"Arne Naess between Reason and Emotion." (This paper was the basis for lectures held at the Universities of Prague, Vienna and Belgrade, May 2003)

I try to convince the reader that we all too often consider our decisions more or less unreasonable – and due to emotions overpowering reason. The dualism: reason/emotion may be dangerously misleading. Psychoanalysis may be said to have been the first systematic effort to help us find the real reasons for our important decisions and views. Personal maturity involves both strength of emotions and clearness of thinking

Subsidiarity and Proportionality in the Single Market: An EU fit for inclusive growth

This report offers a fresh perspective on the principles of subsidiarity and proportionality in the European Union based on a thorough-going economic analysis. Specifically, the report uses the EU Single Market as a case to discuss shortcomings and potential improvements in five key policy areas. It reviews how the principles of subsid- iarity and proportionality can help boost growth in the EU at the aggregate country level – while at the same time allowing EU regions to benefit from growth. The report focuses on the regional level as economic growth has been uneven across the EU’s regions over the last decade and, consequently, growing disparities between re- gions have emerged. This alone merits a review on how we can reconcile the twin objectives in the future

Simulated forecasts for primordial B-mode searches in ground-based experiments

Detecting the imprint of inflationary gravitational waves on the $B$-mode polarization of the Cosmic Microwave Background (CMB) is one of the main science cases for current and next-generation CMB experiments. In this work we explore some of the challenges that ground-based facilities will have to face in order to carry out this measurement in the presence of Galactic foregrounds and correlated atmospheric noise. We present forecasts for Stage-3 (S3) and planned Stage-4 (S4) experiments based on the analysis of simulated sky maps using a map-based Bayesian foreground cleaning method. Our results thus consistently propagate the uncertainties on foreground parameters such as spatially-varying spectral indices, as well as the bias on the measured tensor-to-scalar ratio $r$ caused by an incorrect modelling of the foregrounds. We find that S3 and S4-like experiments should be able to put constraints on $r$ of the order $\sigma(r)=(0.5-1.0)\times10^{-2}$ and $\sigma(r)=(0.5-1.0)\times10^{-3}$ respectively, assuming instrumental systematic effects are under control. We further study deviations from the fiducial foreground model, finding that, while the effects of a second polarized dust component would be minimal on both S3 and S4, a 2\% polarized anomalous dust emission (AME) component would be clearly detectable by Stage-4 experiments.Comment: 19 pages, 11 figure

Mitochondrial disease in children : from clinical presentation to genetic background

Mitochondrial disorders are amongst the most common groups of inborn errors of metabolism. They are caused by deficiencies in the final pathway of the cellular energy production, the mitochondrial respiratory chain. The disorders are clinically and genetically heterogeneous and the aetiology can be found in the mitochondrial, or in the nuclear genome. This thesis describes children with mitochondrial disorders, with focus on clinical symptoms, disease courses, biochemical abnormalities and genetic causes of disease. The research aimed to increase the understanding of the clinical phenotypes and pathophysiological mechanisms. We also aimed to identify novel disease-causing variants in mitochondrial (mtDNA), as well as nuclear, DNA in order to generate better tools for genetic counselling. In a study of patients with deficiencies of complex I of the mitochondrial respiratory chain, we observed a variety of clinical presentations. Early-onset of disease and muscle weakness were features in common. Developmental retardation and failure to thrive were seen in a majority of the patients. Causative variants in mtDNA were identified in six of the 11 patients. Leigh syndrome (LS) is a severe, neurodegenerative disease of early childhood. The genetic aetiology is heterogeneous. In a study of 25 children with LS, we observed early onset of disease, in 80% before six months of age. A subset of patients had a rapidly progressive disease and early death, 60% survived beyond the age of five years. Eight of the patients had a disease causing variant in mtDNA. The age ofonset, clinical symptoms or prognosis did not differ significantly between patients with mitochondrial and nuclear mutations in this cohort. A defect in the POLG gene was detected in a patient with Alpers syndrome. He had a heterozygous variant on one allele, the other allele being entirely deleted. The patient had rapid disease progression and died in a valproate induced liver failure. Massively parallel sequencing of the entire human genome and its implementation in clinical use is a diagnostic leap in the field of mitochondrial disorders. In a cohort of patients with combined deficiencies of the mitochondrial respiratory chain, 31 patients were subjected to whole genome/exome sequencing. A genetic diagnosis was established in 16 of these (52%), so far. Two novel gene defects were identified; SLC25A26 and COQ7. The latter gene encodes an enzyme of the Coenzyme Q (CoQ) biosynthesis. These disorders are responsive to CoQ10 treatment. We demonstrated a new mechanism of treatment using 2,4- dihydroxybenzoic acid in order to bypass the deficient step. In conclusion, paediatric mitochondrial disorders are severe, progressive and usually multisystemic. The most common symptoms are often non-specific and the diagnostic procedure is a challenge. The genetic aetiology is heterogeneous, a substantial proportion of the causative variants are found in mtDNA. The phenotype-genotype correlation is poor, making whole genome sequencing an excellent diagnostic tool

Urban Structures and Travel Behaviour

In line with theoretical considerations, a number of empirical studies reviewed in this paper show that urban structural variables influence the inhabitantsÌ amount of transport and their choice of means of conveyance. This appears to be true across city sizes. The location of the residence relative to the city centre is the urban structural characteristic which, according to our analyses, exerts the strongest influence on travelling distances, modal split between car and non-motorized transport, and energy use for transport. The distance from the residence to the downtown area is a key factor influencing the accessibility to a number of facility types. Population densities at a local scale as well as for the city as a whole are also important to the amount of travel and the use of cars. In addition, a high road capacity contributes to the increase of the proportion of commuters travelling by car in the peak period

A numerical study of the existence and stability of some chaotic attractors by path integration

The response of a harmonically excited Duffing oscillator with chaotic response is studied by replacing the excitation by harmonic excitation plus added noise, a harmonic motion with phase perturbations, and a narrow-band filtered noise. The mean frequency and excitation energy for all the models are the same, assuming that these are basic parameters for the response of the oscillator. The resulting probability densities in the state space show that the chaotic attractor is very stable for the different kinds of perturbations. Also, a new conditional path integration method is described, which is shown to be robust and accurate while the CPU time is kept at a minimu