Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis

Copyright: © 2019 Barc K et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Amyotrophic lateral sclerosis (ALS) is a fast progressing neurodegenerative disease leading to quadriplegia, anarthria and respiratory insufficiency. A large variety of phenotypes and disability progression requires individually tailored management. Identification of predictors of poor prognosis may not only improve management, but also allow for more precise patients' stratification for clinical trials or research studies. The aim of the study was to investigate the influence of emotional lability present at disease onset on ALS progression by exploring its direct impact on the decay of the ALS Functional Rating Scale-Revised (ALSFRS-R). The study was performed in a group of 1145 patients from Germany, Poland, Portugal and Turkey between 2014 and 2018. The analysis showed that the presence of emotional lability at ALS onset was linked to a faster decline of ALSFRS-R (0.70 vs 0.50, p<0.0001), in case of either bulbar (0.80 vs 0.65, p<0.05) or limb disease onset (0.59 vs 0.46, p <0.01). It was most prominent in the bulbar subscore of ALSFRS-R. A multiple regression analysis showed a direct influence of emotional lability at ALS onset on disease progression, regardless of age, gender, site of onset, weight loss, cognitive impairment and diagnosis delay (β=0.071; p=0.019). It can therefore be concluded that the presence of emotional lability at the disease onset is an independent factor of faster disease progression in ALS.This study was supported by OnWebDuals project (JNPD 01ED1511B; DZP/2/JPND-III/2015). This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported through the following funding organization under the aegis of JPND - www.jpnd.eu: Germany, Bundes-ministerium für Bildung und Forschung (BMBF); Poland, Narodowe Centrum Badań i Rozwoju (NCBiR); Portugal, Fundação a Ciência e a Tecnologia (FCT); Sweden, Vetenskapsrådet (VR).info:eu-repo/semantics/publishedVersio

Role of DTI-MRI parameters in diagnosis of ALS: useful biomarkers for daily practice? Tertiary centre experience and literature review

Introduction. Despite the rapid development of neuroimaging techniques, the diagnosis of amyotrophic lateral sclerosis (ALS) remains a significant challenge. Magnetic resonance imaging (MRI) is important for ruling out ALS mimickers, while Diffusion Tensor Imaging (DTI) is a useful tool for the identification of cortical tract damage. The aim of this study was to identify the optimal set of DTI parameters to support the diagnosis of ALS that could be applied to everyday MRI and be used as a disease biomarker in daily practice. Material and methods. Forty-seven ALS patients and 55 age- and gender-matched healthy individuals underwent MRI using a 1.5-Tesla scanner including a DTI sequence with 30 spatial directions and a b-value 0/1,000 s/mm2. Two independent researchers measured the DTI parameters: fractional anisotropy (FA), TRACE and apparent diffusion coefficient (ADC) using freehand regions of interest (ROIs) placed along both corticospinal tracts (CSTs), starting at the level of the internal capsule and ending at the medulla. Results. Statistical significance was only achieved for fractional anisotropy (FA) (ALS vs controls, p &lt; 0.001). The highest sensitivity was found in the brainstem (cerebral peduncles, pons and pyramids) where it ranged from 72.3% to 80.9%, whereas the highest specificity was observed at the level of the internal capsule (94.6%). The combined highest sensitivity and specificity was obtained in the pons (72.3% and 72.7%, respectively). Classifier based positive predictive values for Youden index cut-off scores varied between 60.7% and 69.4%. Conclusions. Fractional anisotropy (FA) measured at the level of the brainstem was shown to be the single most relevant parameter in differentiating patients with ALS from healthy subjects. This has the potential to become an ALS-specific biomarker for patient identification in daily practice

Map Metadata: the Basis of the Retrieval System of Digital Collections

The article presents research on the evaluation of hidden map metadata. A hidden map is a map being part of a book that illustrates certain facts described in the book (e.g., military campaigns, political processes, migrations). The evaluation regards their completeness. Metadata completeness is the degree to which objects are described using all metadata elements. The analysis took into account the metadata of archival maps accessed via the GeoPortOst geoportal. Over 3000 hidden maps from the period 1572&ndash;2018 were analyzed, and the map set was divided into 8 collections. The main purpose of cartographers and librarians is to facilitate understanding of the relationship between individual information (librarians) and spatial data (cartographers). To this end, the research focused on the kind of information about old maps that should be stored in metadata to describe them in terms of space, time, content and context so as to increase their interoperability. The following metadata were taken into account in the assessment: title of content, type of content, date, date range, rights, language, subject, distribution format, geographic location, scale of map, reference system, mapping methods, map format, and source materials used to develop the map. The completeness of individual metadata as well as the completeness of metadata for individual collections was assessed. Finally, good practices of individual collections and metadata that could increase the interoperability of the entire collection were identified. The evaluation enables the owners to show the strengths and weaknesses of a given collection in a quick and easy way

The visualization of a mountain using 3D printing

Flat studies showing terrain (e.g. in form of maps) do not fully reflect its nature. Planning or design projects are definitely better represented in three-dimensional space. Previously used “sand tables”, especially popular in military applications, are expensive and uncomfortable to use due to their large size. In addition, they are modifiable only to a small extent and do not provide such accuracy as traditional maps. With the development of modern model relief techniques, a spectrum of the possibilities for development of customized spatial models (defined as models of the surrounding reality, mapped in the appropriate scale in three dimensions), has increased. The aim of this article is to present the possibilities of using 3D printing for the visualization of a mountain. Based on the digital terrain model (DTM), a model of a part of the Tatra Mountains was developed. It was established by DTM data processing and printing on a 3D printer using the FDM (Fused Deposition Modeling) technology. This article describes the principles for developing such a model and the advantages and disadvantages of the proposed solution. Keywords: cartography, digital terrain model, sand table, FDM technolog

The visualization of a mountain using 3D printing

Flat studies showing terrain (e.g. in form of maps) do not fully reflect its nature. Planning or design projects are definitely better represented in three-dimensional space. Previously used “sand tables”, especially popular in military applications, are expensive and uncomfortable to use due to their large size. In addition, they are modifiable only to a small extent and do not provide such accuracy as traditional maps. With the development of modern model relief techniques, a spectrum of the possibilities for development of customized spatial models (defined as models of the surrounding reality, mapped in the appropriate scale in three dimensions), has increased. The aim of this article is to present the possibilities of using 3D printing for the visualization of a mountain. Based on the digital terrain model (DTM), a model of a part of the Tatra Mountains was developed. It was established by DTM data processing and printing on a 3D printer using the FDM (Fused Deposition Modeling) technology. This article describes the principles for developing such a model and the advantages and disadvantages of the proposed solution. Keywords: cartography, digital terrain model, sand table, FDM technolog

Spatio-Temporal Database of Places Located in the Border Area

As a result of changes in boundaries, the political affiliation of locations also changes. Data on such locations are now collected in datasets with reference to the present or to the past space. Therefore, they can refer to localities that either no longer exist, have a different name now, or lay outside of the current borders of the country. Moreover, thematic data describing the past are related to events, customs, items that are always “somewhere”. Storytelling about the past is incomplete without knowledge about the places in which the given story has happened. Therefore, the objective of the article is to discuss the concept of spatio-temporal database for border areas as an “engine” for visualization of thematic data in time-oriented geographical space. The paper focuses on studying the place names on the Polish-Ukrainian border, analyzing the changes that have occurred in this area over the past 80 years (where there were three different countries during this period), and defining the changeability rules. As a result of the research, the architecture of spatio-temporal databases is defined, as well as the rules for using them for data geovisualisation in historical context

Map Metadata: the Basis of the Retrieval System of Digital Collections

The article presents research on the evaluation of hidden map metadata. A hidden map is a map being part of a book that illustrates certain facts described in the book (e.g., military campaigns, political processes, migrations). The evaluation regards their completeness. Metadata completeness is the degree to which objects are described using all metadata elements. The analysis took into account the metadata of archival maps accessed via the GeoPortOst geoportal. Over 3000 hidden maps from the period 1572-2018 were analyzed, and the map set was divided into 8 collections. The main purpose of cartographers and librarians is to facilitate understanding of the relationship between individual information (librarians) and spatial data (cartographers). To this end, the research focused on the kind of information about old maps that should be stored in metadata to describe them in terms of space, time, content and context so as to increase their interoperability. The following metadata were taken into account in the assessment: title of content, type of content, date, date range, rights, language, subject, distribution format, geographic location, scale of map, reference system, mapping methods, map format, and source materials used to develop the map. The completeness of individual metadata as well as the completeness of metadata for individual collections was assessed. Finally, good practices of individual collections and metadata that could increase the interoperability of the entire collection were identified. The evaluation enables the owners to show the strengths and weaknesses of a given collection in a quick and easy way

Review of the methods of cartographic presentation of warfare

Maps, depicting the warfare, are elaborated according to the rules developed over the years. Although, they are not free of errors. The authors draw our attention to the diversity of methods related to the cartographic presentations, which are shown on the historical maps. This is quite understandable, because every such a map introduces an individual point of view on the historical facts. It is noted, that the most commonly used methods are the qualitative ones, among which, for instance, first of all, the method of signature is frequently used

Comparative Tests of Electric Voltage and Resistance with the Use of Virtual Measurement Systems

The article discusses issues related to virtual measurement systems and presents the results of comparative measurements of resistance and electric voltage measured with various measuring devices, including virtual NI Elvis systems. The accuracy and measurement errors were compared, as well as the advantages and disadvantages of the devices and methods used for the measurements. Moreover, a computer simulation of the measurements in the Multisim software was performed, and its results were compared with the actual indications of the meters