The extraaxial presentation of medulloblastoma is a rare phenomenon. This article describes the case of 19-year old woman who presented with mild headache and nausea and was diagnosed with medulloblastoma. The tumor arose from the right cerebellar cortex, and it was misdiagnosed meningioma on the basis of radiological examination. We review the literature and discuss the such atypical presentation of medulloblastoma.
Cerebellar Cortex ; Female ; Headache ; Humans ; Medulloblastoma* ; Meningioma ; Nausea ; Young Adult

PURPOSE: Periventricular leukomalacia(PVL) is the most important cause of cerebral palsy in premature infants. However, there are relatively few studies demonstrating the correlation between ultrasound findings and neurologic outcomes of low-birth-weight infants. To clarify the situation, we analyzed ultrasound findings and neurologic outcomes of many infants with periventricular leukomalacia. METHODS: Our study includes 36 infants with PVL, born in Chungnam National University Hospital, from October 1998 to December 2001. 25 infants with bilateral PVL were compared with 11 infants with asymmetric PVL. For a period of 12 to 48 months, the children were evaluated with neurologic assessments. RESULTS: In infants with bilateral lesions, 88%(22/25) of them had evidence of cerebral palsy. 16 children had spastic quadriplegia and others had spastic diplegia. 7 children with unilateral lesions were free of motor sequele at follow up. Other neurologic handicaps(mental retardation, opthalmologic abnormality or epilepsy) were not related with the symmetry or sizes of the lesions. CONCLUSION: In this study, unfavorable neurologic outcomes of bilateral PVL are confirmed. Therefore, every effort should be made to prevent the development of periventricular leukomalacia as well as not to miss the diagnosis. In addition, if there are any small lesions, regular neurologic assessments and early start of rehabilitation programs should be done.
Cerebral Palsy ; Child ; Chungcheongnam-do ; Diagnosis ; Follow-Up Studies ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Leukomalacia, Periventricular* ; Quadriplegia ; Rehabilitation ; Ultrasonography

PURPOSE: This study was performed to find the clinical significance in protein C as a differential marker in the beginning stage of infection and prognosis factor in severe infection among pediatric patients who were admitted due to fever. METHODS: A total of 40 pediatric patients who had temperatures higher than 37.5degrees C on admission at the Department of Pediatrics, Chungnam National University between December, 2002 and August, 2003 were enrolled. Total white blood cell count(WBC), erythrocyte sedimentation rate(ESR), C-reactive protein(CRP) and protein C were performed for those patients on admission. Clinical progress, diagnosis and prognosis were reviewed for these patients. The 40 patients were divided into two groups based on the diagnosis of bacterial and nonbacterial infections. RESULTS: Twenty patients(50%) were suspected of bacterial infections that showed positive results in blood, sputum, urine, and spinal cord fluid. There were eight cases with bacterial pneumonia, five with urinary tract infection, four with bacterial meningitis, two with cellulitis, and one with typhoid fever. The remaining 20 patients were diagnosed with nonbacterial infections because they had negative results in blood cultures. ESR and CRP were increased beyond normal range in both groups. However, protein C was significantly decreased in the bacterial infection group and yet normal range in the nonbacterial infection group(P<0.05). CONCLUSION: Protein C can be used as a differential marker in order to distinguish between bacterial and nonbacterial infections. In addition, protein C can possibly be used as a prognostic factor that can predict severe infection.
Bacterial Infections* ; Blood Sedimentation ; Cellulitis ; Chungcheongnam-do ; Diagnosis ; Fever* ; Humans ; Leukocytes ; Meningitis, Bacterial ; Pediatrics ; Pneumonia, Bacterial ; Prognosis ; Protein C* ; Reference Values ; Spinal Cord ; Sputum ; Typhoid Fever ; Urinary Tract Infections

BACKGROUND AND OBJECTIVES: The purpose of this study was to investigate the familial correlation and heritability of the LDL cholesterol level through a segregation analysis. SUBJECTS AND METHODS: This study was based on the data from 9,884 Korean individuals, ascertained as pedigrees, who had participated in the 1998 and 2001 Korean National Health & Nutrition Examination Survey. The subjects of the segregation analysis were 3,613 Korean with more than five family members. RESULTS: The LDL cholesterol level revealed a strong familial correlation among spouses, parental-offspring and siblings; a low correlation with spouses, but a high correlation with parental-offspring and siblings. The heritability of the LDL cholesterol level was 37.3%, when controlled for age, age2, gender, age x gender, waist circumference, smoking, alcohol drinking, exercise and education. After the segregation analysis, models of the major gene effects were rejected in the general population. However, the Mendelian dominant and co-dominant models were found in high risk families. CONCLUSION: In conclusion, the randomly ascertained Korean families of this study showed strong familial aggregation in the LDL cholesterol level. This suggested that variations in the level of LDL cholesterol may be influenced by a major gene effect.
Alcohol Drinking ; Cholesterol, LDL ; Education ; Humans ; Siblings ; Smoke ; Smoking ; Spouses ; Waist Circumference

OBJECTIVES: The purpose of this study was to investigate the familial correlation and heritability in HDL cholesterol through segregation analysis. METHODS: This study, based on data from 11,117 Korean individuals ascertained pedigrees who had participated in 1998 and 2001 Korean National Health & Nutrition Examination Survey. The subjects of segregation analysis were 4,688 Korean who had more than five members in their family. RESULTS: Serum lipid levels revealed strong familial correlation among spouses, parent-offspring and siblings with low correlation of spouse. The heritability of HDL cholesterol was 54.8% after controlling for age, age2, gender, agexgender, waist circumference, smoking, alcohol drinking, exercise and education. Two models of inheritance, the Mendelian dominant model and the Mendelian codominant model were found in HDL cholesterol. In the codominant model, the predicted HDL-cholesterol for genotype AA, AB, and BB were 44.96, 49.13, and 69.67 mg/dl, respectively. However the Mendelian codominant model only was found in high risk families. CONCLUSIONS: In conclusion, randomly ascertained Korean families of this study showed strong familial aggregation in HDL cholesterol. The results suggested that the variations in HDL cholesterol may be influencing by major effect of gene.
Alcohol Drinking ; Cholesterol, HDL ; Education ; Genotype ; Humans ; Siblings ; Smoke ; Smoking ; Spouses ; Waist Circumference ; Wills

Coronary artery fistula (CAF) is a rare congenital malformation, comprising less than 1% of all congenital heart disease. Most fistulas drain into the right-sided heart chambers or pulmonary artery, and termination in left heart chambers is an uncommon finding. Clinical presentation mainly depends on the severity of the left-to-right shunt, and adult patients usually do not develop any symptom. There are few case reports relating CAF manifested as an exceptional form of sudden unexpected death. We experienced a case of a 62-year-old male with a single CAF arising from left main coronary artery which drains into the left ventricle, and reported with review of the related articles.
Adult ; Cardiomegaly ; Coronary Vessels ; Death, Sudden ; Fistula ; Heart ; Heart Diseases ; Heart Ventricles ; Humans ; Male ; Middle Aged ; Pulmonary Artery

OBJECTIVE: We report an outcome of surgical treatment of the elderly patients with spinal stenosis, managed by a bilateral narrowed spinal canal widening technique through unilateral approach. METHODS: The operations were performed in 16 patients who diagnosed with spinal stenosis. All individuals had been presented with low-back pain, neurogenic claudication or radiculopathy and unresponsive to conservative treatment over six months. We perfomed hemi-laminectomy at the appropriate levels on the most symptomatic side preserving the facet joint. And the ligamentum flavum, as well as the cortical bone on the ventral surface of the contralateral laminae were removed. The spinous process was left as possible as we can, and the contralateral side of the spinal canal was decompressed completely. RESULTS: The mean age of the patients was 71 years. The mean operation time was 73 minutes. Despite of old age, the patients were able to walk in three days after the surgery. The significant pain scale improvement(7.73 to 2.68) and widening of the spinal canal diameter(7.60+/-1.75 to 17.77+/-1.47mm) were noted after the operation. No patient was presented spinal instability on their follow-up period over 24 months. CONCLUSION: The bilateral canal widening technique through the unilateral approach, minimizes the damage to the inter-spinous ligament and the inter-spinous muscle, and saves the operation time because it is not necessary to use the instruments which prevent spinal instability, despite spinal canal was sufficiently enlarged.
Aged* ; Follow-Up Studies ; Humans ; Ligaments ; Ligamentum Flavum ; Radiculopathy ; Spinal Canal ; Spinal Stenosis* ; Zygapophyseal Joint

The most common form of genetic nephrogenic diabetes insipidus(NDI), a rare inherited disorder, is congenital and is transmitted in an X-linked recessive mode. It is refractory to the antidiuretic effect of normal to moderately increased levels of plasma arginine vasopressin(AVP) but, in some cases, may respond to high levels of the hormone or its analogue, deamino-D-arginine vasopressin(DDAVP). X-linked congenital NDI has now been linked to over 128 different mutations in diverse coding regions of the AVP receptor 2(AVPR2) gene. The functional effects of these mutations vary from complete loss of responsiveness to a simple shift to the right in the dose response curve. We report a case of congenital partial NDI, with transversion of A to G at codon 280 of the AVPR2 gene, resulting in a subsequent change of amino acid from tyrosine to cysteine, and that has been effective with hydrochlorothiazide and high dose of DDAVP.
Antidiuretic Agents ; Arginine ; Clinical Coding ; Codon ; Cysteine ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Nephrogenic* ; Hydrochlorothiazide ; Plasma ; Tyrosine

Behcet's disease is a systemic vasculitis that is characterized mainly by recurrent oral and genital aphthous ulcers, uveitis, and skin findings. Its neurological manifestations are well recognized. Recurrent meningitis in Behcet's disease is exceptional. We describe herein the case of a 31-year-old man who presented with recurrent aseptic meningitis without any specific cause. A few years later he developed oral and genital ulcers, and uveitis. Behcet's disease should always be considered in a differential diagnosis of recurrent aseptic meningitis without viral infection, particularly in the context of multisystem manifestations.
Adult ; Diagnosis, Differential ; Humans ; Meningitis ; Meningitis, Aseptic ; Neurologic Manifestations ; Skin ; Stomatitis, Aphthous ; Systemic Vasculitis ; Ulcer ; Uveitis

BACKGROUND: The prediction of the absolute risk of ischemic heart disease (IHD) is commonly based on the risk prediction equations, originated from the Framingham Heart Study. METHOD: Framingham equation model was applied to participants from 2001 Korean National Health and Nutrition Examination Survey (KNHNES) to estimate the 5 year risk of IHD among Koreans ranging from 30 to 74 year-olds. The estimated risks were compared to the incidence and admission rates from two statistical reports among Koreans. Five year admission rate was estimated by the annual report from National Health Insurance Corporation (NHIC). RESULTS: The average ages (standard deviation) were 34.31(27.23) year-old for KNHNES and 48.26(12.87) year-old for Framingham population used in this study. The risk of IHD predicted by the Framingham equation model substantially exceeded the risks actually reported in Korea. Five-year predicted risks by Framingham equation model were 4.86% for men and 1.93% for women; whereas from incidence data in Korea, five-year risks for acute myocardial infarction (AMI) were for 0.47% for men and 0.18% for women. These AMI incidence was similar to the admission rate (0.34 for men and 0.15 for women) estimated by NHIC. Also, 5-year admission rate of IHD were 1.16 for men and 0.78 for women. The magnitude of risk overestimation by Framingham mode is approximately at least 150 to 320%. CONCLUSION: Korean guidelines for the management for high risk group of IHD need to develop and correct for overestimation to avoid inflation of costs in primary prevention.
Aged ; Female ; Heart ; Humans ; Incidence ; Inflation, Economic ; Korea ; Male ; Myocardial Infarction ; Myocardial Ischemia* ; National Health Programs ; Nutrition Surveys ; Primary Prevention