Leber's hereditary optic neuropathy (LHON) is a disease due to mitochondrial DNA mutations. We report a man with LHON, who presented with four episodes of respiratory failure. In the last episode, he had a loss of the automatic component of the respiratory drive but retained the voluntary component. Brain MRI showed lesions in the midbrain, pons, and medulla oblongata, involving the neuroanatomic areas of the medullary respiratory center. Our patient provides a further example of the broad manifestations of mitochondrial disease.
Brain ; DNA, Mitochondrial ; Humans ; Magnetic Resonance Imaging ; Medulla Oblongata ; Mesencephalon ; Mitochondrial Diseases ; Optic Atrophy, Hereditary, Leber* ; Pons ; Respiratory Center ; Respiratory Insufficiency*

BACKGROUND/OBJECTIVES: Dietary sugar intake, particularly added sugar and sugar-sweetened beverages, has received worldwide attention recently. Investigation of dietary behaviors may facilitate understanding of dietary sugar intakes of children and adolescents. However, the relationship between dietary sugar intake and dietary behaviors in the Korean population has not been investigated. Thus, this study aimed to estimate dietary sugar intake and food sources according to sex as well as examine the relationship of dietary sugar intake with frequent snacking and dietary patterns among Korean children and adolescents. SUBJECTS/METHODS: We pooled data from five studies involving Korean children and adolescents conducted from 2002 to 2011. A total of 2,599 subjects aged 9-14 years were included in this study. Each subject completed more than 3 days of dietary records. RESULTS: Mean daily total sugar intake was 46.6 g for boys and 54.3 g for girls. Compared with boys, girls showed higher sugar intakes from fruits (7.5 g for boys and 8.8 g for girls; P = 0.0081) and processed foods (27.9 g for boys and 34.9 g for girls; P < 0.0001). On average, 95.4% of boys and 98.8% of girls consumed snacks during the study period, and total sugar intake showed a significantly increasing trend with increasing energy intake from snacks (P < 0.0001 for both sexes). Two dietary patterns were identified by cluster analysis: Traditional and Westernized patterns. Total sugar intake was higher in the Westernized pattern (56.2 g for boys and 57.2 g for girls) than in the Traditional pattern (46.5 g for boys and 46.3 g for girls). CONCLUSIONS: These results suggest that multilateral and practical development of a nutrition education and intervention program that considers dietary behaviors as well as absolute sugar intake is required to prevent excessive sugar intake in Korean children and adolescents.
Adolescent* ; Beverages ; Child* ; Diet Records ; Education ; Energy Intake ; Female ; Fruit ; Humans ; Korea* ; Snacks

Dementia pugilistica (DP) or chronic traumatic encephalopathy (CTE) is a neurodegenerative disease or dementia that may affect amateur or professional boxers as well as athletes in other sports who suffer concussions. The condition is thought to affect around 15% to 20% of professional boxers and caused by repeated concussive or subconcussive blows. CTE was in the past referred to as dementia pugilistica, which reflected the prevailing notion that this condition was restricted to boxers. Recent research, however, has demonstrated neuropathological evidence of CTE in retired American football players, a professional wrestler, a professional hockey player and a soccer player, as well as in nonathletes. It is probable that many individuals are susceptible to CTE, including those who experience falls, motor vehicle accidents, assaults, epileptic seizures, or military combat, and that repeated mild closed head trauma of diverse origin is capable of instigating the neurodegenerative cascade leading to CTE. We report a 62-year old man suspicious of dementia pugilistica with clinical features of frontotemporal dementia and parkinsonism.
Athletes ; Brain Injury, Chronic ; Dementia ; Epilepsy ; Football ; Frontotemporal Dementia ; Head Injuries, Closed ; Hockey ; Humans ; Military Personnel ; Motor Vehicles ; Neurodegenerative Diseases ; Parkinsonian Disorders ; Soccer ; Sports

PURPOSE: The goal of this study is to assess the interactions among alcohol consumption, cigarette smoking, and aspartate aminotransferase (AST) / alanine aminotransferase (ALT) ratios on esophageal cancer. MATERIALS AND METHODS: Alcohol and the risk of incidence and death from esophageal cancer were examined in a 14-year prospective cohort study of 782,632 Korean men, 30 to 93 years of age, who received health insurance from the National Health Insurance Corporation and had a medical evaluation from 1992 to 1995. RESULTS: Smoking, alcohol intake, and AST/ALT ratios were associated with the increased risk of esophageal cancer in a dose-dependent manner independent of each other. Smoking was associated with an increased risk of incidence [Hazard ratio (HR) = 2.2, 95% CI = 1.8 to 2.5] and mortality (HR = 2.5, 2.0 to 3.1). Combined HR of incidence for alcohol consumption (> 25 g/day) and smoking was 4.5 (3.8-5.5); for alcohol (> 25 g/day) and the AST/ALT ratio (> or = 2.0), it was 5.8 (4.6-7.2); for smoking and the AST/ALT ratio (> or = 2.0), it was 6.3 (5.1-7.5). Similar results were seen for mortality from esophageal cancer. Subjects who drank > or = 25 g/day with an AST/ALT ratio > or = 2 had a higher risk of esophageal cancer incidence (HR = 6.5, 4.8 to 8.7) compared with those who drank > or = 25 g/day with an AST/ALT ratio < 2 (HR = 2.2, 1.9 to 2.6). CONCLUSION: Alcohol, smoking, and the AST/ALT ratio are independently associated with increased risk of esophageal cancer but did not interact synergistically. The combination of the AST/ALT ratio with a questionnaire for alcohol consumption may increase the effectiveness for determining the risk of esophageal cancer.
Adult ; Aged ; Aged, 80 and over ; Alanine Transaminase/*blood ; Alcohol Drinking/*adverse effects ; Asian Continental Ancestry Group ; Aspartate Aminotransferases/*blood ; Esophageal Neoplasms/*enzymology/*epidemiology/etiology ; Humans ; Korea/epidemiology ; Male ; Middle Aged ; Risk Factors ; Smoking/*adverse effects

PURPOSE: The goal of this study is to assess the interactions among alcohol consumption, cigarette smoking, and aspartate aminotransferase (AST) / alanine aminotransferase (ALT) ratios on esophageal cancer. MATERIALS AND METHODS: Alcohol and the risk of incidence and death from esophageal cancer were examined in a 14-year prospective cohort study of 782,632 Korean men, 30 to 93 years of age, who received health insurance from the National Health Insurance Corporation and had a medical evaluation from 1992 to 1995. RESULTS: Smoking, alcohol intake, and AST/ALT ratios were associated with the increased risk of esophageal cancer in a dose-dependent manner independent of each other. Smoking was associated with an increased risk of incidence [Hazard ratio (HR) = 2.2, 95% CI = 1.8 to 2.5] and mortality (HR = 2.5, 2.0 to 3.1). Combined HR of incidence for alcohol consumption (> 25 g/day) and smoking was 4.5 (3.8-5.5); for alcohol (> 25 g/day) and the AST/ALT ratio (> or = 2.0), it was 5.8 (4.6-7.2); for smoking and the AST/ALT ratio (> or = 2.0), it was 6.3 (5.1-7.5). Similar results were seen for mortality from esophageal cancer. Subjects who drank > or = 25 g/day with an AST/ALT ratio > or = 2 had a higher risk of esophageal cancer incidence (HR = 6.5, 4.8 to 8.7) compared with those who drank > or = 25 g/day with an AST/ALT ratio < 2 (HR = 2.2, 1.9 to 2.6). CONCLUSION: Alcohol, smoking, and the AST/ALT ratio are independently associated with increased risk of esophageal cancer but did not interact synergistically. The combination of the AST/ALT ratio with a questionnaire for alcohol consumption may increase the effectiveness for determining the risk of esophageal cancer.
Adult ; Aged ; Aged, 80 and over ; Alanine Transaminase/*blood ; Alcohol Drinking/*adverse effects ; Asian Continental Ancestry Group ; Aspartate Aminotransferases/*blood ; Esophageal Neoplasms/*enzymology/*epidemiology/etiology ; Humans ; Korea/epidemiology ; Male ; Middle Aged ; Risk Factors ; Smoking/*adverse effects

BACKGROUND: Agent Orange (AO) is the code name for one of the herbicides and defoliants used in the Vietnam War. Studies conducted thus far show a significant correlation between AO and the occurrence of cardiovascular diseases. But there is little data on the association between AO and stroke, and limited studies have targeted patient groups exposed to AO. METHOD: Bohun medical center Institutional Review Board (IRB) approved the study. (ID: 341) We studied patients with acute ischemic stroke within 7 days of onset in VHS medical center and 4 other general hospitals. Among them, 91 consecutive patients with previous exposure to AO were evaluated. For controlled group, 288 patients with no history of AO exposure were chosen. RESULT: There were 49 (44.0 %) DM patient with a higher frequency in the exposure group (93 (32.3 %) in control P = 0.045). There were 6 (6.6 %) hyperlipidemia in exposure group and 69 (24.0 %) in control. (P < 0.002). Small vessel occlusion was the most common subtype (36, 39.6 %) in exposure group but in control group, the large artery atherosclesosis was (120, 41.7 %) (P = 0.014). The NIHSS of the exposure group on admission showed lower scores (median values, 2 and 4, respectively; P = 0.003). The median mRS was 1 for the exposure group and 2 for the control group, at discharge and after 3 months. After 3 months of discharge, 55 (60.4 %) in the exposure group and 171 (59.4 %) in the control group showed below mRS 1 (P = 0.001). CONCLUSION: This study targeted patients who are Vietnam veteran. There is some difference in vascular risk factors and clinical manifestations suggest AO exposure has contributed to a certain extent to the stroke.
Arteries ; Cardiovascular Diseases ; Cerebral Infarction* ; Citrus sinensis* ; Ethics Committees, Research ; Herbicides ; Hospitals, General ; Humans ; Hyperlipidemias ; Methods ; Prognosis* ; Risk Factors ; Stroke ; Veterans ; Vietnam