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The seismic protection of small and medium historical towns is an important and current issue in Italy and in many other earthquake prone regions of Europe, where seismic actions are generally responsible for severe damages and collapses of buildings. In this context, the seismic vulnerability assessment of unreinforced masonry buildings assumes a crucial role considering the fact that they represent a widespread construction typology in Italian and European towns. Among the various approaches to evaluate the seismic vulnerability of existing buildings, fragility curves represent a very effective one in predicting potential damages of geographical areas characterized by similar building typologies. This paper presents the evaluation of fragility curves for macro-typologies of masonry buildings representative of a specific regional area in Italy and prone to the occurrence of out-of-plane collapse mechanisms in case of seismic events. In order to obtain consistent fragility curves, virtual populations of buildings are generated starting from the buildings of a selected case study, the Borgo San Rocco in Sora (FR).

The assessment of the seismic safety level of masonry buildings is a current and important issue at the basis of the complex process of preservation of historical masonry buildings which constitute mostly the Italian and European towns. Nowadays, different approaches able to provide important information concerning the seismic safety level of masonry structures are available. Among these, fragility curves allow to have a prediction of potential damages during an earthquake of geographical areas characterized by similar construction typologies. The present paper concerns the derivation of fragility curves of masonry buildings, typical of Italian historical towns and mainly characterized by the possible occurrence of local out-of-plane collapse mechanisms. To this end, a real case is accounted in the study in order to consider specific parameters characterizing the constructions composing these territorial realities.

Most of the Italian historical centers are composed of unreinforced masonry (URM) buildings arranged in aggregate configurations. Past and recent seismic events have underlined the high vulnerability of these buildings especially towards out-of-plane mechanisms. In order to reduce their vulnerability, the use of strengthening interventions based on fiber reinforced composite materials has become widespread in the last years. More recently, strengthening systems using natural fibers have been the object of experimental tests since they represent an innovative environmentally sustainable solution. The aim of this paper is to numerically analyze the feasibility of strengthening systems made of natural fibers embedded into cementitious matrices to prevent the out-of-plane mechanisms of perimeter façades belonging to masonry buildings in aggregate configurations. For this purpose, numerical analyses based on a macro-modeling approach for out-of-plane mechanisms are performed by considering the influence of adjacent structural units and the presence of strengthening systems made of natural fibers. Both aspects have been analyzed in detail and taken into account by introducing in the equation governing the problem both the friction acting between adjacent walls of building units, when in aggregate, and the contribution of the strengthening system. A building case study forming part of an aggregate of an Italian historical center has been considered for the development of the numerical analyses.

The recent seismic events occurred in Central Italy have rekindled the attention toward the safeguard of historical cities by leading to a renovated debate within the academic community regarding specific aspects of the seismic vulnerability and their protection. The study of masonry building aggregates is certainly one of these aspects, since they represent a typical urban configuration of the Italian but also European historical towns.

International audience; Biological recording at broad temporal and spatial scales produces large volumes of species occurrence data. Multi-source datasets, which include opportunistic records, are unstructured and contain bias, mainly due to uneven and unknown observation effort, but they also provide meaningful information about species phenology. Butterflies are well known and well represented in citizen-science programs and national inventories, which makes them an interesting case for phenological studies. This work aims to find a simple, flexible, fast-rendering phenology index, which has to prove reliable when compared to standard knowledge. Six indices (two non-corrected and four corrected for observation effort) were built and implemented on butterfly records. They were analysed against blind expert opinion and a set of monitoring data. Surprisingly, all indices produced mostly realistic phenological patterns and non-corrected indices were as good as corrected ones. The number of species records divided by the number of records of all species of the group collected during the same period is the only index that should be avoided, because of an over-correction of recording intensity. Additional work is needed, in particular to refine the analysis by testing the sensitivity of the index to the amount of data, as well as by employing statistical models that are also useful for exploring trends and seasonal shifts.

Background: Some authors have recently reported that the CC genotype of single-nucleotide polymorphism (SNP) rs1541160 mapping within the kinesin-associated protein 3 (KIFAP3) gene is associated with increased survival in sporadic amyotrophic lateral sclerosis (sALS). Objective and Methods: The relationship between the rs1541160 genotype and several clinical features of 228 ALS patients was evaluated with the intent of assessing any association between the ALS phenotype and KIFAP3. The SNP rs1541160 within the KIFAP3 expression profile was investigated using real-time PCR in a group of 6 patients harboring the CC genotype and in 12 patients harboring the TT genotype. Results: Analysis of our patients’ clinical features showed that almost half of those with the CC genotype were classified as having upper motor neuron-predominant ALS (UMN-ALS). Conversely, there was an approximately 10% frequency of UMN-ALS in both the TT and the TC patient groups as well as in the entire cohort considered as a whole (p < 0.005). The SNP rs1541160 genotype did not appear to have any effect on patient survival or on KIFAP3 expression. Conclusions: The incidence of the UMN-ALS phenotype in the CC patients of this cohort supports the hypothesis that the SNP rs1541160 within the KIFAP3 gene is a potential modifier of the ALS phenotype.

Owing to the frequent observation of poverty of movements, facial hypomimia and balance impairment, amyotrophic lateral sclerosis (ALS) variant with predominance of upper motor neuron involvement (UMN-ALS) is prone to be diagnosed with Parkinsonism. A clinical assessment, including the velocity-dependent stretch response test to differentiate between pyramidal and extrapyramidal stiffness; the Unified Parkinson's Disease Rating Scale and the Berg Balance Scale to assess degree of bradykinesia and postural instability; and (123)I-FP-CIT scintigraphy evaluation to investigate the nigrostriatal circuit involvement, were carried out to characterize Parkinson-like features in UMN-ALS patients. Sixteen UMN-ALS patients were included in the study. The velocity-dependent stretch response indicated spasticity in all the muscles tested. The degree of stiffness was found to be related to bradykinesia and postural instability. Eleven patients (70%) showed a reduction in striatal (123)I-FP-CIT uptake found to be related to disease duration and patients' ages but not to scores of the functional scales. Slowness of movements and postural instability noted in our patients could be mostly attributed to spasticity. The lack of any correlation between UPDRS or BBS scores and the degree of nigrostriatal impairment on DaTSCAN seems to disprove nigrostriatal circuit involvement in these extrapyramidal-like features.

Emotional processing may be abnormal in amyotrophic lateral sclerosis (ALS). Our aim was to explore functional anatomical correlates in the processing of aversive information in ALS patients. We examined the performance of nine non-demented ALS patients and 10 healthy controls on two functional MRI (fMRI) tasks, consisting of an emotional attribution task and a memory recognition task of unpleasant versus neutral stimuli. During the emotional decision task, subjects were asked to select one of three unpleasant or neutral words. During the memory task, subjects were asked to recognize words presented during the previous task. Controls showed, as expected, greater activation in the right middle frontal gyrus during selection of unpleasant than neutral words, and a greater activation mainly in right-sided cerebral areas during the emotional recognition task. Conversely, patients showed a general increase in activation of the left hemisphere, and reduced activation in right hemisphere in both emotional tasks. Such findings may suggest extra-motor neurodegeneration involving key circuits of emotions, mostly negative, commonly involved in FTD.

Background and purpose: Several studies have reported an increase in ALS incidence in recent years but population-based studies in Europe do not confirm this trend. To analyze ALS incidence over time we conducted a retrospective incidence study in the Padova district of Italy (1992 to 2005). We had previously conducted a survey in the same area in the years 1980–1991. Methods: We used the archives of all the neurological wards of the Padova district to identify all subjects with a discharge diagnosis of ALS or motor neuron disease and resident in the Padova district. Results: We ascertained 182 patients (85 males and 97 females; male:female ratio 0.88:1) over the 14-year study period. The annual incidence rates adjusted by sex and age increased from 1.31/100000/year in the years 1992–1994 to 1.92/100000/year in the years 2004–2005. Conclusions: This study confirmed an ALS incidence increase over the last 25 years in the Padova district. The increase in incidence may be partially explained by the ageing of the general population rather than by an improved diagnostic assessment.

TAR DNA binding protein (TDP-43) is the pathologic substrate of neuronal and glial aggregates in amyotrophic lateral sclerosis (ALS). Pathologic TDP-43 is hyperphosphorylated and cleaved to generate abnormal protein species that accumulate in the cytoplasm. To assess the hypothesis of TDP-43 pathology as a systemic disorder in ALS we analysed the immunohistochemical and biochemical profile of TDP-43 in muscle biopsies of 30 ALS patients and 30 controls. In all ALS muscle biopsies we observed that TDP-43 was constantly present in an intranuclear localization and TDP-43 Western blotting showed only a 43-KDa band as controls. Our results suggest that TDP-43 pathology is probably confined to the central nervous system in ALS.

The distinction between primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) still remains debated. Recently, PLS patients displaying lower motor neuron (LMN) signs have been defined as 'upper motor neuron (UMN)-dominant ALS', using 'clinically pure PLS' diagnosis to those with no LMN signs. To further characterize the LMN involvement in UMN-dominant ALS we investigated the presence and the extent of neurogenic abnormalities in the skeletal muscle of patients affected with a pyramidal syndrome consistent with UMN-dominant ALS. A total of nine patients affected with UMN-dominant ALS were analysed. In all cases, muscle biopsies showed the presence of scattered or clustered atrophic angulated fibres in small groups, and a mild to moderate fibre type-grouping. Target and targetoid fibres were detected in two cases only. Three patients had a second muscle biopsy which demonstrated a roughly unchanged pattern of chronic denervation with still moderate reinnervation phenomena. This study suggests that in UMN-dominant ALS muscle denervation may be characterized by an early chronic impairment of a restricted number of LMNs. The extent rather than the presence of LMN signs may allow to categorize patients with motor neuron disease involving mainly UMN into distinct entities.