Temblor esencial: aspectos clínicos, diagnósticos y terapéuticos.

Revisión bibliográfica acerca del temblor esencial. Se actualizan aspectos genéticos, fisiopatológicos, clínicos, diagnósticos y terapéuticos de este trastorno, haciendo especial hincapié en las alternativas quirúrgicas de tratamiento.<br /

Determinación de sales solubles y eflorescencias en tejas cerámicas

The appearance of efflorescences constitutes a serious problem for ceramic roofing tile quality, since efflorescences affect roofing tile technical and aesthetic qualities. This paper describes a method of determining the soluble salts present in the raw materials used in ceramic roofing tile manufacture and in the resulting roofing tiles, as well as an analysis of the phases in roofing tile efflorescences. The study was divided into three parts. First, a method of determining the soluble salts was developed, optimising the extraction method and fine-tuning an anion measurement method by ionic chromatography. Secondly, the changes in solubility, as well as the chemical and structural changes that occurred when the raw materials were subjected to firing, were studied. Finally, the efflorescences of a group of unfired and fired ceramic roofing tiles were characterised, in order to relate them to the quantity and type of soluble salts contained. The study concludes that it is necessary to conduct solubility tests on fired samples under real conditions, since important structural changes occur in the firing process, which lead to considerable variations in the soluble salts. Furthermore, in addition to analysing Na(I), K(I), and Mg(II) as indicated in the standard, it is advisable to determine Ca(II) and SO42-, since these are the major components in the efflorescences.La aparición de eflorescencias constituye un serio problema para la calidad de las tejas cerámicas ya que afectan a las características técnicas y estéticas de éstas. En este trabajo se ha realizado un estudio del método de determinación de las sales solubles presentes en materias primas y en tejas cerámicas, así como un análisis de fases de las eflorescencias halladas. El trabajo se ha dividido en tres fases. Primero, se ha desarrollado un método de determinación de las sales solubles, optimizando el método de extracción y poniendo a punto un método de medida de los aniones mediante cromatografía iónica. Seguidamente, se han estudiado los cambios de solubilidad, químicos y estructurales que ocurren al someter los materiales al proceso de cocción. Finalmente, se han caracterizado las eflorescencias de un conjunto de tejas crudas y cocidas para relacionarlas con el contenido y tipo de sales solubles presentes. Del estudio se concluye que es necesario realizar los ensayos de solubilidad sobre las muestras cocidas en condiciones reales ya que en el proceso de cocción ocurren importantes cambios estructurales que conllevan una considerable variación de las sales solubles; y que además de analizar Na+, K+ y Mg2+, como se indica en la norma, se aconseja determinar Ca2+ y SO 2-, por ser los componentes mayoritarios de las eflorescencias

Episodic Vestibulocerebellar Ataxia Associated with a CACNA1G Missense Variant

Episodic vestibulocerebellar ataxias are rare diseases, frequently linked to mutations in different ion channels. Our objective in this work was to describe a kindred with episodic vestibular dysfunction and ataxia, associated with a novel CACNA1G variant. Two individuals from successive generations developed episodes of transient dizziness, gait unsteadiness, a sensation of fall triggered by head movements, headache, and cheek numbness. These were suppressed by carbamazepine (CBZ) administration in the proband, although acetazolamide and topiramate worsened instability, and amitriptyline and flunarizine did not prevent headache spells. On examination, the horizontal head impulse test (HIT) yielded saccadic responses bilaterally and was accompanied by cerebellar signs. Two additional family members were asymptomatic, with normal neurological examinations. Reduced vestibulo-ocular reflex gain values, overt and covert saccades were shown by video-assisted HIT in affected subjects. Hearing acuity was normal. Whole-exome sequencing demonstrated the heterozygous CACNA1G missense variant c.6958G&#x3e;T (p.Gly2320Cys) in symptomatic individuals. It was absent in 1 unaffected member (not tested in the other asymptomatic individual) and should be considered likely pathogenic. CACNA1G encodes for the pore-forming, α1G subunit of the T-type voltage-gated calcium channel (VGCC), in which currents are transient owing to fast inactivation, and tiny, due to small conductance. Mutations in CACNA1G cause generalized absence epilepsy and adult-onset, dominantly inherited, spinocerebellar ataxia type 42. In this kindred, the aforementioned CACNA1G variant segregated with disease, which was consistent with episodic vestibulocerebellar ataxia. CBZ proved successful in bout prevention and provided symptomatic benefit in the proband, probably as a result of interaction of this drug with VGCC. Further studies are needed to fully determine the vestibular and neurological manifestations of this form of episodic vestibulocerebellar ataxia. This novel disease variant could be designated episodic vestibulocerebellar ataxia type 10

Hereditary primary lateral sclerosis and progressive nonfluent aphasia

Objective: to report a kindred with an association between hereditary primary lateral sclerosis (PLS) and progressive nonfluent aphasia. Patients and methods: six members from a kindred with 15 affected individuals spanning three generations, suffered from spasticity without muscle atrophy or fasciculation, starting in the lower limbs and spreading to the upper limbs and bulbar musculature, followed by effortful speech, nonfluent language and dementia, in 5 deceased members. Disease onset was during the sixth decade of life, or later. Cerebellar ataxia was the inaugural manifestation in two patients, and parkinsonism, in another. Results: neuropathological examination in two patients demonstrated degeneration of lateral corticospinal tracts in the spinal cord, without loss of spinal, brainstem, or cerebral motor neurons. Greater loss of corticospinal fibers at sacral and lumbar, rather than at cervical or medullary levels was demonstrated, supporting a central axonal dying-back pathogenic mechanism. Marked reduction of myelin and nerve fibers in the frontal lobes was also present. Argyrophilic grain disease and primary age-related tauopathy were found in one case each, and considered incidental findings. Genetic testing, including exome sequencing aimed at PLS, ataxia, hereditary spastic paraplegia, and frontotemporal lobe dementia, triplet-repeated primed polymerase chain reaction aimed at dominant spinocerebellar ataxias, and massive sequencing of the human genome, yielded negative results. Conclusion: a central distal axonopathy affecting the corticospinal tract, exerted a pathogenic role in the dominantly inherited PLS-progressive nonfluent aphasia association, described herein. Further molecular studies are needed to identify the causative mutation in this disease

Introducing life cycle thinking to define best available techniques for products: Application to the anchovy canning industry

This study presents a method based on life cycle assessment to reduce and simplify the decision-making process and to identify the best available techniques of a product. This procedure facilitates the selection of a technical alternative from an environmental point of view and the reduction of emission levels and the consumption of energy and primary resources. This method comprises the following four steps: (i) the identification of the current techniques of a specific product, (ii) the application of a life cycle assessment to determine the hot spots, (iii) the proposal of the best available techniques and (iv) the development of a best available techniques reference document (step not implemented in our case study). The Cantabrian anchovy canning industry is selected as a case study due to the importance of this sector from economic, social and touristic points of view. An entire life cycle assessment of one can of anchovies in extra virgin olive oil is conducted. The results indicated that the hot spots of the life cycle were the production of aluminium cans (for packaging) and extra virgin olive oil and the management of the packaging waste. According to these results, the study proposes several improvements, such as packaging recycling and several best available techniques for the canned anchovy product.The authors thank the Ministry of Economy and Competitiveness of the Spanish Government for their financial support via the project GeSAC-Conserva: Sustainable Management of the Cantabrian Anchovies (CTM2013-43539-R) and to Julia Celaya for her technical support. Jara Laso thanks the Ministry of Economy and Competitiveness of Spanish Government for their financial support via the research fellowship BES2014-069368. Pere Fullana y Alba Bala thanks the UNESCO Chair in Life Cycle and Climate Change

Finding the best available techniques for an environmental sustainable waste management in the fish canned industry

This work proposes the use of the life cycle assessment (LCA) to identify the best available techniques (BAT) to the management of the residues generated in the anchovy canning sector. This industry generates huge amount of solid and liquid wastes, and their management is one of the hotspots of the canned anchovy life cycle. The application of BATs can improve the environmental performance of the canned anchovy. However, sometimes it is not clear which BAT is the most appropriated, and an environmental analysis is required. In this sense, several BATs are proposed based on the circular economy concept, which promotes the reutilisation of wastes and they were evaluated under a life cycle approach: (i) valorisation of the anchovy residues into fishmeal, fish oil and anchovy paste, (ii) incineration and (iii) disposal in a municipal solid waste landfill. The LCA was conducted from cradle to gate using the global warming (GW) indicator. The results showed that the disposal of the anchovy residues in a landfill was the least environmental-friendly option, while the valorisation was the best alternative.Authors thank to Ministry of Economy and Competitiveness of Spanish Government for the financial support through the project called GeSAC-Conserva: Sustainable Management of the Cantabrian Anchovies (CTM2013-43539-R). Jara Laso also thanks to the Ministry of Economy and Competitiveness of Spanish Government for the financial support through the research fellowship BES-2014-069368

Treatment of cervical cancer with electronic brachytherapy

Purpose: We report the first cervical cancer cases treated with interstitial electronic brachytherapy (eBT) at our hospital and compare them with plans made with high-dose-rate interstitial brachytherapy based on Ir192 (HDR-BT). Materials and methods: Eight patients with cervical cancer were treated with the Axxent eBT device (Xoft, Inc.). Planning was with magnetic resonance imaging and computed tomography following the recommendations of the EMBRACE protocol. The dosimetry parameters of organs at risk (OAR) were evaluated for the bladder, rectum, and sigmoid colon (D2cc, D1cc, and D0.1cc). In addition, the V150 and V200 of irradiated tissue were compared for both eBT and HDR-BT. All patients received intensity-modulated external beam radiation therapy with a regimen of 23 sessions of 2 Gy followed by four sessions of 7 Gy of eBT performed over 2 weeks (two sessions followed by another two sessions a week later) following the EMBRACE recommendations. Each of the eight patients was followed to assess acute toxicity associated with treatment. Results: The doses reaching OAR for eBT plans were lower than for HDR-BT plans. As for acute toxicity associated with eBT, very few cases of mucositis were detected. No cases of rectal toxicity and one case with grade 1 urinary toxicity were detected. The results at 1 month are equally good, and no relapses have occurred to date. Conclusions: The first results of treatment with the Axxent eBT device are promising, as no recurrences have been observed and toxicity is very low. eBT is a good alternative for treating cervical cancer in centers without access to conventional HDR

Incorporating linear programing and life cycle thinking into environmental sustainability decision-making: a case study on anchovy canning industry

Life cycle assessment (LCA) is a powerful tool to support environmental informed decisions among product and process alternatives. LCA results reflect the process stage contributions to several environmental impacts, which should be made mutually comparable to help in the decision-making process. Aggregated environmental indexes enable the translation of this set of metrics into a one final score, by defining the attached weights to impacts. Weighting values reflect the corresponding relevance assigned to each environmental impact. Current weighing schemes are based on pre-articulation of preferences, without considering the specific features of the system under study. This paper presents a methodology that combines LCA methodology and linear programming optimisation to determine the environmental improvement actions that conduct to a more sustainable production. LCA was applied using the environmental sustainability assessment methodology to obtain two main indexes: natural resources (NR) and environmental burdens (EB). Normalised indexes were optimised to determine the optimal joint of weighting factors that lead to an optimised global Environmental Sustainability Index. The proposed methodology was applied to a food sector, in particular, to the anchovy canning industry in Cantabria Region (Northern Spain). By maximising the objective function composed of NR and EB variables, it is possible to find the optimal joint of weights that identify the best environmental sustainable options. This study proves that LCA can be applied in combination with linear programing tools as a part of the decision-making process in the development of more sustainable processes and products.Authors thank to Ministry of Economy and Competitiveness of Spanish Government for the financial support through the project GeSAC-Conserva (CTM2013-43539-R). Jara Laso also thanks to the Ministry of Economy and Competitiveness of Spanish Government for the financial support through the research fellowship BES-2014-069368

Combined application of Life Cycle Assessment and linear programming to evaluate food waste-to-food strategies: Seeking for answers in the nexus approach

The great concern regarding food loss (FL) has been studied previously, but in an isolated way, disregarding interdependencies with other areas. This paper aims to go a step further by proposing a new procedure to assess different waste management alternatives based on the nexus approach by means of an integrated Water-Energy-Food-Climate Nexus Index (WEFCNI). The environmental profile of the waste management techniques is determined using Life Cycle Assessment (LCA) which, in combination with Linear Programming (LP), explores the optimal aggregation of weighting factors that lead to an aggregated nexus index. The management of residues from the anchovy canning industry in Cantabria (Spain) has been used as a case study, considering the three current applied alternatives: (i) valorisation of FL as animal feed in aquaculture (food waste-to-food approach), (ii) incineration of FL with energy recovery, and (iii) landfilling with biogas recovery. The last two considered the use of energy recovered to produce a new aquaculture product (food waste-to-energy-to-food scenarios). The results indicate that incineration is the best performing scenario when the nutritional energy provided by the valorisation alternative is not high enough and the valorisation technology presents the highest water consumption. Therefore, a minimisation in the consumption of natural resources is suggested in order to improve the application of circular economy within the sector. The use of the nexus index as an environmental management tool is extendable to any food system with the aim of facilitating the decision-making process in the development of more sustainable products.The authors thank the Ministry of Economy and Competitiveness of the Spanish Government for its financial support via the project GeSAC-Conserva: Sustainable Management of the Cantabrian Anchovies (CTM2013-43539-R) and to Julia Celaya for her technical support. Jara Laso thanks the Ministry of Economy and Competitiveness of Spanish Government for its financial support via the research fellowship BES-2014-069368. Pere Fullana and Alba Bala thank the UNESCO Chair in Life Cycle and Climate Change. Ian Vázquez-Rowe thanks the Pontificia Universidad Católica del Perú for financing the Walaya Project