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Introduction: Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), is a neurodegenerative autosomal recessive disease caused by TPP1 gene variants, with a spectrum of classic and atypical phenotypes. The aim of treatment is to slow functional decline as early as possible, improving quality of life and survival. This study describes the clinical characteristics as well as response to treatment with cerliponase alfa. Materials and Methods: A retrospective study was conducted in five Latin-American countries, based on clinical records from patients with CLN2. Clinical follow-up and treatment variables are described. A descriptive and bivariate statistical analysis was performed. Results: A total of 36 patients are reported with a mean age of symptom onset and time to first specialized consultation of 4.7±2.3 and 6±3.1 years, respectively. Seizures were the predominant symptom (80.6%). In a subgroup analysis, most patients with the classic phenotype exhibited regression in language (90%), while the patients with the atypical phenotype had seizures as the predominant symptom (87%). The mean age of symptom onset and time to first specialized consultation was 3.5±2.0 and 4.9±3.2 years, respectively, in patients with the classic phenotype and 6.2±1.8 and 7.5±2.4 in patients with the atypical phenotype. The mean time interval between onset of symptoms and treatment initiation was 3.8 years in patients with classic phenotype and 7.4 in patients with atypical phenotype. All patients were treated with cerliponase alfa, maintaining overall functional stability as compared to pretreatment values. Discussion and conclusion: This study reports at this time the largest number of patients with CLN2 in treatment with cerliponase alfa in the world. Data show a higher frequency of patients with atypical phenotype and a high allelic proportion of intron variants in our region. There was evidence of long intervals until first specialized consultation, diagnosis, and enzyme replacement therapy. Follow-up after the initiation of cerliponase alfa showed slower-to-no-progression of the disease, associated with adequate clinical outcomes and stable functional scores. These improvements were consistent in both clinical phenotypes.

The 5th meeting of the IUGS Lower Cretaceous Ammonite Working Group (the Kilian Group) held in Ankara, Turkey, 31st August 2013, discussed the Mediterranean ammonite zonation, and its calibration with different ammonite zonal schemes of the Boreal, Austral and Central Atlantic realms. Concerning the standard zonation, that corresponds to the zonal scheme of the West Mediterranean province, some changes have been made on two stages. For the Valanginian, the Busnardoites campylotoxus Zone was abandoned; the upper part of the lower Valanginian is now characterised by the Neocomites neocomiensiformis and Karakaschiceras inostranzewi zones. For the upper Barremian, the former Imerites giraudi Zone is here subdivided into two zones, a lower I. giraudi Zone and an upper Martellites sarasini Zone. The I. giraudi Zone is now subdivided into the I. giraudi and Heteroceras emerici subzones, previously considered as horizons. The current M. sarasini and Pseudocrioceras waagenoides subzones correspond to the lower and upper parts of the M. sarasini Zone, respectively. The Anglesites puzosianum Horizon is kept. The Berriasian, Hauterivian, Aptian and Albian zonal schemes have been discussed but no change was made. The upper Hauterivian zonal scheme of the Georgian (Caucasus) region (East Mediterranean province) has been compared with the standard zonation. Discussions and some attempts at correlations are presented here between the standard zonation and the zonal schemes of different

This paper presents a solution for the inverse kinematics in a human arm kinematics model of seven degrees of freedom applying Genetic Algorithms in the shoulder joint and the Piper method in the whole arm in order to make the algorithm response faster and accurate. The Genetic Algorithm's code explores a set of initial solutions and optimizing it until find an angular response that satisfies the positional and rotational restrictions proposed to the model with a minimal error and a considerably speed. The accuracy and reliability of the applied algorithm is evaluated applying a statistical analysis upon a set of answers using SPSS Statistics.

Sectorization is a process aimed at efficiently managing Water Supply Networks (WSNs). There are many large WSNs where the number of sectors into which they might be subdivided is initially unknown. Our method uses concepts derived from the Social Network Theory (SNT) such as centrality and community detection to obtain such subdivisions. The output is then evaluated through energy criteria. This methodology entails two very important benefits over other methods previously proposed. Firstly, it is applicable to WSNs depending on a network trunk and, secondly, it considers the technical know-how of the staff of the water utility.

A novel methodology to sectorize water supply networks (WSNs) depending on a main transmission line is presented in this paper. The methodology is based on concepts derived from the social network theory and graph theory (namely, community detection and shortest path respectively); and also on a multi-objective optimization process by means of agent swarm optimization (ASO). A series of energy, operative, and economic criteria are optimized in this process. The core idea is to form sectors over the distribution network based on communities found using a community detection algorithm (Walktrap). The methodology is flexible and enables the technical staff in water utilities to make decisions at different stages. It has been tested by generating four feasible solutions over a portion of a real WSN.

This study summarizes the final results of magnetostratigraphic and biostratigraphic investigations of the Tithonian/Berriasian (J/K) boundary limestones at the locality of Puerto Escano, Spain. The aim is to prepare the background for correlation of an upper Tithonian and lower Berriasian biostratigraphic zonation with global magnetoevents (manifested in detailed magnetostratigraphic profiles) between the Tethyan and the Boreal realms. Magnetostratigraphic studies were applied to an 8.1-m-thick part of the section embracing upper Tithonian and lower Berriasian strata. The average sampling interval was 30 mm. The analysis of the IRM acquisition curves proved the presence of magnetite and hematite, the former mineral being the main carrier of the remanent magnetization. Progressive thermal demagnetization mostly revealed three NRM components, and magnetostratigraphy was based on the directions of the most stable of them, with unblocking temperature varying mostly from 300 to 540 °C. Due to almost parallel beds, the fold test applied to this component did not give convincing results. In contrast, the reversal test received the best classification ‘A’. The detected polarity zones could has been unequivocally identified against the M-sequence of polarity intervals drawn from the Geomagnetic Polarity Time Scale 2004. This fact, together with the results of the reversal test, confirmed the ChRM to be the primary component. The sampled part of the section included a part of magnetozone M20r, full magnetozones M20n to M18r and a part of magnetozone M18n. Especially the detection of two reverse respectively by M20n.1r and M19n.1r with thicknesses only 40 and 90 mm, respectively, required much effort when sampling the section. The calculated sedimentation rate varied from 1 to 5 mm/ky. The positions of the individual events of tintinnoid biostratigraphy (mainly calpionellids) relative to the global magnetic polarity timescale are precisely defined. The base of the Calpionella Standard Zone, which is considered to be a potential J/K boundary indicator in ammonite-free sections from the Tethyan realm, or in sections where calpionellid stratigraphy applies, lies within magnetozone M19n at the level of 35% of its local thickness. None of the boundaries in the calpionellid zonation coincides precisely with any of those in the palaeomagnetic zonation, but the first appearance datum (FAD) of Calpionella grandalpina Nagy, indicating the base of the Intermedia Subzone, lies in close proximity to the base of magnetozone M19r. The last appearance datum (LAD) for Praetintinnopsella andrusovi Borza in Bed 14A corresponds approximately to the base of the Kysuca Subzone.

The discussion is mainly focused on the Hauterivian and Barremian zonal schemes. For the Hauterivian Stage, the main changes concern the Subsaynella sayni and Balearites balearis Zones with the introduction of one horizon (Subsaynella begudensis Horizon) and four subzones (B. balearis, Binelliceras binelli, Crioceratites krenkeli, Spathicrioceras seitzi subzones), respectively. For the Lower Barremian, the main amendments concern the introduction of two subzones in the Taveraidiscus hugii auctorum Zone (T. hugii auctorum and Psilotissotia colombiana subzones) and of four horizons in the Kotetishvilia compressissima Zone (Holcodiscus fallax, Nicklesia didayana, Heinzia communis and Subtorcapella defayae Horizons). The Coronites darsi Zone is replaced by the Moutoniceras moutonianum Zone in which two horizons occur (C. darsi and Heinzia caicedi Horizons). The base of the Upper Barremian is defined by the base of the Toxancyloceras vandenheckii Zone and Subzone. The former Hemihoplites feraudianus and Martelites sarasini Zones are lowered in rank to subzones of the Gerhardtia sartousiana and Imerites giraudi Zones, respectively.

An analysis of the stratigraphic distribution of ammonite faunas collected from several sections in the Essaouira-Agadir area (western High Atlas, Morocco) has enabled the establishment of a detailed succession of assemblages for the uppermost Hauterivian to lower Upper Barremian interval. All Mediterranean standard zones of this interval have been identified. The correlation potential of bioevents which define and characterise these units is discussed. Two significant regional hiatuses, one at the Hauterivian/Barremian boundary, the other involving the lower part of the Kotetishvilia nicklesi Zone, are related to third-order sea level falls. Moreover, the upper part of the succession corresponding to the upper Toxancyloceras vandenheckii Zone and the entire Heinzia sartousiana Zone is markedly condensed. The taxonomic composition of the ammonite assemblage reveals a strong Mediterranean character, yet, it shows some peculiar features as well.

Introduction: A computer model was developed to estimate the arterial pressure and the cardiac output of humans, by adapting the equations of the Windkessel theory. Material and Methods: The model inputs are based on certain characteristics of patients, such as age, the use of specific drugs and smoking habits. It also predicts the effect of smoking on any of the constituent variables of arterial impedance (Compliance and Resistance) and establishes a statistically significant relationship between them. Results: The outputs of the model are strongly correlated with physiological observations, with a minimum error of 3% for the arterial pressure. Conclusion: The proposed model is useful for providing an easy and low cost estimation of arterial pressure.

A conspicuous renewal in the ammonite faunas of the Mediterranean Tethys occurred in the latest Hauterivian. This faunal turnover took place following a stepwise pattern. The first step occurred at the boundary between the Pseudothurmannia ohmi Subzone and the Pseudothurmannia mortilleti Subzone, coinciding with the base of the so-called Faraoni Level. This is a Corgrich interval that has been recognised in several basins of the Mediterranean Tethys and seems to be the expression of a shortlived oxygen-deficient event. It correlates with a well-documented second-order peak transgression. The oxygen depletion preferentially affected the deep nektic ammonites, which would explain the extinctions within this group around the Faraoni Level. On the contrary, an increase in the trophic resources in the photic zone favoured the diversification of epipelagic ammonites. Concurrently, an abrupt change took place at this level in the nannoconid assemblage composition. A minor second event, located at the base of the Pseudothurmannia picteti Subzone, was marked by the replacement of a few planktic ammonite species by closely related forms, and the structure of the ammonite assemblage was not substantially altered. The coincidence of this event with a further restructuring of the calcareous nannofossil assemblage suggests that some changes had to occur in the planktic ecosystem during the sea-level highstand subsequent to the peak transgression. The third and last stage of the renewal process started in the upper part of the P. picteti Subzone, coinciding with a drastic sea-level fall. It is characterised by the extinction of many of the species that had appeared in the two previous events, resulting in an extensive modification of the assemblage structure. The regression would probably cause a drop in the primary productivity and, consequently, an improvement in the oxygenation level of the sea bottom. This would explain the extinction of several planktic ammonite species and the appearance of new nektic and nektobenthic lineages. D 2005 Elsevier B.V. All rights reserved.

A detailed analysis of the stratigraphic distribution of ammonites from fourteen sections in the External Zones of the Betic Cordillera has enabled us to identify accurately the sequence of bioevents that take place throughout the uppermost Hauterivian and to propose a more precise biostratigraphic scheme for this interval. Thus, we have divided the two currently admitted zones (the Crioceratites balearis Zone and the Pseudothurmannia angulicostata auct. Zone) into several subzones. Four subzones can be recognised within the Cr. balearis Zone. The base of each subzone is defined by the first occurrence of one of four successive species belonging to the same Crioceratites lineage. These species are: Cr. balearis, Cr. binelli, Cr. krenkeli and Cr. angulicostatus . The Ps. ohmi Zone (which replaces the classical Ps. angulicostata Zone) can be divided into three subzones characterised by three successive species of the genus Pseudothurmannia: Ps. ohmi, Ps mortilleti (here considered as a senior synonym of Ps. catulloi ) and Ps. picteti . According to the current definition, the first occurrence of Taveraidiscus hugii (OOSTER) marks the lower boundary of the Barremian stage. This event fits into a major faunal-renewal episode that begins in the upper part of the Ps. picteti Subzone.

The study of ammonite assemblages collected from the Calcaire inferieur and the Argiles brunes in the region of Safi (Morocco) has allowed us to pinpoint the age of these formations and to attribute them, respectively, to the Boissieri Zone ower part of the Campylotoxus Zone and to the upper part of the Campylotoxus Zone pper Valanginian. These units are also reinterpreted in terms of sequence stratigraphy. In addition, the analysis of the faunal spectrum shows that, although differing in some respects, these ammonite associations are similar to those of other peri-Tethyan platforms, while differing considerably from the basin assemblages.

The Exoskeleton is classified inside of a wider technology known as Wearable Robots, that group describes a robotic field who studies the interaction between the human body and the robotics. In those systems, a mechatronics structure is attached to different members of the human body, and while the wearer commands the mechanical system using physical signals, like the electronic stimulation produced by the orders of the brain, or the movements of the body generated by the muscles; the mechanical system do the hard work, like carrying heavier objects or helping the movement of handicaps members of the body. Since its conception in the sixties (Croshaw, 1969) the bibliography speaks about two models of wearable robots: the prosthetics and the orthotics systems (Fig. 1). The first group replaces the lost body members, while the second one assists to the body movements or, in other cases, extends the body capabilities.

Calibration and leak identification in Water Distribution Networks are of paramount importance in Water Industry. In this paper, Particle Swarm Optimization (PSO) is applied to tackle these problems. Standard PSO’s main drawback is that it is difficult to keep good levels of population diversity and to balance local and global searches. The formulation proposed, however, is able to find optimum or near-optimum solutions efficiently with considerably low computational effort because of the richer population diversity it introduces. Requiring only a low number of generations is a major advantage in real systems, where costs and time constraints prohibit too many iterations and hydraulic evaluations.

Our Neuchatel meeting of Lower Cretaceous ammonite workers was the last to be organized by Philip Hoedemaeker over a 15 year period. All members of the Kilian Group thank him for his contribution as chairman of: (1) the Lower Cretaceous Cephalopod Team (IGCP: International Geoscience Programme, projects 262: Digne, France, 1990; Mula, Spain, 1992, and 362: Piobbico, Italy, 1994; London, UK, 1997); (2) an independent group (Vienna, Austria, 2000); and (3) the IUGS (International Union of Geological Sciences) Lower Cretaceous Ammonite Working Group, the Kilian Group (Lyon, France, 2002).Progress in constructing the Lower Cretaceous standard ammonite zonation for the Mediterranean region can be followed by reading the successive reports. Fil: Reboulet, Stéphane. Universite´ Lyon 1; Francia Fil: Hoedemaeker, Philip J.. National Museum of Natural History; Países Bajos Fil: Aguirre-Urreta, Maria Beatriz. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Estudios Andinos "Don Pablo Groeber". Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Estudios Andinos "Don Pablo Groeber"; Argentina Fil: Alsen, Peter. Universidad de Copenhagen; Dinamarca Fil: Atrops, François. Universite´ Lyon 1; Francia Fil: Baraboshkin, Evgenij Y.. Moscow State University; Rusia Fil: Company, Miguel. Universidad de Granada, Facultad de Ciencias; España Fil: Delanoy, Gérard. Universite Nice; Francia Fil: Dutour, Yves. Universite´ Lyon 1; Francia Fil: Klein, Jaap. No especifica; Fil: Latil, Jean-Louis. No especifica; Fil: Lukeneder, Alexander. Naturhistorisches Museum, Vienna; Austria Fil: Mitta, Vasily. Paleontological Institute Of Russian Academy Of Sciences; Fil: Mourgues, Francisco A.. Observatoire Midi-pyrénées; Fil: Ploch, Izabela. Redakcja Serwisu Państwowego Instytutu Geologicznego; Fil: Raisossadat, Seyed Naser. University Of Birjand; Irán Fil: Ropolo, Pierre. Universite de Provence; Francia Fil: Sandoval, José. Universidad de Granada; España Fil: Tavera, José M.. Universidad de Granada; España Fil: Vasicek, Zdenek. Vsb – Technical University Of Ostrava; Fil: Vermeulen, Jean. No especifica; Fil: Arnaud, Hubert. Universite Grenoble Alpes; Fil: Granier, Bruno. Univ de Breragne Occidentale; Fil: Premoli-Silva, Isabella. Dipartimento Di Scienze Della Terra A. Desio

We discuss the significance of Mediterranean faunas in the study of the traditional Jurassic-Cretaceous boundary based on ammonites. Reviewing the record of ammonites and calpionellids from southern Spain and also discussing data from SE France, Bulgaria, Czechoslovakia, Cuba, Mexico and Argentina, which permit correlation with the Spanish faunas, we conclude that: a) Berriasella jacobi Mazenot is a good index fossil for the base of the Berriasian (=base of the Cretaceous System), b) the jacobi ammonite association clearly differentiates the latest Tithonian (Durangites Zone), c) the genus Kossmatia occurs in shelf deposits and, when present, is very rare in the European Tethys, d) the Mexican Gulf was not the centre of dispersal for Durangites or Kossmatia .

In the present paper we discuss the possibilities for the correlation of ammonites, calpionellids and calcareousnannofossils as recorded in the Puerto Escano section in Southern Spain (province of Cordoba), where a very detailed sampling was carried out. The section has proved to be a very significant one due both to the favorable outcrop and bedding preservation, and to the abundance of well-preserved fossil remains. We consider the selected section at Puerto Escano to be one of the most advantageous sites for the analysis of the stratigraphic distribution of significant macro and microfossils and thus for the study of the traditional Jurassic-Cretaceous boundary in ammonitico-rosso facies of the Mediterranean area.