Intrusions in story recall: When over-learned information interferes with episodic memory recall. Evidence from Alzheimer\u2019s disease.

Patients with Alzheimer\u2019s disease (AD) suffer from distortions of memory. Among such distortions, intrusions in memory tests are frequently observed. In this study we describe the performance of a group of mild AD patients and a group of normal controls on the recall of three different types of stories: a previously unknown story, a well-known fairy-tale (Cinderella), and a modified well-known fairy-tale (Little Red Riding Hood is not eaten by the wolf). The aim of our study was to test the hypothesis that in patients who tend to produce intrusions, over-learned information interferes with episodic recall, i.e., the retrieval of specific, unique past episodes. AD patients produced significantly more intrusions in the recall of the modified fairy-tale compared to the recall of the two other stories. Intrusions in the recall of the modified fairy-tale always consisted of elements of the original version of the story. We suggest that in AD patients intrusions may be traced back to the interfer- ence of strongly represented, over-learned information in episodic memory recall

Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients.

Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the FLCN gene coding for folliculin. Its clinical expression includes cutaneous fibrofolliculomas, renal tumors, multiple pulmonary cysts, and recurrent spontaneous pneumothoraces. Data on lung function in BHD are scarce and it is not known whether lung function declines over time. We retrospectively assessed lung function at baseline and during follow-up in 96 patients with BHD. Ninety-five percent of BHD patients had multiple pulmonary cysts on computed tomography and 59% had experienced at least one pneumothorax. Mean values of forced expiratory volume in 1 second (FEV <sub>1</sub> ), forced vital capacity (FVC), FEV <sub>1</sub> /FVC ratio, and total lung capacity were normal at baseline. Mean (standard deviation) residual volume (RV) was moderately increased to 116 (36) %pred at baseline, and RV was elevated > 120%pred in 41% of cases. Mean (standard deviation) carbon monoxide transfer factor (DLco) was moderately decreased to 85 (18) %pred at baseline, and DLco was decreased < 80%pred in 33% of cases. When adjusted for age, gender, smoking and history of pleurodesis, lung function parameters did not significantly decline over a follow-up period of 6 years. Cystic lung disease in BHD does not affect respiratory function at baseline except for slightly increased RV and reduced DLco. No significant deterioration of lung function occurs in BHD over a follow-up period of 6 years

The Effects of Uncertainty of Input Parameters on Nuclear Fuel Cycle Scenario Studies

International audienceNuclear systems, composed of reactors and fuel cycle facilities, are complex and in constant evolution. Fuel cycle scenario studies aim at evaluating the consequences of decisions taken in the near term on the eventual outcomes of a chosen fuel cycle.Scenario studies require a large amount of data and hypotheses, such as the energy demand and its evolution or the performances and characteristics of existing and future technologies. Each hypothesis, represented by a parameter, is a potential source of uncertainty that can impact the scenario outcomes. The Nuclear Energy Agency's Expert Group on Advanced Fuel Cycle Scenarios (NEA/AFCS) recently completed an activity that used sensitivity studies to evaluate the impact of uncertainty of the primary scenario input parameters on scenario results.At the beginning of the study, a base case scenario was specified and evaluated. It considers a PWR UOX reactor fleet using low enriched uranium oxide fuel and its gradual replacement by a sodium fast reactor (SFR) fleet using uranium/plutonium mixed oxide fuel. Each member of the group evaluated this scenario with its own scenario code (COSI, COSAC, FAMILY, SITON, TR_EVOL or VISION). It is noteworthy that the different codes show a good consistency in the results and that the differences have been investigated and documented in order to ensure a strong basis for comparison in the sensitivity study.Then, 17 key input parameters and their range of variation as well as 22 scenario outputs were identified and sensitivity analyses were conducted. The key parameters were chosen amongst the general assumptions on the scenario (energy demand, introduction date of fast reactor), reactors and facilities characteristics (burn-ups, losses at reprocessing) and some minor actinides recycling parameters. As far as possible, each parameter was varied independently from the others. This sensitivity study shows the relative impact of each parameter on each output metric. In order to compare the large amount of results, two methods of summarization were adopted, as described in the paper. These comparisons have shown that some parameters, such as the energetic production or the introduction date of fast reactors, have a strong impact on the results whereas others, like the UOX fabrication time, have almost no impact on the studied scenario