Cognitive impairment in myotonic dystrophy type 1 (Steinert's disease)

Traditionally, it has been recognized that patients with myotonic dystrophy type 1 (MD-1) – also known as Steinert disease –, they show a specific behaviour, not including those who suffer from mental or neurodevelopmental diseases. The neurological substrate of this behaviour is described. The aim of this text has two purposes. The first intention is that clinical staff, when faced with a patient with MD-1, always consider the cognitive aspects of the disease. On the other hand, it is intended to combat preconceived ideas about the particular behaviour of these patients.Tradicionalmente se ha reconocido que los enfermos con distrofia miotónica tipo 1 (DM-1), o enfermedad de Steinert, presentan una conducta muy característica, dejando aparte aquellos casos en que existe patología mental o un retraso del neurodesarrollo. Se describe el sustrato neurológico de dicha conducta. El objetivo de este texto es doble. Por una parte, se pretende que los clínicos, ante un paciente con DM-1, consideren siempre los aspectos cognitivos de la enfermedad. Por otra, se busca desmontar ideas preconcebidas sobre el peculiar comportamiento de estos pacientes

Aromatic poly(ether ether ketone)s capable of crosslinking via UV irradiation to improve gas separation performance

The effect of UV-crosslinking on the gas transport properties of two poly(ether ether ketone)s derived from difluorobenzophenone and two bisphenol derivatives, with four (TMBP-DFB) or six (HMBP-DFB) methyl groups, has been studied. The crosslinking reaction was conducted on dense membranes, using polychromatic light, with wavelengths higher than 350 nm, at room temperature and in presence of air. Both polymers were able to produce crosslinked membranes, with gel fractions close to 75%, but a shorter irradiation time was required for HMBP-DFB. A DFT quantum mechanical study has stated that HMBP-DFB radical formation is much easier than for TMBP-DFB, which would support the fastest kinetics of the crosslinking process for HMBP-DFB. The crosslinked membranes have shown greatly improved gas transport properties, especially for the O2/N2 gas pair, where the Robeson upper bound line of 1991 was clearly surpassed. The improvement in selectivity has been ascribed to the better molecular-sieving characteristics of crosslinked membranesWe acknowledge the Financial support provided by MINECO (MAT2013-45071-R, CTQ2012-31076 and MAT2016-76413-C2- R2) and by the National Science Foundation under grant number IIP-1237857. Authors wish to thank Judit González for helping with the characterization of polymersPeer Reviewe

Spatial and temporal variability of periglaciation of the Iberian Peninsula

Active periglacial processes are currently marginal in the Iberian Peninsula, spatially limited to the highest mountain ranges. However, a wide variety of periglacial deposits and landforms are distributed in low and mid-altitude environments, which shows evidence of past periods of enhanced periglacial activity. The purpose of this paper is to summarize the present knowledge of past periglacial activity in the Iberian Peninsula. The chronological framework takes four main stages into account: the last glaciation, deglaciation, Holocene and present-day processes. This study focuses on the highest massifs (Pyrenees, Cantabrian Range, NW ranges, Central Range, Iberian Range, Sierra Nevada) as well as other lower elevation environments, namely the central Iberian Meseta. During the last glaciation the periglacial belt extended to much lower altitudes than today, reaching current sea level in the NW corner of the Iberian Peninsula. A wide range of geomorphological landforms and sedimentary records is indicative of very active periglacial processes during that phase, in some cases related to permafrost conditions (i.e., block streams, rock glaciers). Most of the inactive landforms and deposits in low and mid-elevations in Iberia are also related to this phase. The massive deglaciation of the Iberian massifs was caused by a gradual increase in temperatures. The deglaciation phase was only interrupted by a short period with colder conditions (the Younger Dryas) that reactivated periglacial processes in the formerly glaciated cirques of the highest lands, specifically with the widespread development of rock glaciers. During the Holocene, periglacial processes have been only active in the highest ranges, shifting in altitude according to temperature regimes and moisture conditions. The Little Ice Age saw the reactivation of periglacial activity in lower elevations than today. Currently, periglacial processes are only active in elevations exceeding 2500 m in the southern ranges and above 2000e2200 m in the northern massifs, higher in Sierra Nevada, in the south of Iberian Peninsula.info:eu-repo/semantics/publishedVersio