Angioedma is a group of disorders with multifactorial etiology but a similar clinical expression, is characterized by swelling of subcutaneous or submucosal tissue. Systemic lupus erythematosus(SLE) is a chronic multi-systemtic disease characterized by inflammation and tissue damage resulting from deposition of auto-antibodies and immune complex. We experienced a case of angioedema which was the first manifestation of SLE in 24-year-old female patient. She had suffered from severe facial edema and multiple lymphadenopathy for six months and she also had pleural effusion, positive anti-nuclear and anti-DNA antibody test. Marked decrease of C3 and C4 levels was noted with normal antigenic level, and activity of Cl esterase inhibitor. The angioedema was not improved with anti-hitamine agents and instead disappeared with use of corticosteroid and non-steroidal anti-inflammatory drugs. Complement levels normalized after corticosteroid treament. We report a case of SLE which initial manifestation was angioedema.
Angioedema ; Antigen-Antibody Complex ; Complement System Proteins ; Edema ; Female ; Humans ; Inflammation ; Lupus Erythematosus, Systemic* ; Lymphatic Diseases ; Pleural Effusion ; Young Adult

Temporal bone fracture is usually the result of blunt head injury as ossicular disruption or isolated dislocation of individual ossicles may occur after temporal bone trauma. The incus is particularly prone to displacement because of its fragile ligamentous support. We report a rare case of incus dislocation into the external auditory canal associated with an underlying temporal bone fracture.
Craniocerebral Trauma* ; Dislocations* ; Ear Canal* ; Head Injuries, Closed ; Head* ; Incus* ; Ligaments ; Temporal Bone

We present three neuropathologically-verified and two clinically-probable cases of Creutfeldt Jakob disease. All five had nonspecific prodromal complaints or symptoms prior to overt neurological signs and showed striking progressive neurologic deterioration, especially cognitive decline and cerebellar dysfunction. Myoclonic involuntary movements and complete decapitated states followed in one or two months. The characteristic even pathognomonic in proper clinical settings, features of electroencephalography, magnetic resonance imaging and positron emission tomography and pathologic findings are presented.
Cerebellar Diseases ; Creutzfeldt-Jakob Syndrome* ; Dyskinesias ; Electroencephalography ; Magnetic Resonance Imaging ; Positron-Emission Tomography ; Strikes, Employee

No abstract available.
Magnetic Resonance Imaging ; Pulmonary Artery ; Pulmonary Embolism* ; Radionuclide Imaging

An infraoptic anterior cerebral artery (ACA) arising at a low bifurcation of the internal carotid artery is a rare anomaly, of which about 33 cases have been reported to date, often in association with cerebral aneurysms. We describe a case involving an infraoptic ACA in which a ruptured middle cerebral artery aneurysm was also present. Angiography revealed the presence of an abnormal solitary ACA, arising from the intracranial proximal internal carotid artery near the origin of the ophthalmic artery, and a contralateral middle cerebral artery aneurysm. Magnetic resonance imaging showed that the ACA passed below the ipsilateral optic nerve, anterior to the optic chiasm, to join the normally positioned anterior communicating artery above the optic chiasm.
Angiography ; Anterior Cerebral Artery* ; Arteries ; Carotid Artery, Internal ; Intracranial Aneurysm ; Magnetic Resonance Imaging ; Ophthalmic Artery ; Optic Chiasm ; Optic Nerve

Medullary thyroid carcinoma accounts for 3% of all thyroid gland malignancies. It commonly metastasizes to liver, lung, and bone. It rarely metastasizes to skin, and only a few such cases have been documented. Cutaneous metastasis suggests a poor prognosis, with a mean survival of 7.5-19 months. The most effective treatment for skin metastasis is complete surgical removal of all local and regional lesions. The response to systemic chemotherapy is typically poor. We report a case of medullary thyroid carcinoma with cutaneous metastases, which responded to chemotherapy.
Liver ; Lung ; Neoplasm Metastasis ; Prognosis ; Skin ; Thyroid Gland ; Thyroid Neoplasms

Primary pulmonary diffuse alveolar amyloidosis is a rare form among the pulmonary amyloidosis. Especially in primary form diffuse alveolar type is much more rare disease. the patient was checked simple chest X-ray for the purpose of routine screening test, he had no respiratory symptoms and signs and he had no complaint of other organ symptoms, while in chest roentgenogram it was found bilateral diffuse interstitial infiltration of the lung, but any other organs which commonly involved in amyloidosis were not involved in laboratory and other specific diagnostic procedures. We report a case of pulmonry diffuse alveolar amyloidosis only localized in lung which was confirmed by transbronchial lung biopsy.
Amyloidosis* ; Biopsy ; Humans ; Lung* ; Mass Screening ; Rare Diseases ; Thorax

Twenty-one myasthenic patients were treated with high-dose daily prednisone regimen at Seoul National University Hospital from May 1983 to January 1985. Observations in relation to dosage, drug schedule, clinical responses, and sideeffects led us to following conclusions concerning the management of myasthenia gravis with steroid. In addition, factors influencing the result were considered. 1) Among 21 patients, twenty(95%) showed clinical improvement of variable degrees. 2) Significant improvement could be expected especially in male patients, in older patients, and in those with duration of myasthenia gravis less than 6 months prior to treatment. Performance of thymectomy or thymothymectomy, thymic pathology, and clinical grade at the time of therapy were not considered to affect the outcome. 3) Ten cases (48%) suffered from initial exacerbations, most of which occurred within the first four days of treatment. Steroid-induced crises developed in four cases with preexisting brittle or severe generalized myasthenia. 4) Period taken to show the initial response and the maximum improvement varied widely but majority of them occurred within 15 days and 70 days respectively. 5) Off-day weakness during the alternate-day schedule required special cautions and, if persitent, immediate return to daily schedule was desirable. 6) Thymectomy prior to steroid treatment was not always necessary but, when both regimens were scheduled together, preparation therapy with steroid thought to be more favorable. 7) Aside from initial exacerbations side-effects during the treatment were not remarkable, all of which disappeared with reduction of dosage.
Appointments and Schedules ; Humans ; Male ; Myasthenia Gravis* ; Pathology ; Prednisone ; Seoul ; Thymectomy

To identify the long-term survival rate and prognostic factors of AMI in Korea, total 404 patients who presented between Jan 1984 and mar 1989 at Seoul National University Hospotal were followed for and average of 24.9+/-18.2 months(range 1 to 69 months). 50 patients(12.4%) died during the in-hospital period and 25 patients(6.2%) died after discharge. Among the survivors reinfarction developled in 11 patients(3.3%). Overall survival rates were 0.87, 0.85, 0.83, 0.81, 0.79, 0.77 and event-free survival rates were 0.87, 0.84, 0.83, 0.79, 0.77, 0.72 at 1, 6, 12, 24, 36, 48 months respectively. During the in-hospital period sex, age, peak creatine kinase level, Killip class, Q wave in ECG, heart failure, and AV block in anterior infarction were of prognostic value. After discharge age, exercise duration on pre-discharge treadmill test, cardiac index, ejection fraction, and presence of heart failure were significant prognostic factors. Pre-discharge coronary angiographies were performed in 217 cases. There was no statistically significant difference in survival rate between multiple vessel disease and single vessel disease. But the more the number of involved vessels was, the higher the incidence of reinfarction was. In the group with jeopardy score less than 8, event-free survival rate was signigicantly higher. Overall survival rate was higher and reinfarction rate was lower in the group, but both were not statistically significant. On discriminant analysis of in-hospital prognostic factors, Killip class, heart failure and age were independent prognostic factors, but other factors had no additional prognostic value.
Atrioventricular Block ; Coronary Angiography ; Creatine Kinase ; Disease-Free Survival ; Electrocardiography ; Exercise Test ; Heart Failure ; Humans ; Incidence ; Infarction ; Korea ; Myocardial Infarction* ; Seoul ; Survival Rate* ; Survivors

Malignant glioma is the most frequent type in brain tumors. The prognosis of this tumor has not been significantly improved for the past decades and the average survival of patients is less than one year. Thus, an effective novel therapy is urgently needed. TNF-related apoptosis inducing ligand (TRAIL), known to have tumor cell-specific killing activity, has been investigated as a novel therapeutic for cancers. We have developed Ad-stTRAIL, an adenovirus delivering secretable trimeric TRAIL for gene therapy and demonstrated the potential to treat malignant gliomas. Currently, this Ad-stTRAIL gene therapy is under phase I clinical trial for malignant gliomas. Here, we report preclinical studies for Ad-stTRAIL carried out using rats. We delivered Ad-stTRAIL intracranially and determined its pharmacokinetics and biodistribution. Most Ad-stTRAIL remained in the delivered site and the relatively low number of viral genomes was detected in the opposite site of brain and cerebrospinal fluid. Similarly, only small portion of the viral particles injected was found in the blood plasma and major organs and tissues, probably due to the brain-blood barrier. Multiple administrations did not lead to accumulation of Ad-stTRAIL at the injection site and organs. Repeated delivery of Ad-stTRAIL did not show any serious side effects. Our data indicate that intracranially delivered Ad-stTRAIL is a safe approach, demonstrating the potential as a novel therapy for treating gliomas.
Adenoviridae/genetics ; Animals ; Blood-Brain Barrier ; Brain/drug effects/*metabolism/pathology ; Brain Neoplasms/genetics/metabolism/pathology/*therapy ; Clinical Trials, Phase I as Topic ; DNA, Viral/metabolism ; Disease Models, Animal ; Drug Delivery Systems ; Drug Evaluation, Preclinical ; *Gene Therapy ; Glioma/genetics/metabolism/pathology/*therapy ; Humans ; Liver/drug effects/metabolism/pathology ; Protein Multimerization/genetics ; Rats ; Spleen/drug effects/metabolism/pathology ; TNF-Related Apoptosis-Inducing Ligand/genetics/*pharmacokinetics