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An intrinsic feature of behaviour orbits around its temporal dimension consisting of patterns in time: investigations on behaviour necessarily deal with sequences often impossible to be perceived by the human eye [Eibl-Eibesfeldt I. Ethology: the biology of behavior. New York: Holt, Rinehart and Winston. 1970]. Thus, the study of behavioural sequencing in terms of higher order arrangements, how the sequences are structured and, importantly, their analysis in normal subjects and in subjects affected by specific movement/behavioural disorders may represent a stimulating but challenging task. By means of T-Pattern analysis (TPA), it is possible to study these hidden features of behaviour and, specifically, its sequential organization [Casarrubea M et al. J Neurosci Methods. 2015;239:34-46]. This is particularly interesting when such a multivariate technique is used to study animal models of neurological illnesses characterized by movement disorders such as Parkinson’s disease, Tourette’s syndrome or histamine-depleted conditions. Here we illustrate our recent findings, obtained by means of TPA, in different rodent models presenting movement disorders [Casarrubea M et al. Behav Brain Res 2019;362:28-35; Santangelo A et al. CNS Neurosci Ther 2018;24:703-711; Santangelo A et al. Neuropharmacology. 2017;113:533-542]. The results may represent a stimulating topic of discussion on what, from a translational perspective, each model may suggest in terms of human behavioural abnormalities. We propose TPA as a suitable tool to describe the architecture of a behaviour both in animal models of neurological disorders and human patients as well

The liver can be affected by a wide range of therapeutic and environmental chemicals and here we want to provide a summary of the complex effects of alcohol, cannabinoids and nicotine on liver function. Alcohol is the most important agent that produces liver injury, manifesting as alcoholic fatty liver disease. In addition, it is one of the main etiologic agents for hepatocellular carcinoma development. Studies reviewed in this article regarding cannabinoids, show that Δ9-THC does not produce any harmful effects on the liver, while cannabidiol has hepatoprotective effects in ischemia/reperfusion and alcohol-induced liver injuries. The liver is negatively affected by nicotine exposure, but surprisingly nicotine was shown to have a positive effect on the liver in the diet-induced obese animal model, which should be confirmed by future research., peer-reviewed

Background Late-onset Pompe disease (LOPD) is a recessive disease caused by α-glucosidase (GAA) deficiency, leading to progressive muscle weakness and/or respiratory failure in children and adults. Respiratory derangement can be the first indication of LOPD, but the diagnosis may be difficult for pneumologists. We hypothesize that assessing the GAA activity in suspected patients by a dried blood spot (DBS) may help the diagnosis of LOPD in the pneumological setting. Population and methods We performed a multicenter DBS survey of patients with suspected LOPD according to a predefined clinical algorithm. From February 2015 to December 2017, 140 patients (57 ± 16 yrs., 80 males) were recruited in 19 Italian pneumological units. The DBS test was performed by a drop of blood collected on absorbent paper. Patients with GAA activity

In comparison with wilde type mice, animal models of Duchenne Muscolar Dystrophy (mdx mice) show a different behavioral organization with a more articulated structure of the temporal patterns. Present study sheds light, for the first time, on specific temporal features of behavior in an animal model of DMD.

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The aim of present research is to study the temporal structure of the behavior in two strains of rats with different basal level of emotionality. To this purpose, the temporal profile of the behavior in Wistar rat and in the spontaneously more anxious DA/Han strain was analyzed in the Elevated Plus Maze. Both quantitative and multivariate t-pattern analyses were carried out. In comparison with Wistar, DA/Han subjects showed a significant reduction of the permanence in open arm and a significant increase of the time spent in the central platform of the maze. Mean frequencies of each behavioral element showed significant modifications both in open and in closed arm. Multivariate t-pattern analyses demonstrated a very different temporal profile of behavior in the two strains: Wistar rats presented 197 t-patterns of different composition, whereas DA/Han rats only 26; as to the mean number of t-patterns, Wistar presented a value of 698.90, whereas DA/Han only 92.80. Similar clear-cut differences were detected for the mean number of t-patterns in the two arms and for the time course of such t-patterns. Present study has evidenced that the temporal organization of the bahavior in Elevated Plus Maze is differently structured in two strains of rats with different basal level of emotionality.

Aim of the research was to evaluate, by means of quantitative and multivariate temporal pattern analyses, the behavior of Wistar rat in elevated plus maze (EPM) test. On the basis of an ethogram encompassing 24 behavioral elements, quantitative results showed that 130.14 ± 8.01 behavioral elements occurred in central platform and in closed arms (protected zones), whereas 88.62 ± 6.04 occurred in open arms (unprotected zones). Percent distribution was characterized by a prevalence of sniffing, walking and vertical exploration. Analysis of minute-by-minute duration evidenced a decrease for time spent in open arms and central platform and an increase for time spent in closed arms. As to multivariate t-pattern analysis, 126 different temporal patterns were detected. Behavioral stripes, summarizing distribution of such t-patterns along time, showed that several t-patterns were not homogeneously distributed along the test observational period: t-patterns encompassing behavioral events occurring prevalently in central platform-open arms were observed during the first minutes, whereas t-patterns structured on the basis of events occurring mainly in central platform-closed arms were detected during the last minutes. Therefore, during the observation in elevated plus maze, rat's behavior undergoes significant rearrangements of its temporal features. Present research demonstrates, for the first time, the existence of complex and significantly timed behavioral sequences in the activity of Wistar rats tested in elevated plus maze. Application of t-pattern analysis can provide useful tools to characterize the behavioral dynamics of anxiety-related rodent behavior and differentiate the effect of various anxioselective substances.

The objective of this study was to describe a large Italian cohort of patients with late-onset glycogen storage disease type 2 (GSDII) at various stages of disease progression and to evaluate the clinical effectiveness of alglucosidase alpha enzyme replacement therapy (ERT). Previous studies showed in late-onset patients ERT efficacy against placebo and variable response in uncontrolled studies. Seventy-four juvenile or adult GSDII patients were treated with ERT in a multicenter open label, non-randomized study, from 12 months up to 54 months. Recombinant human alpha glucosidase (rh-GAA) was injected by intravenous route at 20 mg/kg every second week. Patients were divided into three groups according to ERT duration: Group A received treatment for 12–23 months (n = 16), Group B for 24–35 months (n = 14), and Group C for more than 36 months (n = 44). Clinical assessment included a 6-min walk test (6MWT), forced vital capacity (FVC), the Walton and Gardner-Medwin score, the number of hours of ventilation, body mass index, echocardiography and blood creatine kinase (CK). Included in our cohort were 33 males and 41 females (M:F = 0.8:1), with a mean age at first symptoms of 28.3 years (range 2–55 years) and a mean age of 43 years at study entry (range 7–72 years). Seven wheelchair bound patients, as well as 27 patients requiring ventilation support, were included. After treatment we could observe an increase in distance walked on the 6MWT in the large majority of patients (48/58; 83%), with an overall mean increase of 63 m (from 320 ± 161 to 383 ± 178 m). After treatment in the majority of patients FVC was improved or unchanged (45/69; 65%). In ventilated patients we observed an improvement in average number of hours off the ventilator (from 15.6 to 12.1 h). Six patients stopped mechanical ventilation and two others started it. The effect of therapy was not related to ERT duration. Nine of 64 patients (13%) that underwent to echocardiography showed a variable degree of cardiac hypertrophy (left ventriculum or septum), and a positive effect was observed after 36 months of ERT in one adult case. Discontinuation of treatment occurred in four patients: one drop-off case, one patient died for a sepsis after 34 months of treatment and two patients stopped ERT for worsening of general clinical condition. Mild adverse effects were observed in four cases (5%). This study represents the largest cohort of late-onset GSDII patients treated with ERT, and confirm a positive effect of treatment. These results, obtained in a large case series on therapy, indicate a favourable effect of ERT therapy, even in more advanced stage of the disease.

Introduction: The clinical course of late-onset Pompe disease is heterogeneous, and new clinical outcome measures are needed to evaluate enzyme replacement therapy (ERT). Methods: We correlated the 6-Minute Walk Test (6MWT), Walton and Gardner-Medwin (WGM) score, and GSGC (Gait, Stairs, Gower, Chair) scores in 40 patients. Results: At baseline, the GSGC score correlated with both WGM (P < 0.001, n = 33) and 6MWT (P < 0.001, n = 26). After 1 year of ERT, we observed a significant change in gait, stairs and chair performance on the GSGC scale. The 6MWT significantly increased from 319 to 371 meters in 32 patients, and the WGM score was reduced. Conclusions: GSGC is a group of functional tests that requires only a few minutes to perform, therefore, this score might be a good indicator to be used in future studies. Muscle Nerve 45: 831–834, 2012

Respiratory failure is an unavoidable or very frequent event in the natural history of some neuromuscular diseases, whereas it appears very unfrequently in others. In some cases, like Pompe disease, respiratory failure shows a more rapid worsening compared to motor impairment, sometimes being the onset event. The most frequent respiratory onset manifestation is exercise breathlessness, frequently associated to a history of lung infections. Nevertheless these patients may show a physiological picture of chronic respiratory insufficiency. Home mechanical ventilation improves survival and quality of life of these patients, with a reduction in health care costs. As a consequence pulmonologists must perform a more relevant role in the care of these patients and they must increase their skills for such a task. In Pompe disease mechanical ventilation is started during an episode of Acute Respiratory Failure (ARF) without any previous lung function tests: as a consequence these patients require more frequent physiological evaluations to avoid potentially fatal exacerbations. Therefore a collaboration between pulmonologist and neurologist is mandatory. Respiratory follow-up is aimed to early diagnosis and treatment of respiratory complications. It may differ according to different diagnosis and disease stage. The specific interest for adult onset Pompe disease relies on the aivailability of enzimatic therapy which has been shown to be effective in slowing disease progression. The aim of this statement is to give pulmonologists some simple information in order they can fulfil their role of "main caregiver" with appropriate and quick diagnosis and treatment.

The available amount of fresh water for agriculture, and specifically for irrigation, is decreasing all over the world. The quality of irrigation water is deteriorating, and saline/sodic waters are increasingly used in many arid and semi-arid regions of the world. Salinization is closely associated with the process of desertification.Sustainable land management practices are urgently needed to preserve the production potential of agricultural land while safeguarding environmental quality. In cracking soils sustainable management should take into account the occurrence of bypass flow and the influence that land use may have on soil structure and bypass flow phenomena.Measurement of vertical and horizontal shrinkage in confined soil cores was found to be suitable for determining the Soil Shrinkage Characteristic Curve (SSCC) and for incorporating shrinkage in the soil hydraulic parameters/functions determined on confined undisturbed soil samples. An optimization procedure based on multi-step outflow experiments with inverse modelling was developed for determining the soil hydraulic characteristics (HC). The need for accounting for structural porosity and shrinkage processes was recognized on the basis of hydraulic conductivity values determined by the suction crust infiltrometer method and of the SSCC determined on confined soil cores.Analysis of the response of clay soils to ESP values up to 15, showed that the concept of critical threshold needs reconsideration, because increasing soil degradation upon increasing ESP appeared to be a continuum. A major hazard of deterioration of structural and hydraulic properties was recognized even at low ESP values (ESP

This paper aimed at investigating if the application of biochar (BC) to desert sand (DS) from the United Arab Emirates (UAE), characterized by a very poor soil-water retention (SWR) and by a very low value of the maximum water available for crops (AWmax), could positively affect soil water balance, by reducing the irrigation needs (VIRR) and improving the irrigation water use efficiency (IWUE) and the water use efficiency (WUE). The analysis was performed for three crops, i.e., wheat (Triticum aestivum), sorghum (Sorghum vulgare) and tomato (Lycopersicon esculentum). BC was applied to the DS at different fractions, fBC (fBC = 0, 0.091, 0.23 and 0.33). Drip irrigation was adopted as a highly efficient water saving method, which is particularly relevant in arid, water-scarce countries. Soil water balance and irrigation scheduling were simulated by application of the AQUACROP model, using as input the SWR measured without and with BC addition. The effect of BC was investigated under either a no-water stress (NWS) condition for the crops or deficit irrigation (DI). The results showed that the application of BC made it possible to reduce the predicted VIRR and to increase the IWUE under the NWS scenario, especially for wheat and sorghum, with less evident benefits for tomato. When a deficit irrigation (DI) was considered, even at the lowest considered fBC (0.091), BC counterbalanced the lower VIRR provided under DI, thus mitigating the yield reduction due to water stress, and improved the WUE. The influence of BC was more pronounced in wheat and tomato than in sorghum. The results evidenced that the application of BC could be a potential strategy for saving irrigation water and/or reducing the effects of drought stress in desert sand. This means that biochar could be used a management option to promote local production and reduce the dependency on food import, not only in the UAE, but also in other countries with extremely arid climatic conditions and large extensions of sandy soils similar to the considered DS.

Aim of the present review is to offer an outline of the application of T-pattern analysis (TPA) in the study of neurological disorders characterized by anomalies of movement and, more in general, of behavior. TPA is a multivariate technique to detect real time patterns of behavior on the basis of statistically significant constraints among the events in sequence. TPA is particularly suitable to analyse the structure of behavior. The application of TPA to study movement and behavioral disorders is able to offer, with a high level of detail, hidden characteristics of behavior otherwise impossible to detect. For its intrinsic features, TPA is completely different not only from quantitative evaluations of behavior such as assessments of frequencies, durations, percent distributions etc. of individual behavioral components, but also from the largest extent of multivariate approaches based, for instance, on the analysis of transition matrices. Various applications of TPA in the study of behavior in human patients and in animal models of neurological disorders are discussed. TPA is a suitable tool to study the movement and behavioral disorders. This review represents a useful background for researchers, therapists, physicians etc. who intend to use this technique.