Sustained downgaze mostly occurs in association with lesions affecting the dorsal midbrain. We report sustained downgaze in a patient with hepatic encephalopathy. The sustained downgaze existed for seven more days after she regained her consciousness. The persistent downgaze even after regaining full consciousness indicates localized pretectal dysfunction rather than diffuse encephalopathy as the mechanism of sustained downgaze in our patient. The ocular motor dysfunction in hepatic encephalopathy may be due to localized dysfunction of the brainstem

We describe a case of the juvenile form of Pompe's disease that presented as primary alveolar hypoventilation due to respiratory muscle involvement. This 17-year-old girl had been asymptomatic until this admission, although she had a delayed puberty. Arterial blood gas analysis, pulmonary function test as well as physical findings were compatible with chronic alveolar hypoventilation syndrome. Since she had lower extremity muscle weakness and pseudomyotonic discharge on electromyography a muscle biopsy was done, which revealed glycogen storage disease. The patient was managed successfully with nasal intermittent positive pressure ventilation.
Adolescent ; Chronic Disease ; Female ; Glycogen Storage Disease Type II/*complications/pathology ; Humans ; Hypoventilation/*etiology/therapy ; Intermittent Positive-Pressure Ventilation ; Muscles/pathology ; Pulmonary Alveoli

Human cytomegalovirus (HCMV) isolated from Korean patients is different in the antigenic and genomic structure of gB from the laboratory-adapted strain. To dissect the reactivity to HCMV glycoprotein B (gB) domains, each domain gene of gB of HCMV SNUCH1, Korean isolate, was amplified from the extracted DNA of the virus-infected fibroblasts with the specific primers by polymerase chain reaction (PCR). Amplified DNA was cloned into pcDNA3. Immunofluorescent staining and western blot analysis revealed that the expressed gB in mammalian cells was immunoreactive and equivalent to the naturally expressed gB in virus-infected fibroblasts. The antigenic component reactive with monoclonal antibodies, MCMVA 57, 88, and 98 appeared at the D3 domain of gB molecule, and that with MCMVA 66 and 135 at the D2b domain. Antibody titer was measured with HCMV-infected fibroblasts and the domains of gB expressed in mammalian cells. There was no correlation between the antibody titer to the whole HCMV and neutralizing antibody titer, and between the antibody titer to whole HCMV and whole gB. It was more reasonable to use whole gB than whole HCMV in the comparison with the neutralizing antibody titer. D3 was representative domain in gB molecule in the anti-gB reactivity. Conclusively it is highly recommendable to use the representing isolates in Korea and its domains for the detection of antibody or the analysis of antigen in the aspect of immunological properties and molecular structures.
Antibodies, Monoclonal ; Antibodies, Neutralizing* ; Blotting, Western ; Clone Cells ; Cytomegalovirus* ; DNA ; Epitopes* ; Fibroblasts ; Glycoproteins ; Humans* ; Korea* ; Molecular Structure ; Polymerase Chain Reaction

No abstract available.

BACKGROUND: The growing number of spinal cord injured individuals facing old age makes the understanding of age related limitations increasingly important. This study was to investigate the age-related differences in rehabilitation outcome according to the level of cervical spinal cord injury and each category of activity of daily living(ADL). METHODS: Subjects were 79 adults with tetraplegia all of whom were admitted in National Rehabilitation Hospital. The level of injury was classified into upper cervical(C4, C5), mid-cervical(C6), and lower cervical(C7, C8) spinal cord injury. Eight ADL categories including feeding, grooming, bathing, dressing, toileting, bed-transfer, toilet/tub transfer, and indoor mobility were assessed using the scale of Spinal Cord Independence Measure. Age related differences were examined by separating the sample into two age groups(> or =40 and <40 years) RESULTS: There was no age related difference in rehabilitation outcome in upper cervical cord injury patients. In mid-cervical cord injury level, ADL capacities differed only in the feeding and grooming activities. In lower cervical cord injury level, older patients showed lower rehabilitation outcome than younger counterparts in all ADL categories examined. CONCLUSION: Along with injury level, age should be considered when formulating rehabilitation plans and functional prognostic statements in tetraplegic patients.
Activities of Daily Living ; Adult ; Animals ; Bandages ; Baths ; Grooming ; Humans ; Quadriplegia ; Rehabilitation* ; Spinal Cord Injuries* ; Spinal Cord* ; Treatment Outcome*

Radiofrequency ablation (RFA) is a new, minimally invasive modality that serves as an alternative to surgery in patients with thyroid tumors. The Task Force Committee of the Korean Society of Thyroid Radiology developed recommendations for the optimal use of RFA for thyroid tumors in 2012 and revised them in 2017. Herein, we review and summarize the 2017 thyroid RFA guideline and compare it with the 2012 thyroid RFA guideline.
Advisory Committees ; Catheter Ablation ; Humans ; Thyroid Gland ; Thyroid Neoplasms ; Thyroid Nodule ; Ultrasonography

Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis, hyperreninemia, hyperaldosteronism, normal blood pressure, and other clinical symptoms. As a clinical and genetical heterogeneous disorder, this syndrome can be classified into two clinical variants, antenatal Bartter syndrome and classic Bartter syndrome according to the onset age. Nephrocalcinosis is common in antenatal Bartter syndrome, but is rare in classic Bartter syndrome. It can also be classified into five genetic subtypes by the underlying mutant gene, all of which are expressed in the tubular epithelial cells of the thick ascending limb of the loop of Henle. Patients with Bartter syndrome type 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. We have experienced a case of Bartter syndrome with nephrocalcinosis in a 42-year-old woman diagnosed by biochemical and radiologic studies. We had successful response with potassium chloride and spironolactone.
Adult* ; Age of Onset ; Alkalosis ; Bartter Syndrome* ; Blood Pressure ; Epithelial Cells ; Extremities ; Female ; Humans ; Hyperaldosteronism ; Hypokalemia ; Loop of Henle ; Nephrocalcinosis* ; Potassium Chloride ; Spironolactone

BACKGROUND: Dobutamine echocardiography is a useful method to detect myocardial viability in ischemic heart disease. Recently myocardial contrast echocardiography(MCE) is reported to be a new method to evaluate myocardial viability by assessing microvascular integrity of dysfunctional myocardium. We hypothesized if the microvascular integrity is maintained, the dysfunctional myocardium would improve its function by dobutamine infusion. METHOD: 10 myocardial infarction patients (acute : old=8 : 2, M : F=7 : 3, mean age=61+/-11yr) were included in the study. 2 dimensional echocardiography was performed before and during dobutamine infusion and after contrast injection to right and left coronary arteries in the catheterization laboratory. Echocardiographic analysis was done in parasternal short, apical 4 and 2 chamber views. Left ventricule was devided by 20 segments from 3 views. In each segment, will motion score(graded 1, normal, to 5, dyskinesia) before and after dobutamine infusion and opacification grade(0, 0.5, 1 denoting no, intermediate and normal opacification respectively) was compared. RESULTS: The number of segments with abnormal wall motion at baseline were 57 segments. 5 segments was exciuded due to poor image quality. Among 52 segments, 25 segments improved it's function during dobutamine infusion. Improvement of regional function was more frequent in hypokinetic segments than akinetic or dyskinetic segments (69% vs 15%). The improvement of dysfunctional regional wall motion by dobutamine infusion was observed in 80%(19/24), 67%(6/9) and 5%(1/19) of normally, intermediately and none opacified segment respectively. The correlation between wall motion score with opacification grade was 0.598 at baseline and increased to 0.766 after dobutamine infusion. CONCLUSION: In patients with myocardial infarction the dysfunctional segments but intact microvasculature assessed myocardial contrast echocardiography improves function by dobutamine infusion. These findings myocardial contrast echocardiography would be a useful method to detect myocardial viability.
Catheterization ; Catheters ; Coronary Vessels ; Dobutamine* ; Echocardiography* ; Humans ; Microvessels ; Myocardial Infarction* ; Myocardial Ischemia ; Myocardium

PURPOSE: To manage a critically ill patient effectively, an emergency physician must make an accurate assessment of the patient's hemodynamic status. Although the hemodynamic status can be accurately measured through the use of a pulmonary artery catheter, this device has significant limitations in the emergency department. Instead, the most commonly used method of measuring cardiac output (CO) in the emergency department is transthoracic echocardiography (TTE). However, TTE has been reported to underestimate the CO. Impedance cardiography (ICG) is an established and accurate technique for noninvasive determination of hemodynamic parameters. Thus, this study aimed to assess the agreement between CO measurements made by using TTE and ICG. METHODS: In 20 healthy volunteers, the hemodynamic parameters (stroke volume and CO) obtained by TTE were compared with the parameters derived from simultaneous measurements by ICG. RESULTS: The mean stroke volumes and COs were 63.9+/-11.6 ml and 4.1+/-0.7 L/min, respectively, by TTE, and 77.2+/-10.2 ml and 5.0+/-0.6 L/min by ICG. The correlation coefficients for stroke volume and CO between TTE and ICG were 0.77 and 0.64. The mean difference in stroke volume between TTE and ICG was -13.3+/-6.4 ml (-19.5+/-10.5 %), and the mean difference in CO was -0.88+/-0.48 L/min (-20.1+/-11.6 %). CONCLUSION: In healthy volunteers, the comparison between TTE and ICG showed a good correlation for stroke volume and CO. However, the mean differences in stroke volume and CO between TTE and ICG were -19.5+/-10.5 % and -20.1+/-11.6 %, respectively.
Cardiac Output* ; Cardiography, Impedance ; Catheters ; Critical Illness ; Echocardiography* ; Emergencies ; Emergency Service, Hospital ; Healthy Volunteers ; Hemodynamics ; Humans ; Male* ; Pulmonary Artery ; Stroke Volume ; Volunteers*

Anti-glomerular basement membrane antibody mediated rapidly progressive glomerulonephritis is a rare autoimmune disease. It is characterized by acuterenal failure and crescentic glomeruli with linear immune deposits along glomerular basement membrane mediated by anti-GBM antibodies. We report a case of a sixty-years-old man with generalized edema and hematuria. On admission, BUN/Creatinine was 118/19.6 mg/dL, Hb was 10.2 g/dL. On urinalysis, protein was 3+, and many RBCs were found. Renal biopsy specimen which contained 8 glomeruli showed active cellular crescent formation in all glomeruli. On immunofluorescent staining specimen, there were 4 glomeruli which showed strong IgG linear staining along the glomerular basement membrane and mild C3 & C1q deposit along the capillary walls. The titer of anti-GBM antibody was 123 EU by ELISA (normal: <10 EU). We treated with high dose of corticosteroid and plasmapheresis, but renal function was not recovered even after 3 months of hemodialysis.
Antibodies ; Autoimmune Diseases ; Basement Membrane* ; Biopsy ; Capillaries ; Edema ; Enzyme-Linked Immunosorbent Assay ; Glomerular Basement Membrane ; Glomerulonephritis ; Hematuria ; Hemorrhage* ; Immunoglobulin G ; Plasmapheresis ; Renal Dialysis ; Urinalysis