We present a family of FSP with review of literatures. Seeligmuller (1876) and Strumpell (1880) were the first to describe familial case of spastic paraplegia characterized by progressive weakness and spasticity of the lower limbs with little or no involvement of the upper extremities. This syndrome is heterogenous in inheritance, age of onset, severity and associated signs. A pure type without complications has been named "Strumpell's FSP". We have recently experienced a case of 17 years old male who had bilateral weakness and stiffness of lower extremities, and gait disturbance with family history. Differential diagnosis, particularly with familial amyotrophic lateral sclerosis, is discussed.
Adolescent ; Age of Onset ; Amyotrophic Lateral Sclerosis ; Diagnosis, Differential ; Gait ; Humans ; Lower Extremity ; Male ; Muscle Spasticity* ; Paraplegia* ; Upper Extremity ; Wills

Patients with acromegaly have a reduced life expectancy rnainly due to cardiovascular, respiratory or cerebrovascular diseas-. Malignancy also seems to occur with greater than the expected incidence. In particular, the published retrospective or prospective studies have suggested a strong association of colonic neoplasia with acromegaly. But, there were a few reports of thyroid cancer in acrornegaly. We report a case of thyroid papillary cancer derived from diffuse goiter in acromegaly, sugge- sting the possible carcinogenic role of growth hormone.
Acromegaly ; Bites and Stings ; Colon ; Goiter ; Growth Hormone ; Humans ; Incidence ; Life Expectancy ; Prospective Studies ; Retrospective Studies ; Thyroid Gland ; Thyroid Neoplasms

The detailed clinical characteristics of unilateral moyamoya disease (MMD) have not been fully elucidated. It has been reported that some patients with unilateral MMD progress to bilateral involvement, while others remain with the unilateral variant. In this series, we present a case of unilateral MMD that progressed to bilateral involvement over the course of just one month.
Humans ; Moyamoya Disease

OBJECTIVE: The incidence of subarachnoid hemorrhage (SAH) worldwide varies considerably. In spite of many reports about the incidence of SAH, there has been no report about the incidence of SAH on the basis of the Korean population. The purpose of this hospital-based study was to assess the actual incidence rates of aneurysmal SAH in Gwangju city and Jeollanamdo province. METHODS: All cases of SAH confirmed by computerized tomography (CT) between January 2007 and December 2007 were selected for analysis. For the data collection, three major training hospital and ten general hospitals working the CT in Gwangju city and four major general hospitals in Jeollanamdo province participate in this study. RESULTS: According to the official census of Korea, the population was 1,413,444 in Gwangju city and 1,929,836 in Jeollanamdo province in 2007. There were 163 patients in Gwangju city and 266 patients in Jeollanamdo province confirmed SAH by CT in 2007. The crude and the age- and sex-adjusted annual incidence rates per 100,000 population for all ages in Gwangju city were 11.5 and 12.4 for aneurysmal SAH and in Jeollanamdo province were 13.8 and 10.8. The incidence was higher in women and increased with age. The gender distribution varied with age. At young ages, the incidence was higher in men while after the age of 40 years, the incidence was higher in women. CONCLUSION: In the present study, the age- and sex-adjusted annual incidence rates is 11.8 in Gwangju city and Jeollanamdo province. The incidence was higher in women and increased with age.
Aneurysm ; Censuses ; Data Collection ; Female ; Hospitals, General ; Humans ; Incidence ; Korea ; Male ; Risk Factors ; Subarachnoid Hemorrhage

BACKGROUND: The Korean National Health and Nutrition Examination Surveys reported 65% of daily energy intake (EI) as carbohydrate (CHO) in the Korean population and main source of CHO was cooked rice. We used a standardized-small sized rice bowl for diet education and investigated its effectiveness on body weight, glucose and lipid, compared to the conventional food exchange system in type 2 diabetes obese women. METHODS: Type 2 diabetic women with body mass index > or = 23 kg/m2 were randomly assigned to small rice bowl-based meal plan (BM) and food exchange-based meal plan (ExM) group. Both groups were asked to reduce their EI by 500 kcal/day for 12 weeks. The macronutrient composition was instructed: 55 to 60% of EI as CHO, 15 to 20% as protein, and 20 to 25% as fat. BM group received only a simple instruction for application of the rice bowl. Nutrient intake was estimated with the 3-day dietary records. RESULTS: Finally, 44 subjects finished the study. The percent reduction of body weight was significant both BM group (-5.1 +/- 2.6%) and ExM group (-4.8 +/- 2.8%) after 12 weeks (P < 0.001) but there was no difference between the groups. There was no difference in the proportional change of CHO, protein and fat in EI between the groups. Additionally, the change of HbA1c and low density lipoprotein-cholesterol were not significantly different between the two groups. CONCLUSION: The BM group was as effective as ExM for body weight and glucose control in type 2 diabetes obese women.
Body Mass Index ; Body Weight ; Diabetes Mellitus, Type 2 ; Diet, Diabetic ; Diet ; Diet Records ; Energy Intake ; Female ; Glucose ; Humans ; Meals ; Obesity ; Weight Loss

This study was performed to examine the characteristics of protein of red crab (Chionoecetes japonicus) shell powder hydrolyzed by commercial proteases. Red crab shell was digested by commercial proteases, such as Protamex (P), Neutrase (N), Flavourzyme (F), Alcalase (A), Protease M (PM) and Protease A (PA). Protein yield analyzed by Biuret assay, absorbance at 280 nm and brix revealed that PA was the enzyme having the highest proteolytic activity. SDS PAGE showed that molecular weight of proteins produced by protease treatments was various and below 150 kDa. Combinational treatment of proteases (PA + P, PA + PM, PA + F, PA + A) was tried whether these increase protein hydrolysis from red crab shell powder compared to a PA single treatment. Soluble protein content was similar, but amino acid concentration by combinational treatments was higher than PA single treatment [PA + P 247.4 mg/g > PA + F (206.4 mg/g) > PA + A (133.4 mg/g) > PA + PM (59.1 mg/g) > PA (54.9 mg/g)]. Amino acid composition by combinational treatments was slightly different. Most abundant essential amino acids were phenylalanine, glycine, alanine, and leucine, whereas tyrosine and cystine were not detected.
Alanine ; Amino Acids, Essential ; Biuret ; Cystine ; Electrophoresis, Polyacrylamide Gel ; Endopeptidases ; Glycine ; Hydrolysis ; Leucine ; Metalloendopeptidases ; Molecular Weight ; Peptide Hydrolases ; Phenylalanine ; Proteins ; Subtilisins ; Tyrosine

Immunoglobulin G4-related sclerosing disease is a novel clinicopathological disease entity known to involve various organs including the pancreas, bile ducts, gall bladder, retroperitoneum, kidney, salivary gland, lung and prostate. The most common organ involved is the pancreas and cases without pancreatic involvement are uncommon. Positive response to steroids is an important characteristic of this disease and this enables early diagnosis, which is required for good prognosis. We demonstrate a case of immunoglobulin G4-related tubulointerstitial nephritis in a 59-year-old male accompanied by sclerosing cholangitis and sialadenitis without any evidence of pancreatic infiltration. The patient was treated with prednisolone and was fully recovered in 6 months.
Bile Ducts ; Cholangitis ; Cholangitis, Sclerosing ; Early Diagnosis ; Humans ; Immunoglobulins ; Kidney ; Lung ; Male ; Middle Aged ; Nephritis, Interstitial ; Pancreas ; Prednisolone ; Prognosis ; Prostate ; Salivary Glands ; Sialadenitis ; Steroids ; Urinary Bladder

BACKGROUND: The incidence of type 2 diabetes mellitus is increasing annually and patient mortality is high. Coronary artery calcification is a predictor of coronary artery disease. Cardiovascular events, which are the main cause of death in type 2 diabetes patients, may be preventable by addressing risk factors associated with coronary artery calcification. We examined the relationships between coronary artery calcification, lipid profiles, and apolipoprotein levels. METHODS: We calculated the coronary calcium scores (CCS) of 254 subjects with type 2 diabetes (113 males, 141 females) via multi-detector row computed tomography (MDCT). Height, body weight, blood pressure, HbA1c, c-peptide, lipid profile and apolipoprotein were assessed concurrently. RESULTS: In patients with type 2 diabetes, Agatston score and apolipoprotein A-1 were significantly negatively correlated in both males and females (males P = 0.015, females P = 0.021). The negative correlation between Agatston score and apolipoprotein A-1 was retained for the entire patient sample after adjustments for age and sex (P = 0.022). Stepwise multiple regression anaylses with the Agatston score as the dependent variable indicate that apolipoprotein A-1 is a independent predictor (beta coefficient = -0.047, 95%CI = -0.072 ~ -0.021, P < 0.001) of coronary artery calcification. CONCLUSION: The results of our study suggest that apolipoprotein A-1 is a useful independent indicator of coronary artery calcification.
Apolipoprotein A-I ; Apolipoproteins ; Atherosclerosis ; Blood Pressure ; Body Height ; C-Peptide ; Calcium ; Cardiovascular Diseases ; Cause of Death ; Coronary Artery Disease ; Coronary Vessels ; Diabetes Mellitus, Type 2 ; Female ; Humans ; Incidence ; Male ; Risk Factors

Bilateral adrenal neoplasms are associated with metastatic cancer, pheochromocytoma and lymphoma. The coexistence of a unilateral functioning adrenocortical adenoma with contralateral pheochromocytoma is extremely rare. A 52-year-old woman complained of fatigue, headache, palpitation, and progressive weight gain. Hormonal assessment demonstrated high 24 hours urine epinephrine, norepinephrine, and free cortisol. A dexamethasone suppression test (overnight 1 mg, low dose 2 mg) showed insuppressible cortisol. Computerized tomographic scanning revealed a bilateral adrenal tumor. To preserve adrenal function, right adrenalectomy along with left adrenal tumorectomy was performed. Histological finding of the right adrenal tumor was pheochromocytoma and the left adrenal tumor was adrenocortical adenoma. This patient was the first case of a functional adrenocortical adenoma with contralateral cystic pheochromocytoma in Korea. We report the case with a review of the literature.
Adrenal Gland Neoplasms ; Adrenalectomy ; Adrenocortical Adenoma ; Cushing Syndrome ; Dexamethasone ; Epinephrine ; Fatigue ; Female ; Headache ; Humans ; Hydrocortisone ; Korea ; Lymphoma ; Middle Aged ; Norepinephrine ; Pheochromocytoma ; Weight Gain

BACKGROUND: In aortic surgery, division and ligation of the left brachiocephalic vein(LBV) may improve exposure of the aortic arch but controversy continues about the safety of this division and whether a divided vein should be reanastomosed after arch replacement was completed. The safety of LBV division and the fate of the left subclavian venous drainage after LBV division were studied. MATERIAL AND METHOD: From November 1998 to January 2001, planned division and ligation of the LBV on the mid-line after median sternotomy was performed in 10 patients during the aortic surgery with the consideration of local anatomy and distal aortic anastomosis. Assessment for upper extremity edema and neurologic symptoms, measurement of venous pressure in the right atrium and left internal jugular vein, and digital subtraction venography(DSV) of the left arm were made postoperatively. RESULT: In 10 patients there was improvement in access to the aortic arch for procedures on the ascending aorta or aortic arch. The mean age of patients was 62 years(range 24 to 70). Follow-up ranged from 3 weeks to 13 months. One patient died because of mediastinitis from methicilline-resistant staphylococcus aureus strain. All patients had edema on the left upper extremity, but resolved by the postoperative day 4. No patient had any residual edema or difficulty in using the left upper extremity during the entire follow-up period. No patient had postoperative stroke. Pressure difference between the right atrium and left internal jugular vein was peaked on the immediate postoperative period(mean peak pressure difference = 25mmHg), but gradually decreased, then plated by the postoperative day 4. In all DSV studies left subclavian vein flowed across the midline through the inferior thyroid venous plexus. CONCLUSION: We conclude that division of LBV is safe and reanastomosis is not necessary if inferior thyroid vein, which is developed as a main bridge connecting the left subclavian vein with right venous system, is preserved.
Aorta ; Aorta, Thoracic ; Arm ; Brachiocephalic Veins* ; Drainage ; Edema ; Follow-Up Studies ; Heart Atria ; Humans ; Jugular Veins ; Ligation ; Mediastinitis ; Neurologic Manifestations ; Staphylococcus aureus ; Sternotomy ; Stroke ; Subclavian Vein ; Thyroid Gland* ; Upper Extremity ; Veins* ; Venous Pressure