Prosopometamorphopsia and Visual Field Defect Improved by Valproic acid in a CADASIL Patient

Persistent aura without infarction is defined as an aura persisting for 1 week or more without evidence of infarction on neuroimaging. It is difficult to differentiate persistent visual aura without infarction from occipital lobe epilepsy. We report a Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy patient with prosopometamorphopsia and visual field defect improved by valproic acid. We also review ambiguity between visual aura in migraine and occipital lobe epilepsy.

Gerstmann-Sträussler-Scheinker Disease (Pro102Leu) Presenting as Rapidly Progressive Dementia

Genetic prion diseases account for about 10-15% of all cases of human prion disease and are caused by mutations in the prion protein gene. Gerstmann-Sträussler-Scheinker (GSS) disease is a rare genetic prion disease, which is characterized by slowly progressive cerebellar ataxia and the occurrence of cognitive decline in the later stage. P102L is the most common mutation in GSS. We report a patient with a P102L mutation that initially manifested as rapidly progressive dementia without cerebellar symptoms.

Retrieval Rate and Accuracy of Ultrasound-Guided 14-G Semi-Automated Core Needle Biopsy of Breast Microcalcifications

OBJECTIVE: To evaluate the retrieval rate and accuracy of ultrasound (US)-guided 14-G semi-automated core needle biopsy (CNB) for microcalcifications in the breast. MATERIALS AND METHODS: US-guided 14-G semi-automated CNB procedures and specimen radiography were performed for 33 cases of suspicious microcalcifications apparent on sonography. The accuracy of 14-G semi-automated CNB and radiology-pathology concordance were analyzed and the microcalcification characteristics between groups with successful and failed retrieval were compared. RESULTS: Thirty lesions were successfully retrieved and the microcalcification retrieval rate was 90.9% (30/33). Thirty lesions were successfully retrieved. Twenty five were finally diagnosed as malignant (10 invasive ductal carcinoma, 15 ductal carcinoma in situ [DCIS]) and five as benign. After surgery and mammographic follow-up, the 25 malignant lesions comprised 12 invasive ductal carcinoma and 13 DCIS. Three lesions in the failed retrieval group (one DCIS and two benign) were finally diagnosed as two DCIS and one benign after surgery. The accuracy of 14-G semi-automated CNB was 90.9% (30/33) because of two DCIS underestimates and one false-negative diagnosis. The discordance rate was significantly higher in the failed retrieval group than in the successful retrieval group (66.7% vs. 6.7%; p < 0.05). Punctate calcifications were significantly more common in the failed retrieval group than in the successful retrieval group (66.7% vs. 3.7%; p < 0.05). CONCLUSION: US-guided 14-G semi-automated CNB could be a useful procedure for suspicious microcalcifications in the breast those are apparent on sonography.

Brain Abscess Developed on the Lesion Site of Previous Ischemic Stroke

Brain abscess following ischemic stroke is a very rare and dangerous condition that can have catastrophic results, and thus requires urgent and comprehensive evaluation. We report a case of a patient with brain abscess that developed at the lesion site of a previous cerebral infarction. The disruption of the blood-brain barrier in the infarcted lesion might facilitate abscess formation, making it a preferred target for infectious agents.

A Case of Oxcarbazepine Induced Beau's Line / 대한간질학회지

Beau's line is a grooved transverse line on the fingernail or toenail, and is considered as a kind of nail disorder, It is known to occur with temporary arrest of nail matrix proliferation. The causes of Beau's line comprise severe systemic illness as well as drug ingestion. A 51-year-old male patient had developed Beau's line on all his fingers after about three months of oxcarbazepine administration. These nail changes disappeared spontaneously with nail growth two months later. The authors report a case of oxcarbazepine induced Beau's line.

Direct Medical Costs of Type 2 Diabetic Patients in the Tertiary Hospital / 당뇨병

BACKGROUND: Type 2 diabetes mellitus is a common, chronic and costly disease. Its prevalence is rapidly increasing worldwide. Diabetes has big economic burden mainly because of its chronic complications. We analyzed the annual direct medical costs of type 2 diabetic patients, including the costs associated with its complications in Korea retrospectively. METHODS: We enrolled 531 type 2 diabetic patients who had been treated in the 3 Tertiary Hospital in 2005. Clinical characteristics, duration of diabetes, modality of glycemic control, and presence of microvascular and macrovascular complications were assessed by the review of medical records. The annual direct medical costs were assessed using the hospital electronic database and included insurance covered and uncovered medical costs. RESULTS: The annual direct medical costs of type 2 diabetic patients without any complications was 1,184,563 won (95% CI for mean: 973,006~1,396,121 won). Compared to diabetic patients without complications, annual total medical costs increased 4.7-fold, 10.7-fold, and 8.8-fold in patients with microvascular complications, macrovascular complications and both complications, respectively. Hospitalization costs largely increased by 78.7-fold and 61.0-fold in patients with macrovascular complications and both complications, respectively. Major complications to increase medical costs were kidney transplantation (23.1-fold), dialysis (21.0-fold), PTCA or CABG (12.4-fold), and leg amputation (11.8-fold). The total medical costs dramatically increased according to the stage of diabetic retinopathy and nephropathy. CONCLUSION: Diabetic complications have a substantial impact on the direct medical costs of type 2 diabetic patients. The prevention of diabetic complications will benefit the patients as well as the overall healthcare expenditures.

Clinical Characteristics and Direct Medical Costs of Type 2 Diabetic Patients / 당뇨병

BACKGROUND: Type 2 diabetes mellitus is an expensive chronic metabolic disorder and its prevalence has been increasing rapidly in South Korea, owing to a westernized lifestyle. We analyzed the annual direct medical costs attributable to type 2 diabetes and its chronic complications in Korea retrospectively. METHODS: We randomly selected 1,051 patients with type 2 diabetes who visited Ajou University Hospital as an outpatient in 2005. Clinical characteristics, duration of diabetes, and microvascular and macrovascular complications were assessed from a medical chart review. The annual direct medical costs included insurance covered and uncovered medical costs. RESULTS: Of the 1,051 patients with type 2 diabetes, 48.2% had at least one microvascular complication, 5.6% had at least one macrovascular complication, and 12.4% of the patients had both microvascular and macrovascular complications. The average annual direct medical cost was found to be 3,348,488won per patient. In patients with microvascular complications, the total cost of management was increased 1.4 times compared to those without complications. Direct medical costs for patients with macrovascular complications were 2.1-fold as high as patients with no complications. Those patients with both microvascular and macrovascular complications, increased costs by 3.1-fold over those without complications. CONCLUSION: Chronic complications have a substantial impact on the direct medical costs of type 2 diabetes. The prevention of chronic diabetic complications will not only influence the mortality and morbidity of patients with type 2 diabetes, but also potentially reduce medical costs.

Relationship between Childhood and Adolescent Obesity and Remnant Lipoprotein / 대한내분비학회지

BACKGROUND: Remnant lipoproteins are the lipolytic degradation product of the triglyceride-rich lipoproteins produced by the liver (very-low-density lipoprotein cholesterol) and intestine (chylomicrons). Recent studies have demonstrated a correlation between remnant lipoproteins and cardiovascular risk. Our study assessed the relationship between obesity and remnant lipoproteins and evaluated the factors related to remnant lipoprotein in children and adolescents. METHODS: Body mass index (BMI), waist circumference, systolic and diastolic blood pressures, body fat mass, total abdominal fat, visceral and subcutaneous fat areas, total cholesterol, triglyceride (TG), LDL cholesterol (LDL-C), HDL cholesterol (HDL-C) and remnant lipoprotein cholesterol (RLP-C) were measured in 135 children and adolescents (67 boys and 68 girls). Plasma RLP fractions were isolated using an immunoaffinity gel containing specific anti-apoB-100 and anti-apoA-I antibodies. The subjects were divided into three groups: the low ( or = 85 percentile) BMI groups. RESULTS: RLP-C was significantly correlated with age, sex, BMI, waist circumference, systolic and diastolic blood pressures, visceral and subcutaneous fat areas, visceral fat area to subcutaneous fat area ratio (VSR), total cholesterol, TG, HDL-C, apoB, and HOMA-IR. From a multivariate regression analysis, TG (beta = 0.928, P < 0.001) was found to be independently correlated with RLP-C. After excluding TG as an independent variable, a multivariate regression analysis revealed that the HOMA-IR (beta=0.231, P=0.007) and systolic blood pressure (beta=0.169, P=0.046) were independently associated with RLP-C. CONCLUSION: RLP-C was significantly higher in obese children and adolescents. TG, systolic blood pressure, and insulin resistance were related to remnant lipoproteins.

Relationship between Childhood and Adolescent Obesity and Remnant Lipoprotein / 대한내분비학회지

BACKGROUND: Remnant lipoproteins are the lipolytic degradation product of the triglyceride-rich lipoproteins produced by the liver (very-low-density lipoprotein cholesterol) and intestine (chylomicrons). Recent studies have demonstrated a correlation between remnant lipoproteins and cardiovascular risk. Our study assessed the relationship between obesity and remnant lipoproteins and evaluated the factors related to remnant lipoprotein in children and adolescents. METHODS: Body mass index (BMI), waist circumference, systolic and diastolic blood pressures, body fat mass, total abdominal fat, visceral and subcutaneous fat areas, total cholesterol, triglyceride (TG), LDL cholesterol (LDL-C), HDL cholesterol (HDL-C) and remnant lipoprotein cholesterol (RLP-C) were measured in 135 children and adolescents (67 boys and 68 girls). Plasma RLP fractions were isolated using an immunoaffinity gel containing specific anti-apoB-100 and anti-apoA-I antibodies. The subjects were divided into three groups: the low ( or = 85 percentile) BMI groups. RESULTS: RLP-C was significantly correlated with age, sex, BMI, waist circumference, systolic and diastolic blood pressures, visceral and subcutaneous fat areas, visceral fat area to subcutaneous fat area ratio (VSR), total cholesterol, TG, HDL-C, apoB, and HOMA-IR. From a multivariate regression analysis, TG (beta = 0.928, P < 0.001) was found to be independently correlated with RLP-C. After excluding TG as an independent variable, a multivariate regression analysis revealed that the HOMA-IR (beta=0.231, P=0.007) and systolic blood pressure (beta=0.169, P=0.046) were independently associated with RLP-C. CONCLUSION: RLP-C was significantly higher in obese children and adolescents. TG, systolic blood pressure, and insulin resistance were related to remnant lipoproteins.

A Case of Type I Duodenal Perforation Treated with Covered Metal Stent / 대한소화기내시경학회지

Endoscopic retrograde cholangiopancreatography (ERCP) has played an important role in diagnosis and management of biliary disease. Traditionally, duodenal perforations, rare complication of ERCP, have been managed surgically. However, in the past decade, there were arguments for surgical and nonsurgical management of ERCP-related duodenal perforations and reports of successful conservative treatment were increasing especially in patients with old age and poor medical conditions. We experienced a case of type I duodenal perforation following ERCP treated with covered metal stent in 74 years old female with duodenal invasion by Klatskin tumor.

A Case of Familial Medullary Thyroid Carcinoma with a E768D Mutation in RET Proto-Oncogene / 대한내분비학회지

A medullary thyroid carcinoma, a neoplasm of parafollicular C cell origin, occurs as a sporadic or hereditary disease. A hereditary medullary thyroid carcinoma is an autosomal dominantly inherited disease, which is composed of multiple endocrine neoplasia 2A and 2B, with a familial medullary thyroid carcinoma. Germline mutations of the RET gene are the underlying cause of the majority of hereditary medullary carcinomas. Here, the case of a 42 years-old man with a familial medullary thyroid carcinoma, confirmed by the detection of a RET proto-oncogene mutation at exon 13 on codon 768 from a GAG(Glu) to a GAT(Asp), is described. The patient underwent a total thyroidectomy and modified radical neck dissection. His sister was found to have the same mutant gene.

A Case Report of von Hippel-Lindau Disease Manifested in a Monozygous Twin / 대한내분비학회지

Von Hippel-Lindau (VHL) disease is an autosomal dominant disease, which forms hypervascular tumors in multiple organs, such as hemangioblastomas in the retina and central nervous system, renal cell carcinomas, pheochromocytomas and cysts in various organs. Recent advances in gene testing have made it possible to screen family members for VHL disease. We experienced a 28 year-old male, who was diagnosed with bilateral pheochromocytomas through a family screening test when his elder monozygous twin brother was diagnosed with a pheochromocytoma. He received no treatment until December, 2004, when he visited the Emergency room due to a headache. A hemangioma of the cerebellum was seen in the brain MR study, leading to the diagnosis of type 2A VHL disease. An abdominal CT scan revealed no lesions of the pancreas or kidney. There was no evidence of a hemangioma in the retinal scan. The subsequent gene testing showed a germline mutation in exon 3 codon 167 of the VHL gene. The mother of the patient was revealed to have the same mutation of the VHL gene, but the elder brother of the patient did not.

A Case of IgA Nephropathy Associated with LV Thrombus and Cerebral Infarction / 대한신장학회잡지

Thrombosis is one of the important complications in nephrotic syndrome and related to the hypercoagulable state of the nephrotic syndrome, although the exact mechanism is under the debate. While venous trombosis is frequently encountered, arterial thrombosis is rare. Renal vein thrombosis occurs frequently in the patients with nephrotic syndrome due to membranous glomerulonephritis (MGN), membranoproliferative glomerulonephritis (MPGN). It, however, rarely happens in IgA nephropathy. Intracardiac thrombus in the nephrotic syndrome have been described few in the earlier literatures. We reported a case of IgA nephropathy with LV thrombus and cerebral infarction that has been treated successfully with thrombolytic theraphy. So far, the patient on the outpatient clinics have reported no specific complaints.

A Case of Primary Pulmonary Hypertension Diagnosed in Puerperium

Primary pulmonary hypertension is a condition characterized by sustained elevations of pulmonary artery pressure without demonstrable cause. It is incurable and progressive clinical entity. The incidence has been estimated at approximately 1 to 2 cases per million people in the general population. The maternal mortality of primary pulonary hypertension in pregnancy was said to be 30% and the first month after delivery represents the period of highest risk. We experienced a 27-year-old women with primary pulmonary hypertension diagnosed in postpartum period. She was transferred from the department of obstetrics to internal medicine due to dyspnea on the first day of postpartum. She was diagnosed by perfusion lung scan, chest CT and confirmed by echocardiography. This diagnosis were supported by findings of pulmonary angiogram and cardiac catheterization. Initially she was treated with diuretics, aspirin and anticoagulant therapy, and as a result of the treatment, her symptoms improved. However after 8months has elapsed, exertional dyspnea was worsening. Thus she was treated with calcium channel blocker, aspirin and diuretics and was followed at 1 month interval on the outpatient clinics.

Clinical usefulness of Clinical Pulmonary Infection Score of ICU Patients with Sputum Culture positive for Multi-drug resistant Acinetobacter baumannii / 결핵

BACKGROUND: The hospital-acquired pneumonia is the most common nosocomial infection. Recently, the Acinetobacter baummannii infections are rapidly increasing, especially the frequency of Multi-drug resistant A. baumannii. Therefore we assessed clinical features and prognosis of patients in the ICU with Multi-drug resistant A. baumannii from the sputum culture using the Clinical Pulmonary Infection Score(CPIS). METHODS: The medical records of 43 patients with Multi-drug resistant A. baumannii from sputum culture who were suspected had clinically pneumonia and admitted to the ICU from January 2000 to July 2002 were retrospectively analyzed. RESULT: 19 patients were CPIS greater than 6 and 24 patients were CPIS less than or equal to 6. Mean age for the former was 71+/-11 years old, and the latter was 61+/-19 years old. The mean APACHE II score on admission and on sputum study was not different between two groups(17.4+/-5.7 vs 18.5+/-6.1, p=0.553, 20+/-6 vs 17+/-8, p=0.078). But the mortality rate was 73.7% for the former, and 16.7% for the latter(p<0.001). CONCLUSION: In ICU patients who had clinically suspected pneumonia with sputum culture positive for Multi-drug resistant A. baumannii, the mortality was significantly higher if CPIS was greater than 6.

A Serial Echocardiograhic Obesrvation of Acute Myocardial Injury Associated with Pheoechromocytoma Crisis

Pheochromocytoma may cause anatomic and functional cardiac abnormalities. Various echocardiographic abnormalities have been observed, including systolic anterior movement, global hypokinesis as well as hypokinesis of the base and the apex of LV. There are no previous reports of serial echocardiographic finding in patients with pheochromocytoma. We experienced a 38-year-old woman with pheochromocytoma who suffered from acute myocardial injury. Echocardiography revealed that the acute myocardial injury started in the base in the early phase and subsequently extended to the mid-portion of left ventricule. The injury persisted longer in the base than the mid-portion. than 1.5 times the adjacent normal coronary artery.