Model of music cognition and amusia.

INTRODUCTION: The study of the neural networks involved in music processing has received less attention than work researching the brain's language networks. For the last two decades there has been a growing interest in discovering the functional mechanisms of the musical brain and understanding those disorders in which brain regions linked with perception and production of music are damaged. DISCUSSION: Congenital and acquired musical deficits in their various forms (perception, execution, music-memory) are grouped together under the generic term amusia. In this selective review we present the "cutting edge" studies on the cognitive and neural processes implicated in music and the various forms of amusia. CONCLUSIONS: Musical processing requires a large cortico-subcortical network which is distributed throughout both cerebral hemispheres and the cerebellum. The analysis of healthy subjects using functional neuroimaging and examination of selective deficits (e.g., tone, rhythm, timbre, melodic contours) in patients will improve our knowledge of the mechanisms involved in musical processing and the latter's relationship with other cognitive processes.

Childhood-onset chylomicronaemia with reduced plasma lipoprotein lipase activity and mass: identification of a novel GPIHBP1 mutation.

OBJECTIVES: Deficiency in the catabolism of triglyceride-rich lipoproteins is the main cause of childhood-onset chylomicronaemia syndrome. Missense mutations in lipoprotein lipase (LPL) or in proteins influencing LPL activity or stability have been shown to be critical determinants of chylomicronaemia syndrome. The main objective of this study was to assess the primary deficiency in five cases of childhood-onset chylomicronaemia syndrome. SETTING: Lipid clinic at a university hospital, SUBJECTS: Subjects presenting with severe hypertriglyceridaemia and chylomicronaemia syndrome in which reduced LPL activity and mass were observed. INTERVENTIONS: Analysis of LPL and GPIHBP1 genes. RESULTS: Amongst the five patients, one novel homozygous missense mutation (p.C68Y) in exon 3 of GPIHBP1 was identified. The other four patients were homozygous for the common LPL mutation p.G188E. CONCLUSION: These findings provide further evidence that GPIHBP1 is involved in the catabolism of triglyceride-rich lipoproteins and plays a role in childhood-onset chylomicronaemia.

High cardiovascular risk in Spanish workers.

BACKGROUND AND AIMS: To investigate the prevalence of high cardiovascular risk in the Spanish working population, and its distribution among different occupations and gender. METHODS AND RESULTS: Cross-sectional study of 309,955 workers (72.6% males, mean age 36.5 years, range 16-74 years), who underwent a routine medical check-up. Workers were classified as high, intermediate or low cardiovascular risk, according to the SCORE system. Workers with a relative risk greater than 4 were also considered as high-risk. The prevalence of high cardiovascular risk was 7.6% (95% CI 7.5-7.7) in males and 1.7% (95% CI 1.6-1.8) in females. After adjusting for age and gender, the prevalence of high cardiovascular risk was greater in workers from the Agriculture and Construction sectors than in those from Industry and Service sectors. The prevalence of high cardiovascular risk was higher in blue-collar than in white-collar occupations. CONCLUSIONS: A sizeable proportion of workers, especially blue-collar males, are at high cardiovascular risk. Knowledge of this risk for certain workers may serve as a basis for preventive strategies.

Lupus-associated endothelial dysfunction, disease activity and arteriosclerosis.

OBJECTIVE: To analyze endothelial function in systemic lupus erythematosus (SLE), and its relationship with disease activity and subclinical arteriosclerosis. METHODS: We studied a group of 26 patients with SLE and 21 age- and sex-matched controls. None of the patients or controls had had any ischemic event. Data were recorded on medical history, anthropometrics, prior treatment and the lupus activity index (LAI). Endothelial function was quantified by flow-mediated dilatation in the brachial artery. The presence of subclinical arteriosclerosis was assessed by the average intima-media thickness (IMT) on carotid ultrasound. RESULTS: The patients and the controls had a similar degree of carotid IMT (0.58+/-0.08 mm vs. 0.57+/-0.07 mm, NS) and a similar prevalence of carotid plaque (27% vs. 24%, NS). However, the SLE patients had worse endothelial function than the controls (FMD 12.4+/-4.4% vs. 16.9+/-5.5%, p<0.05). This difference remained after adjusting for age, smoking, body mass index, waist circumference, total cholesterol, triglycerides, HDL cholesterol, apolipoproteins A-1 and B100 and postmenopausal status. A significant association was found in the SLE patients between FMD and LAI (Spearman Rho -0.462, p<0.05). CONCLUSION: SLE-associated endothelial dysfunction is present in patients who have no prior ischemic events and with the same degree of subclinical arteriosclerosis as controls. The endothelial dysfunction is significantly associated with the degree of disease activity.

Systemic lupus erythematosus in Europe at the change of the millennium: lessons from the "Euro-Lupus Project".

The "Euro-Lupus Cohort" is composed by 1000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium--the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.

Effects of interferon-beta on plasma lipid and lipoprotein composition and post-heparin lipase activities in patients with chronic hepatitis C.

BACKGROUND: Interferon therapy has been shown to induce lipid abnormalities. AIM: We assessed the effects of interferon-beta on the lipoprotein profile and post-heparin lipase activities in 26 normolipaemic patients with chronic hepatitis C. METHODS: Interferon-beta was administered subcutaneously at doses of 6 x 106 U (units) three times a week, over 6 months, and lipoproteins and post-heparin lipases were measured at baseline and at the end of therapy. RESULTS: Plasma triglycerides increased by 21% due to preferential enrichment in those contained in the very low density lipoprotein (VLDL) and low density lipoprotein (LDL) fractions. The concentration of cholesterol decreased slightly in the high density lipoprotein (HDL) subfractions. Lipoprotein lipase, but not hepatic lipase activity decreased by a 36%, and this change showed a significant negative correlation with changes in plasma triglycerides. Five patients (19.5%) responded to interferon-beta therapy. The lipoprotein profile was no different between responders and non-responders to therapy. CONCLUSIONS: Interferon-beta treatment in normolipaemic patients with chronic hepatitis C induced moderate disturbances in plasma lipoproteins, associated with inhibition of lipoprotein lipase activity.

Apolipoprotein distribution in plasma HDL subfractions in alcohol consumers.

Alcohol intake is known to increase plasma HDL subfractions and apo A levels. Few data are available, however, with regard to precise apoprotein composition of plasma lipoproteins in alcohol consumers. The aim of the present study was to examine the lipid and apoprotein distribution in plasma lipoproteins of subjects with different levels of alcohol intake. Thirty-six male regular drinkers were classified as social (n = 10, average daily consumption greater than or equal to 5 and less than or equal to 20 g), moderate (n = 11, average daily consumption greater than 20 g and less than 70 g) and heavy drinkers (n = 15, average daily consumption greater than or equal to 70 g). Reference data were obtained from 11 subjects, all lifelong abstainers. Lipoproteins were fractioned by sequential ultracentrifugation and apolipoprotein distribution in VLDL, HDL-2 and HDL-3 was determined by isoelectrofocusing in polyacrylamide-urea gel. HDL-2 cholesterol was significantly higher in heavy drinkers (P less than 0.005). A parallel trend for HDL-3 cholesterol was observed. In alcohol consumers HDL-2 particles were enriched in apos C, in particular apo C-II (P 0.005), displacing apo A-I. These data suggest that apolipoprotein distribution in the HDL-2 subfraction is influenced by alcohol intake even at the lowest level.

Absence of the inferior vena cava causing repeated deep venous thrombosis in an adult--a case report.

Agenesis of the inferior vena cava is an uncommon congenital vascular malformation. Although theoretically it may favor venous stasis, it is not normally associated with venous thrombosis. The authors found only three reports of the coexistence of both entities, and only one of these rules out the existence of associated hypercoagulability. They report the case of a patient with a history of repeated venous thrombosis. Magnetic resonance angiography and venography demonstrated complete agenesis of the inferior vena cava and persistence of a cardinal venous system with azygos and hemiazygos continuation, associated with right renal hypoplasia. Studies ruled out hypercoagulability syndrome, either primary or secondary to an occult neoplasm. The authors conclude that venous malformations, although rare, may cause thromboembolic disease, especially in young patients, and that magnetic resonance angiography is a useful diagnostic alternative to conventional phlebography.

Short-term effect of various doses of cyclosporin A on plasma lipoproteins and its distribution in blood: an experimental study.

Hyperlipidaemia commonly develops in both transplant recipients and experimental animals receiving cyclosporin A (CsA). However, the threshold of CsA induced-changes on lipoproteins and the role of parenteral vehicle (cremophor) has not been defined. Male Wistar rats were classified into five groups of six animals each and received CsA in cremophor vehicle at doses of 5, 10 or 20 mg kg-1 d-1, s.c., vehicle alone or saline for 7 d. Blood was obtained 24 h after the last dose and plasma was analysed. Plasma very low density lipoprotein (VLDL), low density lipoprotein (LDL), and high density lipoprotein subfractions (HDL-2, HDL-3) were isolated by sequential ultracentrifugation and their content of cholesterol, triglyceride and phospholipid was determined. Whole blood and trough plasma CsA levels were measured by monoclonal radioimmunoassay. Plasma lipids did not differ significantly among the five groups. At a dose of 20 mg kg-1 d-1 of CsA VLDL cholesterol rose significantly (P < 0.05). Administration of either CsA or cremophor vehicle increased HDL-2 phospholipids (P < 0.05) and decreased HDL-3 cholesterol. There was not a linear relationship between whole blood and plasma CsA levels and increasing CsA doses. Short-term treatment with low doses of CsA have little influence on lipid profile in the rat. Changes on lipoprotein composition can be attributed mainly to cremophor vehicle, conceivably due to its ethanol content.

[Differences in prediction of mortality and non-fatal complications after acute myocardial infarction: a multivariate analysis]. / Diferencias en la predicción de mortalidad y complicaciones no fatales postinfarto agudo de miocardio: un análisis multivariable.

The global prognostic after acute myocardial infarction depends of the left ventricular function and the residual ischemia. However, there is controversy about the capacity of some variables reflecting this prognostic factors to predict futures complications. The main objective of this study is to know the influence of the kind of complications in the prognostic value of the variables more frequently used in the clinical practice. We studied 121 consecutive patients with acute myocardial infarction discharged from hospital. The previous and during acute infarction clinical variables were analyzed. The regional and global left ventricular function was studied by 2D echocardiography. An exercise test symptoms-limited was performed at 3rd week after discharge in 94 patients selected. The patients were followed during selected one year. The first year cardiac mortality rate was 8.6%. In the multivariate, the independent predictors of mortality were: age > 60 years (p = 0.02) and the impossibility to perform exercise test (p = 0.0002). In the univariate analysis the echocardiographic motility score > or = 17, eyection fraction < 35% and Killip class > or = II were also death predictors. Non fatal complications (angina, reinfarction o heart failure) were present in 47 patients (42.3%) of 111 survivors. The ischemic complications were predicted in multivariate analysis only by postinfarction angina (p = 0.0007), and heart failure by eyection fraction < 35% (p = 0.006), previous infarct (p = 0.002) and Killip class (p = 0.04).(ABSTRACT TRUNCATED AT 250 WORDS)

[Right-sided endocarditis due to Fusobacterium nucleatum]. / Endocarditis derecha por Fusobacterium nucleatum.

We present 1 case of right sided endocarditis caused by Fusobacterium nucleatum in a patient with intravenous drug addiction and human immunodeficiency. The clinical features were fever, anemia, and pulmonary embolism. The echocardiogram showed a giant vegetation originated from the right atrial wall prolapsing in diastole into the right ventricle which disappeared after the patient presented pulmonary embolism. The clinical course was uncontrolled with empiric antimicrobial therapy but it was good with metronidazol. The cases previously described in the literature caused by gram-negative anaerobic bacteria are discussed and compared with the present case.

[Diet and hypertriglyceridemia: influence of dietary modifications on plasma lipids, lipoproteins and apoproteins in patients with primary hypertriglyceridemia]. / Dieta e hipertrigliceridemia: influencia de las modificaciones dietéticas sobre los lípidos, lipoproteínas y apoproteínas plasmáticas en pacientes con hipertrigliceridemia primaria.

BACKGROUND: To investigate the influence of dietary changes on plasma lipoproteins and apoproteins in 10 patients with hypertriglyceridemia (plasma triglycerides greater than 1000 mg/dl). METHODS: A sequential study with three periods was designed: 1) Inclusion with uncontrolled free diet; 2) Two weeks of a diet with about 1000 calories/day, controlled with a daily survey; and 3) One week of "free" diet of about 2000 calories. At the end of the three periods the following were measured in each patient: weight, glycemia, plasma IRI and C-peptide, plasma, triglycerides, cholesterol, chylomicron, VLDL, LDL and HDL cholesterol. At the end of the second and third periods the caloric and immediate principles intake were individually considered. RESULTS: A reduction in plasma cholesterol and triglycerides was observed in all cases after the caloric reduction, and also a disappearance of chylomicrons. The reduction of triglycerides took place in all lipoproteins. By contrast, after the low-calorie diet only the VLDL-cholesterol was reduced, while LDL- and HDL-cholesterol increased. The apo C-III/apo C-II ratio was significantly reduced after the low-calorie diet. The plasma glycemia, IRI and C-peptide were negatively correlated with LDL-cholesterol and with the apo C-III/apo C-II ratio. The best model to explain the oscillations of plasma triglycerides depending on the diet was that including the three ingested immediate principles together with the total diet calories. CONCLUSIONS: During the caloric restriction there would be an increased catabolism of VLDL to LDL. This reduction would be mediated by C-III and C-II apoproteins, probably through the oscillations of plasma IRI.

[Effect of various levels of alcohol consumption on plasma lipoproteins and apoproteins]. / Influencia de diversos grados de consumo de alcohol sobre las lipoproteínas y apolipoproteínas plasmáticas.

To assess the abnormalities induced by alcohol on plasma lipids and lipoproteins and their possibly involved mechanisms three basis of the daily intake: social drinkers (less than or equal to 20 g/day; n = 10), moderate drinkers (greater than 20 to less than 70 g/day; n = 11), and severe drinkers (greater than or equal to 70 g/day; n = 15). Eleven nondrinkers were evaluated as control group. Very low density lipoproteins (VLDL), low density lipoproteins (LDL), high density lipoproteins (HDL)-2 and HDL-3 were isolated by preparative sequential isopicnic centrifugation, and their apolipoprotein and lipidic composition were measured. In the group of severe drinkers, a marked tendency of HDL-3 cholesterol to be higher than in the remaining groups was found. There was no correlation between this parameter and hepatic enzymes. The HDL-2 cholesterol of severe drinkers was significantly increased (p less than 0.005). The parallel enrichment in apolipoproteins C of the HDL-2 particle in all groups of alcohol users suggests a transfer phenomenon from VLDL. The apoA/C ratio of HDL-2 might be useful as a marker of alcohol intake.

[Modifications in the composition of plasma HDLs in non-insulin-dependent diabetics]. / Modificaciones en la composición de las HDL plasmáticas en diabéticos no insulin dependientes.

The levels of plasmatic lipids and the lipo and apoprotein composition of lipoprotein of high density were analysed in 18 patients, diagnosed as having non-insulin dependent diabetes, and compared to a control group of 18 healthy patients. 10 patients showed a moderate hypertriglyceridemia, this sub-group having the main HDL alteration. In this lipoprotein fraction an increase of triglycerides was observed, and a decrease in cholesterol and apoprotein III, probably as result of a lower activity of lipoprotein lipase in plasma.

[Hepatic amyloidosis as cause of severe intrahepatic cholestasis]. / Amiloidosis hepática como causa de colestasis severa intrahepática.

The liver is frequently involved by amyloidosis, but hyperbilirubinemia and liver failure are uncommon features. A mild elevation of the serum alkaline phosphatase value and, less frequently, hepatomegaly are the most common findings. Usually the patients have no symptoms related with the liver involvement; the clinical manifestation and the long term prognosis depends on the renal and cardiac disease. We report an unusual clinical presentation of primary amyloidosis in a previously asymptomatic 65 years old woman who was admitted to the hospital because of ictericia and ascitis mimicking a drug induced acute hepatic failure.

[HIV and schizophrenia]. / HIV y esquizofrenia.

We report five cases of psychosis in patients with antibody to human immunodeficiency virus. All patients was man and intravenous drug abuser. The age range was 22 from 31 years with a mean of 25 years. In all cases acute schizophrenia was the first clinical picture of the HIV. Four patients had opportunistic infections and AIDS-Dementia Complex months later. If there is a genuine biological association between HIV carriage and schizophrenia illness, then HIV infection should be considered in the differential diagnosis of such an illness.

[A 73 year-old patient with thrombocytopenia and a giant cerebral vascular malformation]. / Paciente de 73 años con trombopenia y malformación vascular cerebral gigante.

We report the case of a patient who was seen by purpura and thrombocytopenia. The differential diagnosis in this patient included the presence of cerebral giant vascular malformation, the intake antiepileptic drugs and the presence of a chronic liver disease caused by viral hepatitis C infection. However the final diagnoses of Idiopathic Thrombocytopenia Purpura was made because of the presence of antiplatelets antibodies, the fluctuate evolution, and the increase of platelet count after costicosteroid therapy.

Clinical decisions in patients with diabetes and other cardiovascular risk factors. A statement of the Spanish Society of Internal Medicine.

Although the mortality associated to cardiovascular diseases (CVD) has been reduced in the last decades, CVD remains the main cause of mortality in Spain and they are associated with an important morbidity and a huge economic burden. The increasing prevalence of obesity and diabetes could be slowing down the mortality reduction in Spain. Clinicians have often difficulty making clinical decisions due to the multiple clinical guidelines available. Moreover, in the current context of economic crisis it is critical to promote an efficient use of diagnostic and therapeutic proceedings to ensure the viability of public health care systems. The Spanish Society of Internal Medicine (SEMI) has coordinated a consensus document to answer questions of daily practice with the aim of facilitating physicians' decision-making in the management of diabetes and cardiovascular risk factors from a cost-efficiency point of view.

Prevalence of atherogenic dyslipidemia: association with risk factors and cardiovascular risk in Spanish working population. "ICARIA" study.

OBJECTIVE: The aim of this study was to assess the prevalence of atherogenic dyslipidemia (AD) and the lipid triad (LT) in the working population in Spain, their associated variables and how far they are linked to cardiovascular risk (CVR). METHODS: Observational cross-sectional study of 70,609 workers (71.5% male (M), 28.5% female (F), mean age 39.2 ± 10), who attended medical checkups and agreed to participate. Plasma samples were analysed in a central laboratory. AD definition used was: triglycerides ≥150 mg/dl and HDL cholesterol <40 mg/dl (M)/<50 mg/dl (F) and LT when LDL cholesterol > 160 mg/dl is further added. Univariate comparisons in the absence and presence of AD and LT and the probability of AD according to different parameters and their possible association with CVR were assessed. CVR was stratified following the European SCORE model for low risk-population. RESULTS: 5.7% (95% CI 4.7-6.9) of the working population have AD and 1.1% (95% CI 1.0-1.2) LT. In univariate analysis, workers with AD and LT had a higher prevalence of obesity, hypertension, smoking and diabetes than those who had not (p < 0.001). In multivariate analysis, BMI, sex, age 40-49, diabetes, tobacco, uric acid, LDL or blood pressure significantly influenced the risk of AD. AD was significantly associated with CVR after adjusting for alcohol and obesity. However, most of the AD subjects (91.8%) were classified as low risk. CONCLUSIONS: About 6% of the working population in Spain meets AD criteria. Assuming that these subjects have increased CVR, AD allows to identify additional 5% of subjects with increased CVR to that one the SCORE model detects, helping to improve cardiovascular risk stratification.

Fasting apolipoprotein B48 is a marker for peripheral arterial disease in type 2 diabetes.

An earlier study showed that fasting and postprandial concentrations of apolipoprotein B48 were raised in patients with type 2 diabetes (DM2) and peripheral arterial disease (PAD) as compared with persons without DM2 or persons with DM2 but not PAD. The aim of this study was to confirm the association of PAD and B48 in a larger group of patients with DM2 and the relation of B48 with the preheparin lipoprotein lipase (LPL) mass. We studied 456 patients with DM2. PAD was defined as an ankle-brachial index (ABI) <0.9. Apolipoprotein B48 was quantified by ELISA. Apo B48 was significantly higher in the group with an ABI <0.9 than the groups with ABI of 0.9-1.3 and >1.3 (10.7 ± 6.28 vs. 9.24 ± 5.5 vs. 9.17 ± 8.8 mg/L, ANOVA test, p < 0.05). B48 was independently associated with an ABI <0.9 (OR 1.053; 95 % CI, 1.013-1.094; p < 0.05), together with smoking and duration of diabetes. The preheparin LPL mass was similar in the patients with and without PAD. In conclusion, we confirmed that fasting B48 is an independent marker of PAD in patients with DM2, unrelated to the preheparin LPL mass, statin therapy or glucose lowering treatment.