INTRODUÇÃO: A neuromielite óptica (NMO) recorrente não foi completamente estudada em pacientes brasileiros após a descoberta do autoanticorpo sérico NMO-IgG e seu antígeno específico aquaporina-4. Neste trabalho, descrevemos os aspectos clínicos, imunológicos e de ressonância magnética (RM) de pacientes portadores de NMO recorrente. MÉTODOS: Foram estudados retrospectivamente vinte e oito pacientes portadores de NMO recorrente regularmente acompanhados no Ambulatório de Doenças Desmielinizantes do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo e que preenchiam os critérios diagnósticos originalmente propostos em 1999. Foram analisados os dados relativos ao resultado da pesquisa sérica do NMO-IgG, características clínicas e de RM. RESULTADOS: Três homens e 25 mulheres foram estudados. A mediana de idade de instalação da doença foi 26 anos (intervalo: 755); a mediana do tempo de seguimento foi de sete anos (intervalo: 214). O tempo médio decorrido entre o primeiro e o segundo episódios clínicos foi de 17 meses (mediana: 8,5; intervalo 288). A mediana da Escala Expandida do Estado de Incapacidade (EDSS) na última avaliação foi de 5,5 (intervalo de 2 a 10). Não houve diferença estatística entre o resultado do NMO-IgG e o grau de incapacidade medido pela EDSS (p = 0,03; teste de Mann-Whitney). O autoanticorpo NMO-IgG foi detectado em 18 pacientes (64,3%). A desordem associada mais comumente encontrada foi a disfunção da tireóide (quatro casos de hipotireoidismo e um caso de hipertireoidismo subclínico). A alteração laboratorial mais encontrada foi a presença do fator antinúcleo (FAN) em 13 pacientes (46,4%). Dois pacientes apresentaram positividade concomitante para o FAN e para o SSA/SSB (um paciente com positividade para o FAN e SSA e outro paciente para o FAN e o SSB. Não houve diferença estatisticamente significativa entre a prevalência dos autoanticorpos FAN, SSA e SSB entre os pacientes NMO-IgG positivos e NMO-IgG negativos (p = 0,52; teste exato de Fisher). Quatro pacientes faleceram por insuficiência respiratória secundária a mielite cervical (3 casos) ou a tromboembolismo pulmonar (1 caso). Em relação aos achados de RM, a maioria dos pacientes apresentou mielite cervical (36%) e cérvico-torácica (46,4%) na análise longitudinal. O padrão mais comum de acometimento no plano axial foi holoespinhal (50%). Não houve associação estatística entre a extensão longitudinal da mielite e o resultado do autoanticorpo NMO-IgG. CONCLUSÕES: Nesta série de pacientes brasileiros portadores de NMO recorrente foi encontrada uma idade mais precoce de instalação da doença do que aquela previamente relatada na literatura. Em contraste com séries internacionais, não houve correlação entre maior extensão longitudinal da mielite e positividade aumentada do anticorpo NMO-IgG / INTRODUCTION: Neuromyelitis optica has not been thoroughly studied in Brazilian patients following the discovery of NMO-IgG and its specific antigen aquaporin-4. In this study we aimed to describe the clinical NMO-IgG immunological status and neuroimaging characteristics of recurrent neuromyelitis optica in a series of Brazilian patients. SUBJECTS and METHODS: We undertook a retrospective study of 28 patients with recurrent neuromyelitis optica, according to 1999 Wingerchuks diagnostic criteria followed at the Center for Myelin Disorders of the Neurologic Clinic of São Paulo University School of Medicine, São Paulo, Brazil. Data on NMO-IgG status, clinical features, and MRI findings were analyzed. RESULTS: Three men and 25 women were evaluated .Median age at onset of disease was 26 years (range 755); median time of follow-up was 7 years (range 214). The mean time elapsed between the first and the second attack was 17 months (median 8.5; range 288). Median Kurtzkes Expanded Disability Status Scale (EDSS) score on last visit was 5.5 (range 210).There was no statistical difference between positive and negative NMO-IgG groups regarding EDSS score (p = 0.03, Mann-Whitney U test). NMO-IgG was detected in 18 patients (64.3%). The most common associated abnormality was thyroid dysfunction. Hypothyroidism was found in four patients: isolated in two patients and associated with hyperprolactinemia and diabetes mellitus type 1 in two other patients. In one patient subclinical hyperthyroidism was detected. Autoantibodies were detected in 13 patients: anti-nuclear antibody (ANA), with titers ranging from 1/40 to 1/320, in all of them; one patient had both ANA and SSA and another had both ANA and SSB. There was no statistical difference in the prevalence of ANA and SSA/SSB antibodies between patients NMO-IgG positive and NMO-IgG negative (p = 0.52, Fishers exact test). Four patients died due to respiratory failure: three of them because of cervical myelitis and one of them because of pulmonary thromboembolism. Most patients presented with cervical (36%) and cervical-thoracic myelitis (46.4%). Holocord lesion was the most common pattern of involvement (50%) on the axial plane. We did not find a statistical association between myelitis extension and NMO-IgG result. CONCLUSIONS: Our series of Brazilian patients showed a younger age of onset than previously reported. In our series, in contrast to previous reports, there was no correlation between the extension of myelitis and NMO-IgG positivity

Le cholangiocarcinome (CC) est une tumeur maligne au pronostic péjoratif dont le traitement repose sur la résection chirurgicale. Il représente 3 % de l’ensemble des cancers digestifs et il est la deuxième tumeur primitive du foie, en fréquence, derrière le carcinome hépatocellulaire. L’âge moyen est de 70 ans avec une prédominance masculine. On distingue actuellement les cholangiocarcinomes intra et extra-hépatiques. La survie est inférieure à 5% à 5 ans tous stades confondues. 60 à 70 % sont des tumeurs de la convergence des canaux biliaires appelées également tumeurs de Klatskin.Le but de ce travail était de répondre aux interrogations persistantes concernant le cholangiocarcinome péri-hilaire (CCPH) en appliquant différentes méthodes statistiques sur différentes bases de données et revue de la littérature.Les trois axes principaux de ce travail s’articulent selon la temporalité de prise en charge, du diagnostic aux suites post-opératoires en passant par la mise en condition préopératoire.Le premier axe repose sur une base de données locale (registre de cancer digestif du Calvados). Les résultats montrent que le CCPH constitue seulement un tiers des cholangiocarcinomes dans la population générale, que son taux d’incidence est stable avec néanmoins une diminution d’incidence, bien que non-significative, chez les femmes ayant un CCPH et que le sexe féminin est un facteur pronostic négatif pour la survie à 5 ans. Le deuxième axe concernait la prise en charge préopératoire des patients, notamment l’optimisation préopératoire du foie restant par le drainage biliaire. Ce travail repose sur deux méta-analyses. Il a permis de mettre en évidence la supériorité de la voie radiologique sur la voie endoscopique concernant les complications liées à la procédure mais en revanche, l’absence de différence significative sur la morbi-mortalité post-résection hépatique, la survie à 5 ans, la survie sans récidive et le taux de dissémination liée à la procédure quand les procédures sont étudiées en intention de traiter. Nos résultats suggèrent qu’un mauvais choix de voie d’abord pour réaliser le drainage biliaire conduit à des échecs répétés qui influencent la récidive tumorale et donc la survie. Le troisième axe s’intéressait aux facteurs pronostiques de morbi-mortalité immédiates post-résection hépatique à partir d’une base de données Européenne (base de l’association Française de chirurgie). Les résultats montrent que la surface corporelle ≥ 1.82 m², l’hyperbilirubinémie > 50 µmol/l et la résection hépatique droite sont des facteurs prédictifs indépendants influençant la mortalité post-opératoire à 30 jours. / Cholangiocarcinoma (CC) is a malignant tumor with a poor prognosis. Its treatment is based on surgical resection. It accounts for 3% of all digestive cancers and is the second primary tumor of the liver, in frequency, after hepatocellular carcinoma. The average age is 70 years old with male predominance. At present intra and extrahepatic cholangiocarcinomas are distinguished. Survival rate is less than 5% at 5 years in all stages. 60 to 70% are tumors of the biliary convergence also called Klatskin tumors.The aim of this work was to answer persistent questions about peri-hilar cholangiocarcinoma (PHCC) by applying different statistical methods on different databases and review of the literature.The three main axes of this work are articulated according to the temporality of management, from the diagnosis to the postoperative follow-up, going through the preoperative setting.The first axis is based on a local database (registry of digestive cancer of Calvados). The results show that PHCC accounts for only one third of cholangiocarcinomas in the general population, that its incidence rate is stable with a decrease in incidence, although not significant, in women having PHCC and that female gender is a negative prognostic factor for 5-year survival. The second axis concerned the preoperative management of patients, including preoperative optimization of the remaining liver by biliary drainage. This work is based on two Meta-analyzes. It made it possible to highlight the superiority of the radiological way in the endoscopic way concerning the complications related to the procedure but on the other hand, the absence of significant difference on the morbi-mortality post hepatic resection, the survival at 5 years, the recurrence free survival and the rate of dissemination related to the procedure when the procedures are studied in intent to treat. Our results suggest that a poor choice of pathway for achieving biliary drainage leads to repeated failures that influence tumor recurrence and thus survival. The third axis was concerned with the prognostic factors of immediate morbidity and mortality after hepatic resection from a European database (base of the French association of surgery). The results show that body surface area ≥ 1.82 m², hyperbilirubinemia > 50 μmol / l and right hepatic resection are independent predictors influencing post-operative mortality at 30 days.

We present an unusual case of a late recurrent central neurocytoma that was rediagnosed as an ependymoma and neurocytoma in accordance with changes in histological classifications. Case Description. A 56-year-old male teacher presented with incomplete transverse syndrome due to several intradural extramedullary tumors at the level of lumbar vertebrae 1–3. The histological diagnosis at the time was atypical ependymoma. One year later, two additional tumors were removed at the L5-S1 vertebral level. For 12 years, the patient remained tumor free on followup. Fourteen years after the initial diagnosis, the patient presented with thoracic paresthesias due to two new extramedullary tumors in the C7-T1 and the T8-T9 vertebral levels. After complete removal of the tumors, a radiological survey revealed an intracranial lesion in the third ventricle. Five months later, an additional lesion recurrence was removed surgically. The most recent histological diagnosis revealed an atypical central neurocytoma. In retrospect, the previous tumors were reclassified as neurocytoma according to the additional immunohistochemistry evidence. Discussion. There is no standard adjuvant treatment regimen for atypical neurocytoma; therefore, the patient is currently under close followup. Modern histopathological diagnosis is essential in these cases. Potential routes for dissemination of the tumor should be considered upon first recurrence.

Objective: Primary transitional cell carcinoma (TCC) of the upper urinary tract represents 6–8% of all TCC cases. Nephroureterectomy with removal of a bladder cuff is the treatment of choice. The rates of TCC recurrence in the bladder after primary upper urinary tract surgery described in the literature range between 12.5 and 37.5%. In a retrospective analysis we examined the occurrence of TCC after nephroureterectomy for upper tract TCC in patients without a previous history of bladder TCC at the time of surgery. Methods: Between 1990 and 2002, 29 patients underwent primary nephroureterectomy for upper tract TCC. The mean age of the patients was 69.5 years. In 5 cases upper urinary tract tumors were multilocular, in the remaining cases unilocular in the renal pelvis (n = 12) or the ureter (n = 12). The follow-up was available for 29 patients with a mean follow-up of 3.37 (0.1–11.2) years. Results: 11/29 (37.9%) patients had TCC recurrence with 9/11 patients having bladder TCC diagnosed within 2.5 years (0.9–6.0) after nephroureterectomy. 13/29 patients are alive without TCC recurrence, 3/29 patients died due to systemic TCC progression and 5/29 died of unrelated causes without evidence of TCC recurrence. Conclusion: Our data indicate a high incidence of bladder TCC after nephroureterectomy for primary upper tract TCC of up to 6 years after primary surgery. Because of the high incidence of bladder TCC within the first 3 years of surgery, careful follow-up is needed over at least this period. / Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.

Higher-dimensional Hamiltonian systems usually exhibit a mixed phase space in which regular and chaotic motion coexist. While regular trajectories are confined to regular tori, chaotic trajectories can be transported through a web of so called resonance channels which disrupt the regular structures. The focus of this thesis are time-discrete 4D symplectic maps which represent the lowest dimensional system for which the chaotic transport can circumvent regular tori. While the dynamics of 2D maps are well established, many fundamental questions are open for maps of dimension four and higher due to this property. In particular, the mechanism of the power-law trapping is unknown for these maps. In this thesis, the organization and hierarchy of the regular structures of 4D maps is uncovered and the slow chaotic transport close to these structures is examined. Specifically, this transport is shown to be organized by a set of overlapping resonance channels. The transport across these channels is found to be governed by partial transport barriers. For the transport along a channel a stochastic process including a drift is conjectured. Based on each of these two types of chaotic transport a possible mechanism for the power-law trapping in higher-dimensional systems is proposed.

Time has passed since truth and reconciliation commissions (TRCs) started to grow in popularity. The general patterns of their effect on the post-conflict societies are still unexplored. The main claim is that establishing a TRC will reduce the likelihood of conflict recurrence compared to other forms of transitional justice as TRCs are especially well equipped to mitigate reasons for conflict recurrence. This paper uses quantitative methods with the PCJ dataset and the UCDP dyadic dataset to analyse the trends of different types of transitional justice between the years 1946-2006. The logistic regression showed a negative relationship between establishment of TRCs and conflict recurrence. The main implication of these findings is that there are general patterns of TRCs having an effect, which need to be further researched.

Objective: In this study, 22 cases of locally recurrent urological malignant fibrous histiocytoma were reviewed considering therapeutic options, follow-up and prognosis. Patients and Methods: In the available literature on this topic we identified 19 cases of locally recurrent genitourinary malignant fibrous histiocytoma. Three additional cases are discussed, primarily arising from the kidney, the bladder and the paratesticular region. Results: The prognosis of locally recurrent urological malignant fibrous histiocytoma was found to be extraordinarily poor. Only 2 of 22 patients have survived for longer than 3.5 years. One of them reported herein is still alive 10 years after extensive lymphatic spread accompanying the first local recurrence. In this case, late local recurrence occurred after an 8-year interval free of disease. Conclusion: Malignant fibrous histiocytoma is an unusual urological malignancy with a high rate of local recurrence. The latter is frequently accompanied by metastatic disease and unrelenting progression. Despite the poor prognosis early detection of local failure and aggressive salvage therapy might offer the chance of long-term survival in selected cases. Close and life-long follow-up is advisable for patients once treated for recurrent urological malignant fibrous histiocytoma. / Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.

Based on the generating matrix to the Tribonacci sequence, the Tribonacci cat map is a discrete chaotic dynamical system, similar to Arnold's discrete cat map, but on three dimensional space. In this thesis, this new mapping is introduced and the properties of its matrix are presented. The main results of the investigation prove how the size of the domain of the map affects its period and explore the orbit lengths of non-trivial points. Different upper bounds to the map are studied and proved, and a conjecture based on numerical calculations is proposed. The Tribonacci cat map is used for applications such as 3D image encryption and colour encryption. In the latter case, the results provided by the mapping are compared to those from a generalised form of the map.

The Thesis aims to explore the problem of Freedom and Creativity in Nietzsche. It attempts to do so in respect to the two main concepts of his high and late writings. Concept of Superman and concept of the Eternal recurrence of the same. In this respect our text follows especially interpretations of Martin Heidegger. We begin our treatise with situating Nietzsche's concept of freedom among more traditional thought and we put emphasis on comparison of it to the philosophy of Baruch Spinoza. In the next phase we reject (with Martin Heidegger and Ivan Dubský) simplifying understanding of the Eternal reccurence which understands it as dull determinism. We also analyse the topic of Creativity in Nietzsche and introduce it as fundamental anthropological theme. In the following chapters we identify certain moods that allow us to experience perspective of the Eternal reccurence and the one of Superman, which gives us chance to understand both concepts in their connection. But this connection remains enigmatic and in the last chapter we try, with help of Kierkegaard's concept of the moment, to understand both thoughts in their complementarity not only united in the concept of creativity, but also through Nietzsche's concept of the moment, understood as the original understanding of one of the aspects of...

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