Cerebrotendinous xanthomatosis with bradyphrenia and psychiatric disorders: a case with 18F-FDG PET imaging and a literature review

Background and Objectives: Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive lipid-storage disease caused by mutations in the CYP27A1. The purpose of this study is to determine the clinical characteristics, neuroimaging and mutation detect in a family with CTX systematically. Methods: Collecting history materials and detecting the routine clinical biochemical tests and imaging examination, and for the first time taking the whole body positron emission tomography (PET)-CT examination for probed in the world to research abnormal metabolism activities in CTX. To observe the effect of treatment with chenodeoxycholic acid (CDCA) and stains before and after the intervention, using serum lipid level detection and neuropsychological evaluation. Genetic testing was carried out to screen the nine exons and exon-intron boundaries about 200-300bq of CYP27A1. Results: A 37-year-old woman with typical clinical characteristics of CTX. Magnetic resonance imaging (MRI) of brain showed bilateral lesions in the dentate nucleus of the cerebellum, then, PET images revealed multiple abnormal hypermetabolism areas at distal tendon, and multifocal areas of hypometabolism in bilateral sides of cerebellar hemispheres, the frontal lobe and temporal lobe. Histopathology reveals accumulation of xanthoma cells and dispersed lipid crystal clefts in xanthomas. In genetic analysis, it shown an insertion of cytosine (77-78insC) located in the first exon of CYP27A1 in the proband. Conclusions: We found that a Chinese patient presented a typical clinical feature of CTX along with clear correlation on both structural and functional imaging had a novel mutation in the CYP27A1 gene.

The Mary Carleton narratives, 1663-1673, a missing chapter in the history of the English novel,

The singular life and curious biographies of Mary Carleton.--The Carleton publications of 1663.--The minor publications of 1673.--"The counterfeit lady," a fiction.--The narrative technique of "The counterfeit lady."--The historical significance of "The counterfeit lady."--The current doctrine of the rise of the English novel.--Bibliographical difficulties.

Reconciling History and International Law: Territorial and Maritime Claims in the South China Sea and East China Sea

High-ranking Chinese military officials are often quoted in international media as stating that China cannot afford to lose even an inch of Chinese territory, as this territory has been passed down from Chinese ancestors. Such statements are not new in Chinese politics, but recently this narrative has made an important transition. While previously limited to disputes over land borders, such rhetoric is now routinely applied to disputes involving islands and maritime borders. China is increasingly oriented toward its maritime borders and seems unwilling to compromise on delimitation disputes, a transition mirrored by many states across the globe. In a similar vein, scholarship has found that territorial disputes are particularly intractable and volatile when compared with other types of disputes, and a large body of research has grappled with producing systematic knowledge of territorial conflict. Yet in this wide body of literature, an important question has remained largely unanswered - how do states determine which geographical areas will be included in their territorial and maritime claims? In other words, if nations are willing to fight and die for an inch of national territory, how do governments draw the boundaries of the nation? This dissertation uses in-depth case studies of some of the most prominent territorial and maritime disputes in East Asia to argue that domestic political processes play a dominant and previously under-explored role in both shaping claims and determining the nature of territorial and maritime disputes. China and Taiwan are particularly well suited for this type of investigation, as they are separate claimants in multiple disputes, yet they both draw upon the same historical record when establishing and justifying their claims. Leveraging fieldwork in Taiwan, China, and the US, this dissertation includes in-depth case studies of China’s and Taiwan’s respective claims in both the South China Sea and East China Sea disputes. Evidence from this dissertation indicates that officials in both China and Taiwan have struggled with how to reconcile history and international law when establishing their claims, and that this struggle has introduced ambiguity into China's and Taiwan's claims. Amid this process, domestic political dynamics have played a dominant role in shaping the options available and the potential for claims to change in the future. In Taiwan’s democratic system, where national identity is highly contested through party politics, opinions vary along a broad spectrum as to the proper borders of the nation, and there is considerable evidence that Taiwan’s claims may change in the near future. In contrast, within China’s single-party authoritarian political system, where nationalism is source of regime legitimacy, views on the proper interpretation of China’s boundaries do vary, but along a much more narrow range. In the dissertation’s final chapter, additional cases, such as South Korea’s position on Dokdo and Indonesia’s approach to the defense of Natuna are used as points of comparison to further clarify theoretical findings.

Pytanie o pamięć. Relacje autobiograficzne więźniów obozów koncentracyjnych

This book consists of two main parts. The first part offers a basic methodological introduction, presenting a concise but multifaceted overview of current problems of collective memory. The second part contains a set of interviews with former prisoners of concentration camps carried out by the authors. The research was conducted by Paweł Greń and Łukasz Posłuszny and focuses on issues of collective and cultural memory illustrated by individual life experiences of concentration camps prisoners. The field of oral history serves as the framework of analysis and narrative inquiry as its research tool. Interviews and additional research materials were collected by the authors and are not available in previous publications, making this work a precious supplement to the current scholarly body of knowledge and achievements in the discipline of memory studies. According to the authors, current historical and literary publications provide an incomplete picture of the WWII and its aftermaths for survivors, because descriptions of the war and imprisonment in the camp play still a dominate role in narratives. The importance of these issues in autobiographies is unquestionable and highly needed to create a common identity among generation of prisoners, though authors often wanted to perceive the fate of individuals in a broader perspective – including the periods before and after the war. Hence, interviews stressed personal experiences and their understanding over time by former prisoners. The interviews covered many topics on life before, during and after the camp – among them daily and neutral routines, but also difficult matters. The latter were connected on the one hand with traumatic events or harsh memories and emotions, and on the other hand with less extensively highlighted threads of prisoners’ lives - such as issues of the body and sexuality – and their dependence on particular representation or narrative. The authors are convinced that the book serves not only as a record of past remembered by eyewitnesses, but it also depicts their accounts in wider contexts and discourses, which expose specific dimensions of told and written stories. In the book Questions for Memory one examine the approach proposed by young scholars. Interviews were conducted from 2009-2011, seventy years after the end of the second world war, and this initiative was the result of questions and doubts of the authors from the existing literature. They also wanted to use the unique opportunity to meet with eyewitnesses and record their stories, because when they pass away we will irretrievably lose the possibility to listen to them and to pose sensitive questions. The majority of the interviewees were prisoners of KL Auschwitz-Birkenau, and their experiences differed greatly from each other based on social background and specific experience in the camps as well as their post-camp and postwar life. Aside from persons whose stories are already well known and open, readers will hear the stories of those who spoke only reluctantly and very rarely, or who had remained silent until the present author’s research. Qualitative differences between interviews occurred on the level of established relationship and atmosphere of trust, which varied according to circumstances and individual character and personality. For P. Greń and Ł. Posłuszny, each interviewed person is equally and highly valued due to the collected material and the personal experience of the meetings. Among the ten interviews placed in the book, seven of them are the stories told by women. Their testimonies exemplify realities of everyday prisoners’ existence and gravitate towards mirroring specifically feminine perspectives of imprisonment. For women, crucial problems stemmed from experiences of body that intertwine with suffering, feeling of shame and humiliation. Early discussions on holocaust literature and issues of representation that shaped the Polish narrative and collective memory imposed imperatives of silence on certain topics. A solution for reconciling heroic and inhuman deeds in stories with completely human physiology was impossible and improper for many years. There were also questions about life after, ways of dealing with a trauma or reflections on the present time. During conversations the authors attempted to come closer to something distant and incomprehensible for their generation and for people who did not experience the camps. Despite the fact that there have been seventy years of dealing with these events in literature, art, drama, film, memoirs and scientific works, the past still breeds more questions than answers. The book Questions for Memory serves as an example of this phenomenon.

La scène de reconnaissance : allégorie politique et métaphore de l’adaptation dans "The History of King Lear" de Nahum Tate (1681)

International audience

A Worldwide History Spanning Genocide for the Sake of Corporate Profit

A Worldwide History Spanning Genocide for the Sake of Corporate Profit is the title of a thesis consisting of four stories. Crosky’s Knife is presented first for the sake of allowing potential readers to embrace a sense of amiable audience engagement and is based on a true story of a veteran of one of the many global conflicts currently raged on behalf of freedom. The three pieces following this feint are reworked versions of stories written from the heart and delivered to machines. As it presents numerous aspects of reality that the average person may not wish to consider, doing so with shockingly casual acceptance of such horror and/or banality, the conscious reception of the duty of engagement and possible appreciation of the text is not advised. Knives, rabid dogs, severed tongues, and a downpour of malnourished Iraqi babies are components intrinsic to the direction of this thesis.

Solidarity, the network and the history of workers' self-management from the Gdańsk Agreement to shock therapy

This PhD thesis is an empirical research project in the field of modern Polish history. The thesis focuses on Solidarity, the Network and the idea of workers’ self-management. In addition, the thesis is based on an in-depth analysis of Solidarity archival material. The Solidarity trade union was born in August 1980 after talks between the communist government and strike leaders at the Gdansk Lenin Shipyards. In 1981 a group called the Network rose up, due to cooperation between Poland’s great industrial factory plants. The Network grew out of Solidarity; it was made up of Solidarity activists, and the group acted as an economic partner to the union. The Network was the base of a grass-roots, nationwide workers’ self-management movement. Solidarity and the self-management movement were crushed by the imposition of Martial Law in December 1981. Solidarity revived itself immediately, and the union created an underground society. The Network also revived in the underground, and it continued to promote self-management activity where this was possible. When Solidarity regained its legal status in April 1989, workers’ self-management no longer had the same importance in the union. Solidarity’s new politico-economic strategy focused on free markets, foreign investment and privatization. This research project ends in July 1990, when the new Solidarity-backed government enacted a privatization law. The government decided to transform the property ownership structure through a centralized privatization process, which was a blow for supporters of workers’ self-management. This PhD thesis provides new insight into the evolution of the Solidarity union from 1980-1990 by analyzing the fate of workers’ self-management. This project also examines the role of the Network throughout the 1980s. There is analysis of the important link between workers’ self-management and the core ideas of Solidarity. In addition, the link between political and economic reform is an important theme in this research project. The Network was aware that authentic workers’ self-management required reforms to the authoritarian political system. Workers’ self-management competed against other politico-economic ideas during the 1980s in Poland. The outcome of this competition between different reform concepts has shaped modern-day Polish politics, economics and society.

The Drama of History: Representation and Revolutionaries in Haitian Theater, 1818-1907

Since the beginning of the Haitian theatrical tradition there has been an ineluctable dedication to the representation of Haitian history on stage. Given the rich theatrical archive about Haiti throughout the world, this study considers operas and plays written solely by Haitian playwrights. By delving into the works of Juste Chanlatte, Massillon Coicou, and Vendenesse Ducasse this study proposes a re-reading of Haitian theater that considers the stage as an innovative site for contesting negative and clichéd representations of the Haitian Revolution and its revolutionary leadership. A genre long mired in accusations of mimicking European literary forms, this study proposes a reevaluation of Haitian theater and its literary origins.

Placental site trophoblastic tumor: a case report and review of the literature

We present a case of a gravida 1 para 1 woman, who presented with an 11- month history of amenorrhea after cesarean delivery. The patient was taking birth control pills at the time of presentation. She was observed with a slight elevation of serum β-hCG level, an enlarged heterogeneous uterus and hematometra. A biopsy was performed, and the patient was diagnosed with placental site trophoblastic tumor; the patient then underwent surgery. Placental site trophoblastic tumor is the rarest form of gestational trophoblastic disease, derived from intermediate trophoblast cells. It does not have a pathognomonic appearance; therefore, correlation with medical history, as well as results of laboratory tests and pathological analysis is mandatory. It is a relatively chemoresistant tumor, posing considerable therapeutic challenges; patients with localized disease are managed with surgery and those with metastatic disease require additional chemotherapy. Herein, we review the main features of this entity and top differential diagnosis, as the rarity of this tumor is associated with imaging and pathological pitfalls, reinforcing the need for further experience in this field.

Papillary carcinoma arising in struma ovarii versus ovarian metastasis from primary thyroid carcinoma: a case report and review of the literature

We present a case of a postmenopausal woman diagnosed with an ovarian mass containing thyroid follicles and foci of papillary thyroid carcinoma during pathological examination. This patient referred having had a metachronous thyroid malignancy 10 years before. The differential diagnosis between a thyroid malignancy arising from a struma ovarii and a metastatic ovarian tumor originating from thyroid-cancer is challenging. Struma ovarii should be considered when thyroid components are the predominant element or when thyroid malignant tissue is identified within an ovarian lesion. Thyroid carcinoma arising from a struma ovarii is reported to occur in a minority of cases. Of these, papillary carcinoma is the most frequent subtype encountered. Regarding primary thyroid carcinomas, papillary carcinomas have a lower metastatic potential when compared to follicular carcinomas, and most of the metastases occur in the cervical lymph nodes. Ovarian metastases are exceedingly rare and generally associated with widespread disease. However, they must be considered in the presence of previous history of malignant thyroid carcinoma. The authors review the main clinical, imaging and therapeutic aspects of both these entities and present the most likely diagnosis.

Rectal inflammatory fibroid polyp in a Nigerian: case report & brief review of literature.

Background: Inflammatory fibroid polyps are very unusual gastrointestinal tumours. Furthermore, their occurrence in the rectum is a rarity indeed. Objective: To document this very rare pathologic entity in a patient from our region of Africa and highlight some pertinent clinical and pathologic aspects of the disease. Case report: We present a 59 year old Nigerian who had neither significant previous medical history nor current complaint and had a routine colonoscopy done. The finding in the rectum was of a small polypoidal mass which was excised whole and whose histopathologic findings were consistent with a diagnosis of inflammatory fibroid polyp. Conclusion: A unique case of rectal inflammatory fibroid polyp in a Nigerian is hereby presented. This, to the best of our knowledge, is the first such documented case from our region of the world. Hence, the entity should be considered in the differential diagnosis of colonic polyps from the continent.

Faulkner revisited : narrating property, race, gender and history in William Faulkner's Go Down, Moses, Toni Morrison's Song of Solomon and Gloria Naylor's Mama Day

My thesis explores the formation of the subject in the novels of Faulkner’s Go Down, Moses, Toni Morrison’s Song of Solomon, and Gloria Naylor’s Mama Day. I attach the concept of property in terms of how male protagonists are obsessed with materialistic ownership and with the subordination of women who, as properties, consolidate their manhood. The three novelists despite their racial, gendered, and literary differences share the view that identity and truth are mere social and cultural constructs. I incorporate the work of Judith Butler and other poststructuralist figures, who see identity as a matter of performance rather than a natural entity. My thesis explores the theme of freedom, which I attached to the ways characters use their bodies either to confine or to emancipate themselves from the restricting world of race, class, and gender. The three novelists deconstruct any system of belief that promulgates the objectivity of truth in historical documents. History in the three novels, as with the protagonists, perception of identity, remains a social construct laden with distortions to serve particular political or ideological agendas. My thesis gives voice to African American female characters who are associated with love and racial and gender resistance. They become the reservoirs of the African American legacy in terms of their association with the oral and intuitionist mode of knowing, which subverts the male characters’ obsession with property and with the mainstream empiricist world. In this dissertation, I use the concept of hybridity as a literary and theoretical devise that African-American writers employ. In effect, I embark on the postcolonial studies of Henry Louise Gates, Paul Gilroy, W. E. B Du Bois, James Clifford, and Arjun Appadurai in order to reflect upon the fluidity of Morrison’s and Naylor’s works. I show how these two novelists subvert Faulkner’s essentialist perception of truth, and of racial and gendered identity. They associate the myth of the Flying African with the notion of hybridity by making their male protagonists criss-cross Northern and Southern regions. I refer to Mae Gwendolyn Henderson’s article on “Speaking in Tongues” in my analysis of how Naylor subverts the patriarchal text of both Faulkner and Morrison in embarking on a more feminine version of the flying African, which she relates to an ex-slave, Sapphira Wade, a volatile female character who resists fixed claim over her story and identity. In dealing with the concept of hybridity, I show that Naylor rewrites both authors’ South by making Willow Springs a more fluid space, an assumption that unsettles the scores of critics who associate the island with authenticity and exclusive rootedness.

A review on the recent history of wind power ramp forecasting.

Forecasting large and fast variations of wind power (the so called ramps) helps achieve the integration of large amounts of wind energy. This paper presents a survey on wind power ramp forecasting, reflecting the increasing interest on this topic observed since 2007. Three main aspects were identified from the literature: wind power ramp definition, ramp underlying meteorological causes and experi-ences in predicting ramps. In this framework, we additionally outline a number of recommendations and potential lines of research.

Beyond the Spanish classics - The ephemeral awakening of the history of international law in pre-democratic Spain

Cuadernos para el Diálogo (1963-1978) played a key-role in nurturing the intellectual soil for the Spanish Transition to democracy and it has spawned an extensive amount of literature among historians. This work links for the first time the course of this emblematic monthly journal with the short-lived period of methodological and historiographical innovation of Revista Española de Derecho Internacional under the direction of the international jurist Mariano Aguilar Navarro.