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Objectives Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarctions and Leukoencephalopathy (CADASIL) is a rare genetic disorder. It is caused by a mutation in the NOTCH3 gene on chromosome 19 which damages vascular smooth muscle cells resulting in impaired cerebral blood flow and a progressive vascular dementia. Onset typically occurs by the mid-30’s but varies widely. This case study describes a unique case of rapid disease progression. Methods The patient was a 58-year-old right-handed male. Initial symptom onset occurred at the age of 53, with blurred vision, severe headache, and difficulty speaking. Brain imaging (CT, MRI) showed significant white matter degeneration and cortical volume loss. Multiple sclerosis was hypothesized. Following the development of seizures and a right hemisphere stroke, the patient underwent genetic testing and was diagnosed with CADASIL. Initial neuropsychological evaluation revealed deficits in perceptual reasoning, working memory, processing speed, memory, and executive functioning. Serial evaluation included intelligence testing, academic achievement, the Meyers Neuropsychological Battery, and psychological functioning. Results Intellectual functioning and the overall neuropsychological test battery mean were one and a half to two standard deviations below his estimated premorbid functioning. Executive functioning declined from prior evaluation. Psychologically, the patient reported mild anger, irritability, and interpersonal difficulties. His wife endorsed significant increases in apathy and disinhibition and decline in executive functioning. The patient passed away approximately six months post-evaluation. Conclusion Findings were consistent with moderate-to-severe diffuse and lateralized deficits (RCH > LCH) resulting from subcortical/higher cortical vascular dementia due to CADASIL and its medical sequelae, with quite rapid symptom onset and decline.

Parmi les processus en cours dans les sociétés contemporaines, la migration internationale est, sans doute, celle qui est au centre des controverses majeures aussi bien pour les conséquences sociales que pour les conséquences politiques qui derivent de la mobilité humaine. Pour cette raison, les dynamiques migratoires et les politiques visant à les gérer, ainsi que les impacts en termes d'intégration socio-économique sont depuis de nombreuses années au centre du débat public et politique dans la plupart des pays à économies avancées. L'accroissement des flux migratoires internationaux au cours des dernières décennies et la dimension importante atteinte par la population étrangère dans de nombreux pays , ainsi que les crises multiples qui se sont produites globalement ont également contribué à augmenter les intérêts et les craintes à l'égard du phénomène non seulement par les acteurs institutionnels et non institutionnels, impliqués dans la gestion des flux, mais aussi par l'opinion publique.Sur la base de ces prémisses, l'objectif de ce travail est de dresser un profil de la communauté marocaine dans le contexte migratoire italien, pour une meilleure compréhension de la réalité. Un autre objectif visé est la mise en évidence, à travers une connaissance du passé, du présent et des évolutions possibles, des liens de cette grande communauté avec à la fois l'Italie et le pays d'origine, car persuadées que les relations et les échanges, bien soutenues, peuvent apporter une contribution significative au développement humain, économique et culturel aussi bien des deux pays que des deux rives de la Méditerranée.

In this paper we analyze the notion of a finite mean from an axiomatic point of view. We discuss several axiomatic alternatives, with the aim of establishing a universal definition reconciling all of them and exploring theoretical links to some branches of Mathematics as well as to multidisciplinary applications.

Different results have been reported concerning the postoperative outcomes of different surgical approaches for hemorrhoids.We aimed to determine the postoperative outcome following 3 main surgical techniques.A prospective, randomized trial was designed with 180 patients in 3 arms of 60 patients each.The study was conducted from April 1999 to January 2007 at the University Hospital "Campus Bio-Medico di Roma."All of the patients who were referred for hemorrhoid surgery were enrolled according to inclusion and exclusion criteria (ISRCTN12040297).Treatments according to the open, semiclosed, and stapled techniques were compared.Sample size was calculated to determine a difference in terms of the intensity of postoperative pain at the first week and the days required for return to work activity.After 1 week, patients who underwent semiclosed hemorrhoidectomy reported significantly less pain (p0.01) and a significant decrease in analgesic intake from the third postoperative day (p0.01) than after the other 2 techniques. The patients resumed work ≈11 days after semiclosed and stapled techniques (11.8 and 11.6 days), which was earlier compared with 21.3 days in the open group (p0.05).The high number of patients excluded might be considered a limitation of the study, but our selection criteria including patients living within 50 km of the hospital allowed for a low rate (9.4%) of patients lost to follow-up.This study found an earlier resumption of work and less pain in patients who underwent the stapled and semiclosed procedures rather than open, which was associated with more complications, particularly because of a higher rate of stenosis (see Video, Supplemental Digital Content 1, http://links.lww.com/DCR/A196).

Modern organizations execute processes to deliver product and services, whose enactment needs to adhere to laws, regulations and standards. Conformance checking is the problem of pinpointing where deviations are observed in the process event data. Literature proposes solutions for the conformance-checking problem that, in fact, are implementations of planning algorithms built ad-hoc for the specific problem. Unfortunately, in the era of big data, these ad-hoc implementations do not scale sufficiently compared with robust, well-established planning systems. Furthermore, their ad-hoc nature does not allow for seamlessly plugging in new outperforming planning algorithms or heuristics, causing a massive amount of work to be necessary to incorporate and evaluate alternatives. This paper summarizes our last results on how instances of the conformance checking problem can be represented as classical planning problems in PDDL for which planners can find a correct solution in a finite amount of time. If conformance checking problems are converted into planning problems, one can seamlessly update to the recent versions of the best performing automated planners, with evident advantages in term of versatility and customization. Experiments with three different planners highlight this versatility. Furthermore, by employing several processes and event logs of different sizes, we show how our planning-based approach outperforms existing approaches of several order of magnitude and, in certain cases, carries out the task while existing approaches run out of memory.

Il capito analizza la nascita e lo svilippo della sociologia della medicina e della salute nel contesto milanese dagli anni Settanta agli anni Novanta del secolo scorso.