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

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

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

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

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: Video-assisted thoracoscopic surgery (VATS) for decortication or debridement in the management of empyema thoracis has increased the available treatment options but requires validation. We present and evaluate our technique and experience with thoracoscopic management of pleural empyema, irrespective of chronicity. MATERIAL AND METHOD: VATS debridement or decortication was performed with endoscopic shaver system in 40 consecutive patients presented with pleural space infections. A retrospective review was performed and the effect of this technique on perioperative outcome was assessed. RESULT: VATS evacuation of infected pleural fluid and decortication was successfully performed in 35 of 40 patients. The mean duration of preoperative symptoms before referral was 23 +/- 1.8 days. The mean duration of hospitalization before transfer was 13.5 +/- 1.5 days. Blood loss was 250 to 200 mL. Intercostal drainage was required for 5 +/- 3 days. The postoperative hospital stay was 5 +/- 0.7 days. There were no operative mortalities. CONCLUSION: Video-assisted evacuation of infected pleural fluid and decortication is an effective modality in the management of the fibropurulent stage of empyema. An organized empyema should be approached thoracoscopically, but may require open decortication.
Debridement ; Drainage ; Empyema* ; Empyema, Pleural ; Hospitalization ; Humans ; Length of Stay ; Mortality ; Referral and Consultation ; Retrospective Studies ; Thoracic Surgery, Video-Assisted ; Thoracoscopy

BACKGROUND: Atherosclerosis, the most common cause of death in type 2 diabetic patients, is closely associated with coronary artery calcium deposition. The coronary calcifications can be easily measured using coronary calcium scoring computed tomography (CT). And microalbuminuria is known as an independent risk factor of cardiovascular disease. So, we examined the association of urinary albumin to creatinine ratio (UACR) and coronary calcification score (CCS) in type 2 diabetic patients. METHODS: Among type 2 diabetic patients who underwent the multidetector CT scanning for the evaluation of CCS at Kyungpook National University Hospital between December 2007 and May 2008, 155 subjects were included. CCS, demographic and laboratory data were assessed. RESULTS: Coronary artery calcifications were identified in 90 patients (51%) and mean, median CCS was 205.8 +/- 476.9, 8.74 (0, 132.0). 60 subjects revealed UACR greater than 30 ug/mg. With the UACR increment, CCS revealed a significant increase (P < 0.001). Age, duration of diabetes, serum Apo A1 level, serum high sensitivity C-reactive protein (hs-CRP) level were also associated with CCS. However, after adjusting for age, UACR and CCS exhibited a significant positive relationship (P = 0.002). CONCLUSION: Increased UACR is associated with coronary artery calcification in type 2 diabetic patients and these results will be useful in early evaluating the presence of macrovascular complications in these patients.
Albuminuria ; Apolipoprotein A-I ; Atherosclerosis ; C-Reactive Protein ; Calcium ; Cardiovascular Diseases ; Cause of Death ; Coronary Artery Disease ; Coronary Vessels ; Creatinine ; Diabetes Mellitus, Type 2 ; Humans ; Risk Factors