Lennox-Gastaut Syndrome: A State of the Art Review.

Lennox-Gastaut syndrome (LGS) is a severe age-dependent epileptic encephalopathy usually with onset between 1 and 8 years of age. Functional neuroimaging studies recently introduced the concept of Lennox-Gastaut as "secondary network epilepsy" resulting from dysfunctions of a complex system involving both cortical and subcortical structures (default-mode network, corticoreticular connections, and thalamus). These dysfunctions are produced by different disorders including hypoxic-ischemic encephalopathies, meningoencephalitis, cortical malformations, neurocutaneous disorders, or tumors. The list of etiologies was expanded to pathogenic copy number variants at whole-genome array comparative genomic hybridization associated with late-onset cases or pathogenic mutations involving genes, such as GABRB3, ALG13, SCN8A, STXBP1, DNM1, FOXG1, or CHD2. Various clinical trials demonstrated the usefulness of different drugs (including rufinamide, clobazam, lamotrigine, topiramate, or felbamate), ketogenic diet, resective surgery, corpus callosotomy, and vagus nerve stimulation in the treatment of epileptic manifestations. The outcome of LGS often remains disappointing regarding seizure control or cognitive functioning. The realization of animal models, which are still lacking, and the full comprehension of molecular mechanisms involved in epileptogenesis and cognitive impairment would give a relevant support to further improvements in therapeutic strategies for LGS patients

Point pattern analysis: an application to the loyalty networks of chain-stores

At the 41th congress of ERSA., Staufer-Steinnocher (2001) proved that kernel density estimation, a technique of spatial analysis belonging to the point pattern methods, can be usefully applied to geomarketing. Following this point of view, the aim of this paper is to show that other point pattern techniques (center-grahic statistics, global and local autocorrelation indexes, clustering methods, 'nearest neighbour index', 'Ripley's K statistic', etc.) are able to suggest important considerations in marketing researches, making explicit the "geographical knowledge" embedded in available informations. Namely this argument is demonstrated, analysing spatial distributions of big stores chained in a promotional network, finalised to improve fidelity in the consumers.

Interview of Rosanna Mastrangelo

The Interviewee: Rosanna Mastrangelo was born in February 1964, in South Philadelphia. Her parents, along with the rest of her family, were Italian immigrants who had come to America after the end of World War II in hopes of rebuilding a better life for themselves. Raised in a tight-knit Italian neighborhood and surrounded by Old World traditions, Rosanna quickly realized the importance of remaining close to one’s roots, especially in forming her unique sense of identity. But as she went to school and became acquainted with people of other backgrounds and experiences, it became ever more clear that her sense of identity was divided. By the language she spoke, the type of education she received, and the abundant opportunities which presented themselves to her-Rosanna came to perceive a grave difference between herself and her parents. She was not just Italian, she was an Italian-American. And her home and her heart belonged forever in America. Her life story, which is recounted in this interview, proffers excellent insight into the process of assimilation among first-generation immigrants. Her story is also full of interesting facts about mafia history, and even allows for some insight into the problems and concerns of those weathering midlife. Today, Rosanna still resides in a predominantly Italian neighborhood of South Philadelphia. Her home, which she shares with her mother and daughter, is still devoted to keeping the Italian culture alive. She has also spent nearly 40 years of her life serving the United States Federal Government as an auditor at the Defense Logistics Agency, and she couldn’t be more proud to be an American, to support the war fighter and the American way of life. Surely, the sacrifices her family made in order to achieve a better life were not in vain

Risk and Abnormal Returns in Markets for Congestion Revenue Rights

In organized energy markets that use locational pricing, power generators and energy suppliers procure financial transmission rights (FTRs) to hedge against grid con- gestion charges, while third-party speculators attempt to capture a return with these extremely volatile contracts. This paper develops a novel methodology for estimating the systematic risk of individual FTRs and detecting the presence of abnormal returns among these financial instruments. The prevalence of congestion paths with abnormal returns could be used by policy experts as an efficiency measure when assessing the performance of FTR markets. Being the only organized energy market in the Western Interconnection, California has implemented a version of FTRs officially known as congestion revenue rights (CRRs). This paper applies the proposed methodology to all auctioned CRRs from 2009 to 2015. Our analysis identifies the paths that exhibit persistent abnormal returns, with the majority of them being positive. We also compare the patterns of risk and abnormal returns between on-peak and off-peak CRRs, and find no significant differences

Regulating Greenhouse Gases from Coal Power Plants under the Clean Air Act

The Clean Air Act has assumed the central role in U.S. climate policy, directing the Environmental Protection Agency to develop regulations governing the emissions of greenhouse gases from existing coal-fired power plants. The cost and environmental effectiveness of policy options depend on abatement costs, the magnitude of emissions reduction opportunities, and the sensitivity of plant utilization. This paper examines the operation of electricity-generating units over 25 years to estimate the marginal costs and potential magnitude of emissions reductions that could result from improvements in their operating efficiency. We find that a 10 percent increase in coal prices causes a 0.3 to 0.9 percent heat rate reduction, broadly consistent with engineering assessments of abatement costs and opportunities. We also find that coal prices have a significant effect on utilization, but that will vary depending on the policy design. The results are used to compare cost-effectiveness of alternative policies.efficiency, regulation, greenhouse gas, carbon dioxide, coal, performance standards

Jaim Etcheverry, Guillermo. La tragedia educativa. Buenos Aires, Fondo de Cultura Económica, 1999, 231 p.

Fil: Mastrangelo, Fabiana

Zimmermann, Eduardo A. Los liberales reformistas. La cuestión social en la Argentina (1890-1916). Buenos Aires, Sudamericana - Universidad de San Andrés, 1995, 250 p.

Fil: Mastrangelo, Fabiana

De enemigo vencido a tesoro cercado: Un estudio etnohistórico sobre el ambiente en la producción forestal del Alto Paraná de Misiones (Arg.). En: Avá, nº 20

Se presenta una etnohistoria ambiental de la producción forestal en el bosque atlántico altoparanaense de Misiones (Argentina), entre 1874 y 2010. Para ello se utiliza el enfoque de la historia ambiental que se propone conocer las prácticas e ideas con las que una sociedad interviene la naturaleza (Arnold, 1992). Es etnohistoria, porque se orienta a caracterizar la diversidad de grupos sociales ágrafos y subalternos. En base a diferencias tecnológicas, de recurso y de territorio en uso, el período estudiado se subdividió en tres etapas: a) 1874 a 1920: la representación hegemónica es la selva como enemigo a vencer para integrar a Misiones a la Nación. b) 1930 a 1960: la selva es fuente de materia prima a costo cero. c) 1970 a 2010: la selva es resguardada en áreas de reserva, se define a la plantación monocultivo de árboles como bosque. La hipótesis central del artículo es que este abordaje histórico y etnográfico a la vez, posibilita entender la relación naturaleza-cultura como un proceso de configuración simultánea de dominios excluyentes, donde la clase y la diversidad étnica determinan experiencias sociales particulares del ambiente

Roles of different glial populations in neuronal maturation: implications in Rett syndrome and Alzheimer’s disease

2013/2014It is well established that in healthy brain, the interplay between neurons and glia play a very important role in the maintenance of a correct physiological activity. Recent studies have shown the existence of an active involvement of glia in both the formation and function of the synapse (Araque et al. 1999; Parpura et al. 2012). These findings have led to reconsider the role of glia also in the case of pathological situations. Indeed, beyond its fundamental implication in healthy brain, glia has been found to play a critical role in several neurological disorders. It is now clear (Nguyen at al. 2012, Maezawa and Jin, 2010) that glial cells are involved not only in neurodegenerative diseases such as Alzheimer's disease (Angelova PR at al. 2014), but also in diseases characterized by intellectual disability. In this regard, recent evidence has shown the involvement of glial cells in Rett Syndrome (Ballas et al., 2009, Maezawa et al., 2009, Derecki et al., 2012 Okabe 2012). The purpose of my thesis is to investigate the role of different populations of astrocytes in the development and survival of neurons using in vitro models of both neurodegenerative and neurodevelopmental disorders. To this aim, in the first part of my project I participated in the setting up of a microfluidic system, an experimental model aim at studying the interconnections between different brain cells, focusing my attention on the contribution of glial cells derived from different brain areas in these pathological states. Secondly, through the use of the microfluidic system, we have shown that astrocytes derived from distinct brain areas have different effects on neurons if exposed to several harmful stimuli. Specifically I have evaluated both the development and viability of hippocampal neurons co-cultured with either cortical or hippocampal astrocytes in a neuroinflammatory context represented by the stimulation of beta amyloid peptide (Aß) together with several proinflammatory cytokines. Taking advantage of the microfluidic system, we showed that hippocampal and cortical astrocytes exposed to these stimuli were able to significantly increase neuronal cell death. Interestingly, hippocampal astrocytes induced an elevated neuronal cell death if compared to cortical astrocytes. (Bianco F, N Tonna, Lovchik RD, Mastrangelo R, Morini R, Ruiz A, E Delamarche, Matteoli M. Anal. Chem. 2012). To assess whether neuronal death was due to soluble factors released by astrocytes, we monitored the neuronal calcium responsiveness upon the exposure of astrocytes to Aß and IL 1ß. We found that both cortical and hippocampal astrocytes elicited a specific calcium transient in neurons upon stimulation, however, the calcium transients were higher in neurons exposed to stimulated hippocampal rather than cortical astrocytes. Such calcium transient were blocked by the specific NMDA receptor antagonist APV, thus highlighting that the calciumtransients were mediated by the activation of these receptors following the release of astrocytic glutamate. More importantly, hippocampal astrocytes exposed to neuroinflammatory environment induced higher cell death and neuronal calcium transient compared to cortical astrocytes (Bianco F, N Tonna, Lovchik RD, Mastrangelo R, Morini R, Ruiz A, E Delamarche, Matteoli M. Anal. Chem. 2012) We also used the microfluidic system to perform a pharmacological study in an experimental model of Alzheimer's disease. In particular, we evaluated the effectiveness of the drug FTY720 (Fingolimod, normally used for the treatment of multiple sclerosis) to prevent cell death in neurons cultured alone or co-cultured with microglia upon the stimulation with either fibrillar or oligomeric Aß form. The results showed that the drug prevented cell death in neurons exposed to Aß oligomers, both in the presence and absence of microglia. (Ruiz A, Joshi P, R Mastrangelo, Francolini M, Verderio C, Matteoli M Lab Chip. 2014). In the second part of my PhD thesis, I have focused my attention on the role of different populations of astrocytes in supporting neuronal development in a model of Rett Syndrome. Observations made by Prof. Tongiorgi's lab, showed a different neuronal atrophy at the level of cortex and hippocampus in mouse model of Rett Syndrome, thus prompting us to investigate the effects of glial cells derived from these two different brain areas on neurons. Neurons were grown in conditioned medium obtained from hippocampal or cortical astrocytes established from WT or KO mouse, and the growth in terms of neurites length was evaluated. The morphological analysis showed that neurons grown in conditioned medium derived from hippocampal astrocytes displayed longer neuritic processes than those grown in cortical astrocytes-derived medium. In contrast, the growth of hippocampal neurons in conditioned medium from wt cortical astrocytes, hippocampal and cortical astrocytes Rett not significantly different compared to neurons cultured alone. This suggests that hippocampal astrocytes have a higher trophic effect on neuronal development than cortical astrocytes. In light of these results, we can assume that the atrophy observed in cortical region of Rett Syndrome mouse model may be due to a lower trophic capability of cortical astrocytes compared to hippocampal in supporting neuronal development. Taken together these data suggest that astrocytic populations belonging to distinct brain areas have different capability in supporting neuronal growth, thus opening the possibility that region-specific atrophy observed in RETT mouse model may stem from a different release of trophic factors from glial cells.XXVII Ciclo198