Mind the gap: IR and the challenge of international politics

The discipline of International Relations (IR) for a long time of its history has developed in the form of Great Debates that involved competing paradigms and schools. More recently, it has been described as a cacophony of voices unable to communicate among themselves, but also incapable to provide keys to understand an ever more complex reality. This collection aims at evaluating the heuristic value of a selection of traditional paradigmsrealism and liberalism), schools (constructivism), and subdisciplines (security studies and international political economy) so as to assess the challenges before IR theory today and the ability of the discipline to provide tools to make the changed world still intelligible

Regionalism and African agency : negotiating an Economic Partnership Agreement between the European Union and SADC-Minus

This article investigates the regional dynamics of African agency in the case of negotiations for an Economic Partnership Agreement (EPA) between the EU and a group of Southern African countries, known as SADC-Minus. I argue that these negotiations were shaped by a pattern of differentiated responses to the choice set on offer under the EPAs by SADC-Minus policymakers and by a series of strategic interactions and power plays between them. I offer two contributions to an emerging literature on the role of African agency in international politics. First, I argue for a clear separation between ontological claims about the structure-agency relationship and empirical questions about the preferences, strategies and influence of African actors. Second, I suggest that in order to understand the regional dynamics of African agency it is important to pay close attention to the diversity and contingency of African preferences and to the role of both power politics and rhetorical contestation in regional political processes

Allogeneic haematopoietic stem cell transplantation for mitochondrial neurogastrointestinal encephalomyopathy

Haematopoietic stem cell transplantation has been proposed as treatment for mitochondrial neurogastrointestinal encephalomyopathy, a rare fatal autosomal recessive disease due to TYMP mutations that result in thymidine phosphorylase deficiency. We conducted a retrospective analysis of all known patients suffering from mitochondrial neurogastrointestinal encephalomyopathy who underwent allogeneic haematopoietic stem cell transplantation between 2005 and 2011. Twenty-four patients, 11 males and 13 females, median age 25 years (range 10-41 years) treated with haematopoietic stem cell transplantation from related (n = 9) or unrelated donors (n = 15) in 15 institutions worldwide were analysed for outcome and its associated factors. Overall, 9 of 24 patients (37.5%) were alive at last follow-up with a median follow-up of these surviving patients of 1430 days. Deaths were attributed to transplant in nine (including two after a second transplant due to graft failure), and to mitochondrial neurogastrointestinal encephalomyopathy in six patients. Thymidine phosphorylase activity rose from undetectable to normal levels (median 697 nmol/h/mg protein, range 262-1285) in all survivors. Seven patients (29%) who were engrafted and living more than 2 years after transplantation, showed improvement of body mass index, gastrointestinal manifestations, and peripheral neuropathy. Univariate statistical analysis demonstrated that survival was associated with two defined pre-transplant characteristics: human leukocyte antigen match (10/10 versus <10/10) and disease characteristics (liver disease, history of gastrointestinal pseudo-obstruction or both). Allogeneic haematopoietic stem cell transplantation can restore thymidine phosphorylase enzyme function in patients with mitochondrial neurogastrointestinal encephalomyopathy and improve clinical manifestations of mitochondrial neurogastrointestinal encephalomyopathy in the long term. Allogeneic haematopoietic stem cell transplantation should be considered for selected patients with an optimal donor

Le organizzazioni internazionali e le Relazioni Internazionali

Quali sono le funzioni delle organizzazioni internazionali? Come cambiano nel tempo? Con quali criteri si valutano l\u2019efficacia e la legittimit\ue0 della loro azione? Queste sono alcune tra le principali domande di ricerca che gli studiosi delle Relazioni Internazionali si pongono nello studio delle organizzazioni internazionali. Realisti, studiosi della teoria critica, liberali e costruttivisti, partendo da assunti diversi, forniscono risposte per molti aspetti contrastanti a questi quesiti, offrendo molteplici spunti di ricerca empirica agli studenti della politica internazionale. L'obiettivo di questo capitolo \ue8 proprio quello di esaminare le principali caratteristiche delle organizzazioni internazionali secondo le teorie di Relazioni Internazional

The EU as Promoter of Environmental Norms in the Doha Round

This article investigates the reasons why the EU tried to promote environmental norms in the Doha round. It argues that the EU's support of a \u2018greener\u2019 World Trade Organization stems from tensions between the rigidity of the domestic dynamics of positive integration in the EU and the increased bindingness of negative integration commitments undertaken under the WTO. Consensual decision-making procedures in the EU led societal groups to push for stringent food safety and environmental regulations in the EU, and made them very resistant to change. These dynamics of positive integration, however, produced rules that were inconsistent with negative integration commitments undertaken under the WTO, at a time when the creation of a quasi-judicial dispute settlement mechanism in the trade regime had greatly increased the bindingness of WTO rules. As a result of the twofold effect of domestic and international institutional constraints, EU decision-makers were subject to compelling incentives to try and strengthen legitimate exceptions from WTO rules and immunise European regulation against WTO legal challenges. Empirical evidence on how the EU shaped its trade-and-environment agenda in the run-up to the Doha Round in 2001, as well as how it negotiated in the subsequent period, lends support to the argument

Le Organizzazioni Internazionali: struttura, funzioni e impatto

Il libro si apre con un capitolo introduttivo sul fenomeno delle organizzazioni internazionali, il ruolo di esse nella politica internazionale e l\u2019analisi dei principali approcci teorici. Il capitolo si propone di fornire un bilancio dello stato dell\u2019arte. I successivi capitoli empirici sono suddivisi in tre parti. La prima parte analizza il ruolo delle organizzazioni internazionali nelle politiche di promozione della pace e della sicurezza. Le Nazioni Unite, l\u2019Unione Europea e la NATO sono analizzate con particolare riferimento, rispettivamente, alle operazioni di pace, alla politica estera, e alla sicurezza collettiva. La seconda parte discute aspetti economici e finanziari e si occupa in particolare dell\u2019assistenza finanziaria fornita dal Fondo Monetario Internazionale, delle operazioni di liberalizzazione dei servizi compiute dall\u2019Organizzazione Mondiale del Commercio, della lotta alla povert\ue0 promossa dalla Banca Mondiale, delle forme di integrazione economica concepite da alcune organizzazioni regionali. La terza parte si concentra sulle questioni umanitarie e sociali. Il primo capitolo discute il ruolo della Corte Penale Internazionale nella prevenzione dei conflitti; il secondo capitolo analizza gli organismi delle Nazioni Unite che si occupano di ambiente; il terzo \ue8 dedicato all\u2019Alto Commissariato per i Rifugiati e al problema umanitario. Il capitolo conclusivo riflette sul ruolo delle organizzazioni internazionali nella governance globale e sul processo di progressiva ridefinizione della sovranit\ue0 statale

Fulminant intravascular lymphomatosis mimicking acute haemorrhagic leukoencephalopathy.

Intravascular lymphomatosis (IVL) is a rare non-Hodgkin's lymphoma, usually of B cell lineage, characterized by massive angiotropic growth. The clinical presentation of IVL may include changes in mental status, non-localizing neurological deficits, seizures, fever of unknown origin and skin changes. Because of its rarity and the absence of specific diagnostic procedures except for cerebral biopsy, diagnosis is often postmortem. Brain MRI usually shows non-specific abnormalities. The purpose of this case report is to increase the knowledge of clinical and neuroimaging features of IVL by describing the findings observed in a 71-year-old patient. CASE REPORT: A 71-year-old male was admitted for right hemiparesis, acute cognitive impairment and febricula. A bone marrow biopsy resulted normal. He then developed a rapid progressive impairment of his mental status and left hemisoma motor seizures. Brain CT and MRI were interpreted as consistent with acute haemorrhagic leukoencephalopathy (AHLE), including multiple areas of restricted diffusion without gadolinium enhancement and a small focal area of gadolinium enhancement in the left temporal lobe white matter. The patient died within a few days and the autopsy led to the diagnosis of IVL. CONCLUSION: IVL may present with a variety of clinical signs and symptoms, including stroke and hemiparesis. IVL may mimic AHLE at brain MRI. However, the evidence of multiple areas of restricted diffusion without gadolinium enhancement and of a small area of gadolinium enhancement could have led to the correct diagnosis. IVL should be added to the differential diagnosis of AHLE at brain MRI. Copyright © 2012 Elsevier B.V. All rights reserved

A syndrome of autosomal recessive pontocerebellar hypoplasia with white matter abnormalities and protracted course in two brothers.

We describe two brothers with an autosomal recessive syndrome characterized by neonatal onset, severe impairment of cognitive and motor functions, abnormal ocular movements and slight dystonic postures. Brain MR and CT scan showed a reduction in size of the cerebellum and to a lesser extent pons, accompanied by cerebral and cerebellar white matter abnormalities. These data suggest that they have a particular phenotype of pontocerebellar hypoplasia. Extensive laboratory investigation excluded known metabolic causes of pontocerebellar hypoplasia. We discuss the nosological status of pontocerebellar hypoplasia in relation to other early-onset pontocerebellar disorders