Late-onset spinal motor neuronopathy- a new neuromuscular disease

The aim of this study was to clarify the clinical phenotype of late-onset spinal motor neuronopathy (LOSMoN), an adult-onset autosomal dominant lower motor neuron disorder identified first in two families in Eastern Finland, in order to clarify its genetic background. Motor neuron disorders (MNDs) are characterized by dysfunction and premature death of motor neurons in the brain and spinal cord. MNDs can manifest at any age of the human lifespan, ranging from pre- or neonatal forms such as spinal muscular atrophy type I (SMA I) to those preferentially affecting the older age groups exemplified by sporadic amyotrophic lateral sclerosis (ALS). With a combination of genetic linkage analysis and genome sequencing using DNA from a total of 55 affected members of 17 families and a whole genome scan, we were able to show that LOSMoN is caused by the c.197G>T p.G66V mutation in the gene CHCHD10. This study showed that LOSMoN has very characteristic features that help to differentiate it from other more malignant forms of motor neuron disease, such as ALS, which was erroneously diagnosed in many patients in our cohort. Lack of fibrillations in the first dorsal interosseus muscle on EMG and extensive grouping of non-atrophic type IIA/2A fibers on muscle biopsy were shown to be common findings in LOSMoN, but rare or absent in ALS patients. The results of this study will help clinicians recognize the characteristic phenotype of LOSMoN disease and thus improve their diagnostic accuracy, and will also allow physicians to provide adequate genetic counseling for patients.

On site measurements of DC link electrolytic capacitor bank of frequency converter using constant DC magnetization of the motor

Preventive maintenance of frequency converters has been based on pre-planned replace-ment of wearing or ageing components. Exchange intervals follow component life-time expectations which are based on empirical knowledge or schedules defined by manufac-turer. However, the lifetime of a component can vary significantly, because drives are used in very different operating environments and applications. The main objective of the research was to provide information on methods, i.e. how in-verter's operating condition can be measured reliably under field conditions. At first, the research focused on critical components such as current transducers, IGBTs and DC link capacitor bank, because these aging have already been identified. Of these, the DC link capacitor measurement method was selected for closer examination. With this method, the total capacitance and its total series resistance can be measured. The suitability of the measuring procedure was estimated on the basis of practical measurements. The research was made by using so called triangulation method, including a literature review, simulations and practical measurements. Based on the results, the new measu-rement method seems suitable with some reservations to practical measurements. How-ever, the measuring method should be further developed in order to improve its reliability.

Control of multi-actuator system by an electrically driven hydraulic pump-motor

The awareness and concern of our environment together with legislation have set more and more tightening demands for energy efficiency of non-road mobile machinery (NRMM). Integrated electro-hydraulic energy converter (IEHEC) has been developed in Lappeenranta University of Technology (LUT). The elimination of resistance flow, and the recuperation of energy makes it very efficient alternative. The difficulties of IEHEC machine to step to the market has been the requirement of one IEHEC machine per one actuator. The idea is to switch IEHEC between two actuators of log crane using fast on/off valves. The control system architecture is introduced. The system has been simulated in co-simulation using two different software. The simulated responses of pump-controlled system is compared to the responses of the conventional valve-controlled system.

Motor Types Comparison Determining Efficiency in Pumping Systems

Pumping, fan and compressor systems consume most of the motor electricity power in both the industrial and services sectors. A variable speed drive brings relevant improvements in a fluid system leading to energy saving that further on can be translated into Mtons reduction of CO 2 emissions. Standards and regulations are being adopted for fluid handling systems to limit the less efficiency pumps out of the European market on the coming years and a greater potential in energy savings is dictated by the Energy Efficiency Index (EEI) requirements for the whole pumping system and integrated pumps. Electric motors also have an International Efficiency (IE) classification in order to introduce higher efficiency motors into the market. In this thesis, the applicability of mid-size common electric motor types to industrial pumping system took place comparing the motor efficiency characteristics with each other and by analyzing the effect of motor dimensioning on the pumping system and its impact in the energy consumption.

[Travaux au niveau du 79 boulevard de Magenta, pilonneuse The New Way Motor Co de Lansing, Michigan, U.S.A., entrepreneur M. Bancel, 83 rue Petit] : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 58604

[Travaux au niveau du 79 boulevard de Magenta, pilonneuse The New Way Motor Co de Lansing, Michigan, U.S.A., entrepreneur M. Bancel, 83 rue Petit] : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 58608

Pilonneuse The New Way Motor Co de Lansing, Michigan, U.S.A., entrepreneur M. Bancel, 83 rue Petit [Travaux au niveau du 79 boulevard de Magenta] : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 58609

Behavioural and neural correlates of operant conditioning in Lymnaea stagnalis : role of previous experience during development /

The freshwater mollusc Lymnaea stagnalis was utilized in this study to further the understanding of how network properties change as a result of associative learning, and to determine whether or not this plasticity is dependent on previous experience during development. The respiratory and neural correlates of operant conditioning were first determined in normally reared Lymnaea. The same procedure was then applied to differentially reared Lymnaea, that is, animals that had never experienced aerial respiration during their development. The aim was to determine whether these animals would demonstrate the same responses to the training paradigm. In normally reared animals, a behavioural reduction in aerial respiration was accompanied by numerous changes within the neural network. Specifically, I provide evidence of changes at the level of the respiratory central pattern generator and the motor output. In the differentially reared animals, there was little behavioural data to suggest learning and memory. There were, however, significant differences in the network parameters, similar to those observed in normally reared animals. This demonstrated an effect of operant conditioning on differentially reared animals. In this thesis, I have identified additional correlates of operant conditioning in normally reared animals and provide evidence of associative learning in differentially reared animals. I conclude plasticity is not dependent on previous experience, but is rather ontogenetically programmed within the neural network.

A cognitive schematic paradigm for expository essay writing in secondary schools

The methodology outlined in this study for teaching exposit ory writing to advanced (five year phase) grade eleven students is based on the assumption that writing as a problem solving strategy is a high level cognitive skill . In adhering to this assumption, a cognitively based schematic organizer known as a cross-classification chart was tested for its effectiveness a t the planning stage of the writing process . Results were not significant in any of the three components that were evaluated; however , a post- hoc analysis undertaken because of recorded observed data indicated a significant difference in the mean score on the Organization component for the treatment subgroup using the cross- classification organizer . Furthermore, the treatment group's positive response from the attitude survey towards planning writing is encouraging enough that replication and extension of the application of schema theory to wri ting should be pursued in cross-section and longitud i nal studies.

Organizational change: implications of culture and leadership in the transformation to a total quality management paradigm

The study was undertaken to investigate organizational readiness for change to a total quality management (TQM) paradigm as the corporate-wide strategy within a long-term care facility. The focus of the study was on leadership values and organizational cultural characteristics that could either accelerate or impede the change process at The Public Hospital. structurally, the ~tudy included 'three distinct components. The first component examined the management philosophy outlined by Deming (1986) and his contemporary Juran (1989) in order to determine what leadership values best support the new Total Quality Management paradigm. Secondly, this information was compared to present leadership values at The Public Hospital with the purpose of identifying opportunities for improvement within the organization's current culture as the hospital moves toward the desired TQM culture. The final component, a roadmap, was developed to reflect the most appropriate direction for organizational change at The Public Hospital.