Diagnostic ability of multifocal electroretinogram in early multiple sclerosis using a new signal analysis method

Purpose To determine if a novel analysis method will increase the diagnostic value of the multifocal electroretinogram (mfERG) in diagnosing early-stage multiple sclerosis (MS). Methods We studied the mfERG signals of OD (Oculus Dexter) eyes of fifteen patients diagnosed with early-stage MS (in all cases < 12 months) and without a history of optic neuritis (ON) (F: M = 11:4), and those of six controls (F:M = 3:3). We obtained values of amplitude and latency of N1 and P1 waves, and a method to assess normalized root-mean-square error (FNRMSE) between model signals and mfERG recordings was used. Responses of each eye were analysed at a global level, and by rings, quadrants and hemispheres. AUC (area under the ROC curve) is used as discriminant factor. Results The standard method of analysis obtains further discrimination between controls and MS in ring R3 (AUC = 0.82), analysing N1 waves amplitudes. In all of the retina analysis regions, FNRMSE value shows a greater discriminating power than the standard method. The highest AUC value (AUC = 0.91) was in the superior temporal quadrant. Conclusion By analysing mfERG recordings and contrasting them with those of healthy controls it is possible to detect early-stage MS in patients without a previous history of ON

Hepatic disease as the first manifestation of progressive myoclonus epilepsy of Lafora

5 páginas, 2 figuras -- PAGS nros. 1369-1373Background: Lafora disease (LD; progressive myoclonus epilepsy type 2; EPM2) is an autosomal recessive disorder caused by mutations in the EPM2A and EPM2B genes. LD is characterized by the presence of strongly PAS-positive intracellular inclusions (Lafora bodies) in several tissues. Glycogen storage disease type IV (GSD-IV; Andersen disease) is an autosomal recessive disorder characterized by cirrhosis leading to severe liver failure. GSD-IV has been associated with mutations in the glycogen branching enzyme gene (GBE). Histopathologic changes of the liver in both diseases show an identical appearance, although cirrhosis has never been described in patients with LD. We report a LD family in which the proband presented severe liver failure at onset of the disease. Methods: Clinical histories, physical and neurologic examination, laboratory tests, EEGs, MRI of the brain, and liver or axillary skin biopsies were performed in the two affected siblings. The diagnosis was confirmed by molecular genetic analysis of the EPM2A, EPM2B, and GBE genes and loci. Results: During the first decade of life, abnormalities in liver function tests were detected in the two affected siblings. The proband's liver dysfunction was severe enough to require liver transplantation. Subsequently, both sibs developed LD. Mutation analysis of EPM2A revealed a homozygous Arg241stop mutation in both patients. Conclusions: This is the first description of severe hepatic dysfunction as the initial clinical manifestation of LD. The phenotypic differences between the two affected siblings suggest that modifier genes must condition clinical expression of the disease outside the CNSPeer reviewe

Effects of trichothecene production by Trichoderma arundinaceum isolates from bean-field soils on the defense response, growth and development of bean plants (Phaseolus vulgaris)

[EN] The trichothecene toxin-producing fungus Trichoderma arundinaceum has potential as a biological control agent. However, most biocontrol studies have focused only on one strain, IBT 40837. In the current study, three Trichoderma isolates recovered from bean-field soils produced the trichothecene harzianum A (HA) and trichodermol, the latter being an intermediate in the HA biosynthesis. Based on phylogenetic analysis, the three isolates were assigned to the species T. arundinaceum. Their genome sequences had a high degree of similarity to the reference IBT 40837 strain, in terms of total genome size, number of predicted genes, and diversity of putative secondary metabolite biosynthetic gene clusters. HA production by these bean-field isolates conferred significant in vitro antifungal activity against Rhizoctonia solani and Sclerotinia sclerotiorum, which are some of the most important bean pathogens. Furthermore, the bean-field isolates stimulated germination of bean seeds and subsequent growth of above ground parts of the bean plant. Transcriptomic analysis of bean plants inoculated with these T. arundinaceum bean-field soil isolates indicated that HA production significantly affected expression of plant defense-related genes; this effect was particularly significant in the expression of chitinase-encoding genes. Together, these results indicate that Trichoderma species producing non-phytotoxic trichothecenes can induce defenses in plants without negatively affecting germination and developmentSIThis work is a part of the Spanish I+D+i Grants RTI2018-099600-B-I00 and PID2021-123874OB-I00, financed by the MCIN/ AEI/10.13039/501100011033. GC-H was awarded with a Grant from the Ministry of Education, Culture, and Sport (Spain) (Grant number FPU15/04681). NM-R was awarded with a Grant from the Junta de Castilla y León (Spain) (ORDEN EDU/875/2021, July 13th, 2021

Bluefin tuna (Thunnus thynnus) tagging survey in the Bay of Biscay in summer 2005

A brief summary of the conventional tagging survey carried out during the summer fishing season in 2005 in the Bay of Biscay is presented. A total of 1696 juveniles of bluefin tuna have been tagged, corresponding to group ages 1 and 2. Two different conventional tags have been used. Fifty-one recaptures have been recovered up to date.Le présent document fournit un résumé de la campagne de marquage conventionnel de thon rouge menée au cours de l’été 2005 dans le Golfe de Gascogne. Au total, 1.696 juvéniles de thon rouge ont été marqués, correspondant aux groupes d’âge 1 et 2. Deux types distincts de marques conventionnelles ont été utilisés. Cinquante-et-une marques ont été récupérées à ce jour.Se presenta un resumen de la campaña de marcado convencional de atún rojo llevada a cabo durante el verano del 2005 en el Golfo de Vizcaya. En total se han marcado 1696 ejemplares de 1 y 2 años de edad. Se han utilizado dos modelos distintos de marcas convencionales. Hasta la fecha se han recapturado cincuenta y un ejemplares

Bisutería a base de fruta deshidratada elaborada artesanalmente

El presente trabajo analiza el mercado de la joyería artesanal en la Región de Tuxpan, Ver., con el fin de ofrecer una visión global y orientar a los emprendedores que se encuentran en el proceso de comercialización de bisutería artesanal obteniendo un conocimiento previo, intentando conseguir el éxito en sus proyectos. El sector de la bisutería en México ha experimentado profundos cambios con la llegada masiva de productos asiáticos al mercado, esto, debido a que estos son de muy bajo costo y lo que ha influido de manera radical en la producción local, impactando directamente en el volumen de la comercialización nacional y a las exportaciones, provocando caídas constantes en ambas cifras. Sin embargo, las importaciones crecen cada año desde 2002, México, tradicionalmente es joyero, donde los principales centros de producción están ubicados en Guadalajara y México, DF. La bisutería es una joyería artesanal y tradicional, que es consumida, no tan solo por mujeres, sin no también por hombres. La demanda de bisutería ha crecido y mantiene su tendencia de crecimiento, debido a que es un producto que permite seguir las tendencias de la moda de una manera más económica  y segura que la joyería. El motivo que impulsa a la compra de bisutería es más emocional que racional, y su asociación con las tendencias cambiantes de la moda hace que el ciclo del producto sea más corto