3) The cannulation of one or both duct system was suecessful in 69 of the 73 patients, the success rate was 94. 5%. 4) The biliary pathology was found in 41 of the 73 patients and the pancreatic pathology in 6 of the 73 patients. The rnost common finding was biliary calculi which were demonstrated in 35 patients(47. 9%).
Catheterization ; Cholangiopancreatography, Endoscopic Retrograde* ; Diagnosis* ; Diverticulum* ; Gallstones ; Humans ; Incidence ; Male ; Pathology

PURPOSE: Ketogenic diet has been frequently used in the treatment of intractable childhood epilepsy since proven strong antiepileptic effects. Because of prolonged maintenance of high fat diet, long-term side effects of hyperlipidemia and atherosclerosis has been concerned, but there are no sufficient data revealing lipid profile from ketogenic diet. This study is intended to investigate the changes of lipid profiles in patients with ketogenic diet. METHODS: Sixty-six children (0.8-14.7 yrs, mean age 4.6+/-3.6 yrs, 33 males and 33 females) with intractable epilepsy treated with the classic (4:1) ketogenic diet were studied at Inje University Sang-gye Paik Hospital, from January 1997 to July 2001. Follow-up period was more than 1year (range:1-2 yrs). Cholesterol, HDL-cholesterol and Triglyceride (TG) concentrations were measured at 1 day, 7 days, 6 months, 12 months, 18 months and 24 months, and LDL-cholesterol concentrations were measured. RESULTS: 1) Total cholesterol concentrations were significantly increased from 7 days to 24 months after ketogenic diet than that before ketogenic diet (p<0.05). 2) HDL-cholesterol concentrations were not significantly changed for 2 years. 3) LDL-cholesterol concentrations were significantly increased at 6 months (119.9 mg/dL, p<0.05), and 24 months (128.0 mg/dL, p<0.05). 4) TG concentrations were significantly increased at 7 days (164.9 mg/dL, p<0.05), 6 months (209.7 mg/dL, p<0.01), and 12 months (192.0 mg/dL, p<0.01), and were normalized at 18 months, 24 months. CONCLUSION: Most of lipid profiles were significantly changed during ketogenic diet. Long-term complication for cardio-vascular diseases should be further investigated.
Atherosclerosis ; Child ; Cholesterol ; Diet, High-Fat ; Epilepsy* ; Follow-Up Studies ; Humans ; Hyperlipidemias ; Ketogenic Diet* ; Male ; Triglycerides

BACKGROUND: In-line intravenous filters have been used in intravenous administration sets of fluid to retain bacteria and bacterial endotoxin. We evaluated the effects of intravenous filters on Staphylococcus epidermidis, Pseudomonas aeruginosa, Candida and bacterial endotoxin retention. METHODS: S. epidermidis, C. albicans, P. aeruginosa and endotoxin were injected into each 10 bags of amino acid solution. After incubation for 24 and 72 hours, aspirates of 0.1 mL from 30 bags, with filtration and without filtration, were inoculated into culture media. The effluent from bags containing endotoxin was tested by limulus amebocyte lysate test. RESULTS: When effluents were filtered before culture, those from each 10 bags containing P. aeruginosa, C. albicans and endotoxin were sterile; also there was only one positive bacterial culture among 10 effluents from bags containing S. epidermidis. But by contrast all effluents without filtration showed positive cultures and endotoxin detection. CONCLUSION: This result showed that inline intravenous filters were useful device to remove bacteria, Candida, and bacterial endotoxin.
Administration, Intravenous ; Bacteria* ; Candida* ; Culture Media ; Filtration* ; Horseshoe Crabs ; Pseudomonas aeruginosa ; Staphylococcus epidermidis

PURPOSE: To evaluate myocardial conductivity to understand cardiac involvement in patients with mitochondrial disease. METHODS: We performed retrospective study on fifty-seven nonspecific mitochondrial encephalopathy patients with no clinical cardiac manifestations. The patients were diagnosed with mitochondrial respiratory chain complex defects through biochemical enzyme assays of muscle tissue. We performed standard 12-lead electrocardiography (ECG) on all patients. RESULTS: ECG abnormalities were observed in 30 patients (52.6%). Prolongation of the QTc interval (>440 ms) was seen in 19 patients (33.3%), widening of the corrected QRS interval in 15 (26.3%), and bundle branch block in four (7.0%). Atrioventricular block, premature atrial contraction and premature ventricular contraction were seen in two patients each (3.5%) and Wolff-Parkinson-White syndrome in one patient (1.8%). CONCLUSION: Given this finding, we recommend active screening with ECG in patients with mitochondrial disease even in patients without obvious cardiac manifestation.
Atrial Premature Complexes ; Atrioventricular Block ; Bundle-Branch Block ; Child ; Electrocardiography ; Electron Transport ; Enzyme Assays ; Heart ; Humans ; Mass Screening ; Mitochondria ; Mitochondrial Diseases ; Mitochondrial Encephalomyopathies ; Muscles ; Retrospective Studies ; Ventricular Premature Complexes ; Wolff-Parkinson-White Syndrome

BACKGROUND: Accurate quantitative testing of HBV DNA is very important for choosing antiviral treatment targets and evaluating treatment response in chronic HBV patients. We evaluated the performance of LG AdvanSure HBV Real-Time QPCR kit (LG) utilizing real-time quantitative PCR. METHODS: The LG kit was conducted for 201 chronic hepatitis patients undergoing treatment at the Korea University Ansan hospital and 48 normal control volunteers. The precision, limit of detection, sensitivity, and specificity of LG Kit were evaluated. Correlation analysis was done with Abbott Real Time HBV kit (Abbott) and the Cobas Amplicor HBV Monitor kit (Cobas) and the concordances rate of the three methods were calculated. RESULTS: The LG assay showed linear range of detection from 10(2) to 10(6) and coefficient of variation (CV) was 1.10~0.52% at > or =1,000 IU/mL and 1.19% at 100 IU/mL. The coefficient of determination for precision analysis was 0.997. The limit of detection for detection of 95% of positive samples was 9.71 IU/mL (54.4 copies/mL). In 201 clinical samples, the log HBV DNA/ml showed good correlation between Roche vs Abott, Roche vs LG and Abott vs LG, respectively (n=105, 108, 133, r2=0.91, 0.89, 0.94, P<0.0001). The overall concordance rate of three methods were 79.1% (159/201), 82.1% (165/201), and 85.1% (171/201), respectively, showing no stastically significant difference (P>0.05). CONCLUSION: LG AdvanSure HBV Real-Time QPCR kit showed outstanding precision, linearity, limit of detection, good correlation with previous methods, and is a valuable tool in treatment monitoring of chronic HBV infections.
DNA ; Hepatitis B ; Hepatitis, Chronic ; Humans ; Korea ; Limit of Detection ; Organothiophosphorus Compounds ; Sensitivity and Specificity

PURPOSE: To evaluate the clinical and electrophysiological characteristics of malformation of cortical development (MCD) with epilepsy. METHOD: We studied clinical and electroencephalographic (EEG) features of 54 childhood epilepsy patients with MCD diagnosed by magnetic resonance imaging (MRI) and pathologic examinations. RESULTS: 1) Bilateral diffuse MCD's were in 5 patients, bilateral focal MCD's were in 8, unilateral diffuse MCD's in 7, and unilateral focal MCD's were noted in 34 patients. 2) Partial seizures were manifested in 35 patients, and 4 of them evolve to infantile spasm (IS), isolated IS was noted in 15 cases, and generalized seizures were noticed in 2 cases 3) Asymmetric EEG backgraound slowing was noted in 30 patients, and 29 patients (96.7%) had MCD's in abnormal side of brain. 4) Polymorphic slowing was noted in 36 patients, and 28 cases (77.8%) had MCD's in those area. 5) Sensitivity of partial epileptiform discharges (ED's) for MCD was 79.6%, but specificity was 68.5%. 6) Localized paroxysmal fast activity was noted in 16 cases (29.6%), and specificity for MCD was 90.7%. 7) Spindle shaped fast activity was noted in 8 patients (14.8%), and its specificity was 100%. 8) Thirty-one cases (57.4%) were intractable to antiepileptic drugs (AED's). Seventeen cases of them were treated by ketogenic diet, and 12 patients (66.7%) were completely controlled. Among 12 cases of surgical resection, 11 patients (91.7%) became seizure free for 6 months to 2 years. 9) In pathologically confirmed cases, EEG sensitivity for MCD lesion was 100%, but sensitivity of MRI was 69.2%. CONCLUSION: EEG is most sensitive diagnostic tool for MCD in childhood epilepsy. and many of intractable epilepsy could be controlled by ketogenic diet and surgery.
Anticonvulsants ; Brain ; Electroencephalography ; Epilepsy* ; Humans ; Infant ; Infant, Newborn ; Ketogenic Diet ; Magnetic Resonance Imaging ; Seizures ; Sensitivity and Specificity ; Spasms, Infantile