[Moyamoya disease: clinical, neuroradiological, neuropsychological and genetic perspective]. / Enfermedad de moyamoya: aspectos clinicos, neurorradiologicos, neuropsicolologicos y geneticos.

INTRODUCTION: Moyamoya disease (MMD) is an occlusive cerebrovascular disease characterized by progressive stenosis or occlusion in the terminal portion of the bilateral internal carotid arteries, affecting both children and adults. AIM: To conduct a review and update on MMD from a clinical, neuroradiological, neuropsychological and genetic perspective. DEVELOPMENT: In this pathology, which occurs with ischemia or cerebral hemorrhage, an unusual compensatory vascular network (moyamoya vessels) develops at the base of the brain in the form of collateral channels. MMD can present clinically with hemiparesis, dysarthria, aphasia, headache, seizures, visual deficits, syncopes or changes in personality. At the neuropsychological level, and even in the absence of obvious strokes, patients usually present impairment of attention, memory, behavior and executive functions. High resolution angiography and magnetic resonance imaging have been a neuroradiological advance towards an early detection of this disease. At the genetic level, the RING (really interesting new gene) RNF213 has recently been identified, and it is considered the most important genetic risk factor known up to now in the MMD. CONCLUSIONS: MMD is a rare pathology that predominantly affects the frontal lobes. The genetic and neuroradiological advances, in addition to a cognitive profile, contribute to early diagnosis and treatment to improve the quality of life of these patients.

TITLE: Enfermedad de moyamoya: aspectos clinicos, neurorradiologicos, neuropsicolologicos y geneticos.Introduccion. La enfermedad de moyamoya (EMM) es una enfermedad cerebrovascular oclusiva caracterizada por estenosis progresiva o la oclusion en la porcion terminal de las arterias carotidas internas bilaterales, que afecta tanto a niños como a adultos. Objetivo. Realizar una revision y actualizacion sobre la EMM desde una perspectiva clinica, neurorradiologica, neuropsicologica y genetica. Desarrollo. En esta patologia, que cursa con isquemia o hemorragia cerebral, se desarrolla una red vascular inusual compensatoria (vasos moyamoya) en la base del cerebro en forma de canales colaterales. La EMM puede cursar clinicamente con hemiparesia, disartria, afasia, cefalea, convulsiones, deficits visuales, sincopes o cambios en la personalidad. Neuropsicologicamente, y aun en ausencia de ictus evidentes, los pacientes suelen presentar afectacion de la atencion, memoria, conducta y funciones ejecutivas. La angiografia y la resonancia magnetica de alta resolucion han supuesto un avance neurorradiologico hacia una deteccion precoz de esta enfermedad. Recientemente se ha identificado el RING (really interesting new gene) RNF213, que se considera el factor genetico de riesgo mas importante conocido hasta ahora en la EMM. Conclusiones. La EMM es una patologia rara que afecta de forma predominante a los lobulos frontales. Los avances geneticos y neurorradiologicos, ademas de un perfil cognitivo, contribuyen a un diagnostico y tratamiento tempranos para mejorar la calidad de vida de estos pacientes.

[Neuropsychology of Tourette's disorder: cognition, neuroimaging and creativity]. / Neuropsicologia del trastorno de Tourette: cognicion, neuroimagen y creatividad.

INTRODUCTION: Tourette's disorder is the result of fronto-striatal brain dysfunction affecting people of all ages, with a debut in early childhood and continuing into adolescence and adulthood. DEVELOPMENT: This article reviews the main cognitive, functional neuroimaging and creativity-related studies in a disorder characterized by an excess of dopamine in the brain. CONCLUSIONS: Given the special cerebral configuration of these patients, neuropsychological alterations, especially in executive functions, should be expected. However, the findings are inconclusive and are conditioned by factors such as comorbidity with attention deficit hyperactivity disorder and obsessive-compulsive disorder, age or methodological variables. On the other hand, the neuroimaging studies carried out over the last decade have been able to explain the clinical symptoms of Tourette's disorder patients, with special relevance for the supplementary motor area and the anterior cingulate gyrus. Finally, although there is no linear relationship between excess of dopamine and creativity, the scientific literature emphasizes an association between Tourette's disorder and musical creativity, which could be translated into intervention programs based on music.

TITLE: Neuropsicologia del trastorno de Tourette: cognicion, neuroimagen y creatividad.Introduccion. El trastorno de Tourette es el resultado de una disfuncion cerebral frontoestriatal que afecta a personas de todas las edades, con un inicio en la primera infancia y continuacion en la adolescencia y la adultez. Desarrollo. Este articulo revisa los principales aspectos cognitivos, de neuroimagen funcional y estudios relacionados con la creatividad en un trastorno caracterizado por un exceso de dopamina en el cerebro. Conclusiones. Dada la especial configuracion cerebral de estos pacientes, deberian esperarse alteraciones neuropsicologicas, especialmente en las funciones ejecutivas. Sin embargo, los hallazgos son poco concluyentes y estan condicionados por factores como la comorbilidad con el trastorno por deficit de atencion/hiperactividad y el trastorno obsesivo compulsivo, la edad o variables metodologicas. Por otro lado, los estudios de neuroimagen realizados a lo largo de la ultima decada han podido explicar la sintomatologia clinica de pacientes con trastorno de Tourette, con especial relevancia del area motora suplementaria y el giro cingulado anterior. Finalmente, a pesar de no existir una relacion lineal entre el exceso de dopamina y la creatividad, la literatura cientifica destaca una asociacion entre el trastorno de Tourette y la creatividad musical, lo que podria traducirse en programas de intervencion basados en la musica.

Influence of brine concentration on swelling pressure of pork meat throughout salting.

The influence of sodium chloride on the water holding capacity of meat is well known and described in literature. An increase in water holding capacity is associated with an increase in the swelling pressure in the protein matrix. A novel device was used to measure the swelling pressure during brine salting of muscle cylinders (40×50 mm), in different NaCl brine concentrations (2.5 to 25%) over 6 days at 4°C. Changes in meat composition, weight and height of non-constrained control samples were also measured. The aim of this work was to prove the feasibility of using the developed device in order to quantify the influence of the brine concentration on the magnitude of the swelling pressure. The obtained results indicated the adequacy of the developed device for measuring the meat swelling, while a good correlation of the swelling pressure and the changes in weight and height of the samples was observed.

Physicochemical changes in dry-cured hams salted with potassium, calcium and magnesium chloride as a partial replacement for sodium chloride.

The reduction of added sodium chloride in dry-cured ham has been proposed to reduce dietary sodium intake in Mediterranean countries. The effect of substituting sodium chloride with potassium chloride, calcium chloride and magnesium chloride on some physicochemical characteristics of dry-cured ham during processing was evaluated. The results showed that hams salted with a mixture of sodium and potassium chloride registered higher salt concentrations and lower water contents and thus, needed less time to reach the required weight loss at the end of the process. The opposite effect was observed when calcium and magnesium chloride were added to the salt mixture. The observed differences in the texture and colour parameters were mainly due to differences in water and salt content.

Influence of sodium replacement on physicochemical properties of dry-cured loin.

The consumption of cured meat products is not recommended to hypertensive consumers due to its high sodium content. This constitutes an important restriction for this industry, which is becoming more and more important due to the current trends in consumption. The partial replacement of sodium chloride by potassium chloride has been proposed as a possible strategy to reduce the sodium content of this type of products. The aim of this study was to evaluate the effect brought about by partial replacement of sodium chloride with potassium chloride (up to 70%) on physicochemical and microbiological parameters of dry-cured pork loin after the curing and drying process. The results showed that it is possible to obtain low sodium dry-cured loin, up to a 50% replacement of sodium by potassium, with similar physicochemical characteristics to the commercial product with usual amounts of sodium.

Microbiology and physico-chemical changes of dry-cured ham during the post-salting stage as affected by partial replacement of NaCl by other salts.

Dry-cured ham consumption is restricted by hypertensive consumers due to its high sodium content. This constitutes an important matter for this industry, being relevant due to the current trends in consumption. In order to prevent the problems related to the high sodium intake, one of the possibilities is the total or partial replacement of sodium by other ions, such as potassium, calcium and magnesium. The aim of this study was to characterise the post-salting stage in Spanish cured ham production with the results obtained after salting with low sodium salt content. The results showed that lower sodium hams needed more time of post-salting to reach similar water activity values than those achieved by hams salted with 100% NaCl. Nevertheless, no differences in microbial counts were observed among the studied batches, although a sharp decrease in microbiota was observed when the, post-salting time was prolonged in the lower sodium hams.