Tremor in motor neuron disease may be central rather than peripheral in origin

BACKGROUND AND PURPOSE: Motor neuron disease (MND) refers to a spectrum of degenerative diseases affecting motor neurons. Recent clinical and post-mortem observations have revealed considerable variability in the phenotype. Rhythmic involuntary oscillations of the hands during action, resembling tremor, can occur in MND, but their pathophysiology has not yet been investigated. METHODS: A total of 120 consecutive patients with MND were screened for tremor. Twelve patients with action tremor and no other movement disorders were found. Ten took part in the study. Tremor was recorded bilaterally using surface electromyography (EMG) and triaxial accelerometer, with and without a variable weight load. Power spectra of rectified EMG and accelerometric signal were calculated. To investigate a possible cerebellar involvement, eyeblink classic conditioning was performed in five patients. RESULTS: Action tremor was present in about 10% of our population. All patients showed distal postural tremor of low amplitude and constant frequency, bilateral with a small degree of asymmetry. Two also showed simple kinetic tremor. A peak at the EMG and accelerometric recordings ranging from 4 to 12 Hz was found in all patients. Loading did not change peak frequency in either the electromyographic or accelerometric power spectra. Compared with healthy volunteers, patients had a smaller number of conditioned responses during eyeblink classic conditioning. CONCLUSIONS: Our data suggest that patients with MND can present with action tremor of a central origin, possibly due to a cerebellar dysfunction. This evidence supports the novel idea of MND as a multisystem neurodegenerative disease and that action tremor can be part of this condition

Versatile Electro-Dynamic Tethers Dynamics Simulator for Debris Mitigation Tools Design

As far as the space debris mitigation is concerned, the electro-dynamic tethers (EDTs) represent a valuable alternative for de-orbiting. The paper presents a high accuracy numerical simulator developed to support the design and verify the effectiveness of such systems: accurate models are exploited for the mechanical, electrodynamical and environmental representation. Results confirmed the known instabilities of EDTs; to cope with them a control strategy is here proposed, traded off among different laws. The selected control relies on varying either the load resistance or the cathodic emitter voltage drop, at the system cathode, being the current profile the controlled variable. The sensitivity analysis, run on several design parameters, is presented and the interdependencies with stability and performance are discussed

Sensitivity of the spherical gravitational wave detector MiniGRAIL operating at 5 K

We present the performances and the strain sensitivity of the first spherical gravitational wave detector equipped with a capacitive transducer and read out by a low noise two-stage SQUID amplifier and operated at a temperature of 5 K. We characterized the detector performance in terms of thermal and electrical noise in the system output sygnal. We measured a peak strain sensitivity of $1.5\cdot 10^{-20} Hz^{-1/2}$ at 2942.9 Hz. A strain sensitivity of better than $5\cdot 10{-20}Hz{-1/2}$ has been obtained over a bandwidth of 30 Hz. We expect an improvement of more than one order of magnitude when the detector will operate at 50 mK. Our results represent the first step towards the development of an ultracryogenic omnidirectional detector sensitive to gravitational radiation in the 3kHz range.Comment: 8 pages, 5 figures, submitted to Physical Review

Quark nuggets search using 2350 Kg gravitational waves aluminum bar detectors

The gravitational wave resonant detectors can be used as detectors of quark nuggets, like nuclearites (nuclear matter with a strange quark). This search has been carried out using data from two 2350 Kg, 2 K cooled, aluminum bar detectors: NAUTILUS, located in Frascati (Italy), and EXPLORER, that was located in CERN Geneva (CH). Both antennas are equipped with cosmic ray shower detectors: signals in the bar due to showers are continuously detected and used to characterize the antenna performances. The bar excitation mechanism is based on the so called thermo-acoustic effect, studied on dedicated experiments that use particle beams. This mechanism predicts that vibrations of bars are induced by the heat deposited in the bar from the particle. The geometrical acceptance of the bar detectors is 19.5 $\rm m^2$ sr, that is smaller than that of other detectors used for similar searches. However, the detection mechanism is completely different and is more straightforward than in other detectors. We will show the results of ten years of data from NAUTILUS (2003-2012) and 7 years from EXPLORER (2003-2009). The experimental limits we obtain are of interest because, for nuclearites of mass less than $10^{-4}$ grams, we find a flux smaller than that one predicted considering nuclearites as dark matter candidates.Comment: presented to the 33rd International Cosmic Ray Conference Rio de Janeiro 201

SSADH variation in primates: Intra- and interspecific data on a gene with a potential role in human cognitive functions

In the present study we focus on the nucleotide and the inferred amino acid variation occurring in humans and other primate species for mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase, a gene recently supposed to contribute to cognitive performance in humans. We determined 2527 bp of coding, intronic, and flanking sequences from chimpanzee, bonobo, gorilla, orangutan, gibbon, and macaque. We also resequenced the entire coding sequence on 39 independent chromosomes from Italian families. Four variable coding sites were genotyped in additional populations from Europe, Africa, and Asia. A test for constancy of the nonsynonymous vs. synonymous rates of nucleotide changes revealed that primates are characterized by largely variable d(N)/d(S) ratios. On a background of strong conservation, probably controlled by selective constraints, the lineage leading to humans showed a ratio increased to 0.42. Human polymorphic levels fall in the range reported for other genes, with a pattern of frequency and haplotype structure strongly suggestive of nonneutrality. The comparison with the primate sequences allowed inferring the ancestral state at all variable positions, suggesting that the c.538(C) allele and the associated functional variant is indeed a derived state that is proceeding to fixation. The unexpected pattern of human polymorphism compared to interspecific findings outlines the possibility of a recent positive selection on some variants relevant to new cognitive capabilities unique to humans

Analysis of 3 years of data from the gravitational wave detectors EXPLORER and NAUTILUS

We performed a search for short gravitational wave bursts using about 3 years of data of the resonant bar detectors Nautilus and Explorer. Two types of analysis were performed: a search for coincidences with a low background of accidentals (0.1 over the entire period), and the calculation of upper limits on the rate of gravitational wave bursts. Here we give a detailed account of the methodology and we report the results: a null search for coincident events and an upper limit that improves over all previous limits from resonant antennas, and is competitive, in the range h_rss ~1E-19, with limits from interferometric detectors. Some new methodological features are introduced that have proven successful in the upper limits evaluation.Comment: 12 pages, 12 figure

Spatial multicriteria analysis for sustainability assessment : a new model for decision making

Policy makers have to consider the sustainability perspective in strategic planning decisions. Identifyand measure the level of sustainability, through its three dimensions, is a priority. Therefore, the aim of this work is to present a new model, called GeoUmbriaSUIT, integrating Multicriteria Analysis and Geographic Information Systems, specifically developed for helping Decision Makers to take policy decisions about sustainability in planning. The model provides outputs which are easy to be understood by not experts; the evaluation path is traceable and transparent, thanks to back analysis. To better explain the potentiality of GeoUmbriaSUIT and its functioning, a case study about Malta is described. Our results showed that in four regions of Malta the best dimension was the environmental one, while only for two regions (Northern Harbour and Southern Harbour) respectively the economic and social dimensions obtained the best scores. The integration of MCDA-GIS resulted to be a useful tool for sustainability assessment.peer-reviewe