Motor neuron diseases are a group of rare neurodegenerative disorders that selectively affect motor neurons, the cells which control voluntary muscles of the body. According to ICD-11, the following disorders are counted among motor neuron diseases: amyotrophic lateral sclerosis, progressive bulbar palsy, pseudobulbar palsy, progressive muscular atrophy, primary lateral sclerosis, monomelic amyotrophy, as well as some rarer variants resembling ALS. Motor neuron diseases affect both adults. While each motor neuron disease affects patients differently, they all cause movement-related symptoms muscle weakness. Most of these diseases seem to occur randomly without known causes. Studies into these inherited forms have led to discoveries of various genes that are thought be important in understanding how the disease occurs. Symptoms of motor neuron diseases can be first seen at birth or can come on later in life. Most of these diseases worsen over time. There are no approved treatments for the majority of motor neuron disorders, care is symptomatic.
Signs and symptoms depend on the specific disease, but motor neuron diseases manifest as a group of movement-related symptoms. They come on and worsen over the course of more than three months. Various patterns of muscle weakness are seen, muscle cramps and spasms may occur. One can have difficulty breathing with climbing stairs, difficulty breathing when lying down, or respiratory failure if breathing muscles become involved. Bulbar symptoms, including difficulty speaking, difficulty swallowing, excessive saliva production, can occur. Sensation, or the ability to feel, is not affected. Emotional disturbance and cognitive and behavioral changes are seen. There can be upper motor neuron findings, or both. Motor neuron diseases are seen both in adults; those that affect children tend to be inherited or familial, their symptoms are either present at birth or appear before learning to walk. Those that affect adults tend to appear after age 40; the clinical course depends on the specific disease, but most progress or worsen over the course of months.
Some are fatal. Various patterns of muscle weakness occur in different motor neuron diseases. Weakness can be symmetric or asymmetric, it can occur in body parts that are distal, proximal, or both... According to Statland et al. There are three main weakness patterns that are seen in motor neuron diseases, which are: Asymmetric distal weakness without sensory loss Symmetric weakness without sensory loss Symmetric focal midline proximal weakness Motor neuron diseases are on a spectrum in terms of upper and lower motor neuron involvement; some have upper motor neuron findings, while others have a mix of both. Lower motor neuron findings include muscle atrophy and fasciculations, upper motor neuron findings include hyperreflexia, muscle spasm, abnormal reflexes. Pure upper motor neuron diseases, or those with just UMN findings, include PLS. Pure lower motor neuron diseases, or those with just LMN findings, include PMA. Motor neuron diseases with both UMN and LMN findings include both familial and sporadic ALS.
Most cases are sporadic and their causes are not known. It is thought that environmental, viral, or genetic factors may be involved. TARDBP referred to as TDP-43, is a critical component of the non-homologous end joining enzymatic pathway that repairs DNA double-strand breaks in pluripotent stem cell-derived motor neurons. TDP-43 is recruited to double-strand breaks where it acts as a scaffold for the recruitment of the XRCC4-DNA ligase protein complex that acts to repair double-strand breaks. About 95% of ALS patients have abnormalities in the nucleus-cytoplasmic localization in spinal motor neurons of TDP43. In TDP-43 depleted human neural stem cell-derived motor neurons, as well as in sporadic ALS patients’ spinal cord specimens there is significant double-strand break accumulation and reduced levels of NHEJ. In adults, men are more affected than women. Differential diagnosis can be challenging due to the number of overlapping symptoms, shared between several motor neuron diseases; the diagnosis is based on clinical findings, family history of MND, a variation of tests, many of which are used to rule out disease mimics, which can manifest with identical symptoms.
Please refer to individual articles for the diagnostic methods used in each individual motor neuron disease. Motor neuron disease describes a collection of clinical disorders, characterized by progressive muscle weakness and the degeneration of the motor neuron on electrophysiological testing; as discussed above, the term "motor neuron disease" has varying meanings in different countries. The literature inconsistently classifies which degenerative motor neuron disorders can be included under the umbrella term "motor neuron disease"; the four main types of MND are marked in the table below. All types of MND can be differentiated by two defining characteristics: Is the disease sporadic or inherited? Is there involvement of th
A bamboo floor is a type of flooring manufactured from the bamboo plant. The majority of today's bamboo flooring products originate in other portions of Asia. Moso bamboo is the species most used for flooring. Bamboo has been used as an alternative for flooring because of its physical similarities to true hardwoods. Bamboo floor manufacturers and sellers promote its strength, its eco-friendliness and its natural resistance to insects and moisture; the hardness of traditional bamboo flooring ranges from 1180 to around 1380, while newer manufacturing techniques including strand woven bamboo flooring range from 3000 to over 5000 using the Janka hardness test. Other flooring materials have comparable Janka ratings, with a higher number indicating a harder material: red oak. Different forms of bamboo flooring exist; each varies in its manufacturing process and differs based on economic viability and local preferences. The most common form in southeast Asia, uses thin bamboo stems that are cut as flat as possible.
They are cut to similar lengths and can be stained, varnished, or used as is. They are nailed down to wooden beams or bigger pieces of bamboo stems; this form results in more space between each bamboo stem. This technique is used on stilted houses, resulting in better air circulation during the warmer summer months; the manufactured bamboo flooring found in North American markets is highly processed. A Bamboo flooring is made by slicing mature bamboo poles or culms into strips; these culms are crosscut to length and sliced into strips depending on the width desired. The outer skin and nodes are removed. To remove starch and sugars the strips of bamboo are boiled in a solution of boric acid or lime; the bamboo is dried and planed. Natural bamboo color is similar to beech wood. If a darker color similar to oak is desired, the bamboo goes through a carbonizing process of steaming under controlled pressure and heat; the carbonizing process can reduce the floor's final hardness compared to non-carbonized bamboo, rendering it softer than some pines and softer than more common red oak.
Most bamboo flooring uses a urea-formaldehyde adhesive in the lamination process. Though the use of UF resins, which emit volatile organic compounds, is harmful to indoor air quality, bamboo flooring uses a small amount compared with other materials, such as particleboards. Bamboo flooring products that avoid formaldehyde use are available, including some listed in the GreenSpec Directory; the panels are heat pressed to cure the adhesive. The cured boards are planed and milled. An ultraviolet curing lacquer is applied to the boards. Manufactured bamboo floors are made available in planks with either vertical- or horizontal-grain orientation. In vertical bamboo floors, the component pieces are stood vertically on their narrowest edge and press laminated side to side; the effect is a lined uniform look to the surface of the finished floor plank. In horizontal bamboo floors, the slats are arranged in a horizontal direction, on their widest edge, joined side by side with adjacent pieces using a high-pressure laminate system.
The characteristic nodes of the bamboo are visible on the finished horizontal surface. Locking bamboo flooring is the easiest to install. Individual flooring planks have interlocking joints that click into place. By combining plank alignment and color a lot of different styles can be produced. Strand woven bamboo flooring can be refinished with a clear-coat quite however applying a stain on-site can be challenging. Bamboo is an eco-friendly renewable source of material; as a grass, bamboo grows much faster than wood. Moso Bamboo is the primary species used for the manufacturing of plywood. Moso bamboo can grow up to 119 cm in 24 m high in 40 to 50 days, it takes about 3–5 years for bamboo to reach full maturity. Traditional hard woods can take 20–120 years to mature. Bamboo can be harvested without the need to replant because the root system is left intact when it is harvested; the rhizome root structure has the ability to hold the soil in place preventing erosion. Rhizome root structures are horizontal stems that grow below the surface and help a plant reproduce vegetatively.
Plants with rhizomes will spread laterally. The Lacey Act strengthened the accountability in the sourcing of timber products; however enforcement is still in question. Bamboo reaches maturity in five years, the optimal age to harvest. In a sustainably harvested forest only 20% of the forest is harvested annually allowing for 100% harvest in a five-year period. In its natural environment it will need no irrigation, no pesticides, no fertilizer. Bamboo has few pests. Bamboo certified to the standards of the Forest Stewardship Council meets criteria for environmental sustainability and social responsibility, several flooring products are available with this option. Bamboo can sequester up to 70% more carbon per year than a hardwood forest. All these factors keep the carbon footprint low; the United States Green Building Council's LEED program allows points for the use of bamboo floors by virtue of it being a renewable resource. Since the majority of bamboo timber comes from China, it has to be shipped to the international destinations by boat and by truck to vendors.
Gury Ivanovich Marchuk was a prominent Soviet and Russian scientist in the fields of computational mathematics, physics of atmosphere. Academician. Among his notable prizes are the USSR State Prize, Demidov Prize, Lomonosov Gold Medal. Marchuk was born in Russia. A member of the Communist Party of the Soviet Union since 1947, Academician Marchuk was elected to the Central Committee of the Party as a candidate member in 1976 and as a full member in 1981, he was elected as deputy of the Supreme Soviet of the Union of Soviet Socialist Republics in 1979. He was appointed to succeed Vladimir Kirillin as chairman of the State Committee for Science and Technology in 1980. Marchuk was a proponent of the Integrated Long-Term Programme of Cooperation in Science & Technology, established in 1987 as a scientific cooperative venture between India and the Soviet Union; the programme allowed the scientists of the countries to collaboratively undertake research in areas as diverse as healthcare and lasers. Marchuk co-chaired the programme's Joint Council with Prof. C.
N. R. Rao for 25 years and was made an honorary member of India's National Academy of Sciences. In 2002, the Government of India conferred the Padma Bhushan on him. Hero of Socialist Labour Honorary Citizen of Obninsk Four Orders of Lenin Keldysh Gold Medal — for his work "The development and creation of new methods of mathematical modeling" Karpinski International Prize Chebyshev Gold Medal — for outstanding performance in mathematics Lomonosov Gold Medal - for his outstanding contribution to the creation of new models and methods for solving problems in the physics of nuclear reactors, the physics of the atmosphere and ocean, immunology Cavalier silver sign "Property of Siberia" Lenin Prize in Science Friedman Prize USSR State Prize State Prize of the Russian Federation Demidov Prize Honorary Doctorates of the University of Toulouse, Charles University, Dresden University of Technology, Technical University of Budapest Foreign Member of the Bulgarian Academy of Sciences, German Academy of Sciences at Berlin, Czechoslovak Academy of Sciences, Polish Academy of Sciences Order of Merit for the Fatherland, 2nd and 4th classes Jubilee Medal "300 Years of the Russian Navy" Medal "For the Victory over Germany in the Great Patriotic War 1941–1945" Medal "For Valiant Labour in the Great Patriotic War 1941-1945" Commander of the Legion of Honour Order of Georgi Dimitrov Padma Bhushan Gury Marchuk — scientific works on the website Math-Net.
Ru Scientific biography
Duhri is a village in the municipality of Kiseljak and Herzegovina. Duhri, as well as the nearby village of Han Ploča, were on the eastern front of the Croatian Republic of Herzeg-Bosnia against the Republic of Bosnia and Herzegovina as well as the Serbian Republic during the Bosnian War; the strategic importance of the area lengthened communication lines of the Army of the Republic of Bosnia and Herzegovina. As a precautionary measure against the ARBiH offensive against Croatian-held territories, the Croatian Defence Council, the armed forces of Herzeg-Bosnia, disarmed the Muslims in the area in August, 1992. On the orders of Colonel Blaskic, their weapons were returned so that they could defend themselves from the Serbs. In April 22-23, the Muslims in Duhri once again surrendered their weapons to the HVO while those in Han Ploča refused to do so
The TAI T625 is a twin-engined light transport/utility helicopter developed by Turkish Aerospace Industries. Turkey's Undersecretariat for Defence Industries plans to offer the new platform to Turkish Armed Forces and cooperating nations. Turkish Aerospace Industries launched the preliminary design studies in 2010; the project commenced in 2013 when Undersecretariat for Defence Industries signed a contract with the Turkish Aerospace Industries to develop a 6-ton class multi-role helicopter for land operations. Alp Aviation is responsible for production and assembly of landing gear and dynamic components, while Spanish CESA was selected to supply hydraulic systems; the T625 is in the interim powered by two LHTEC CTS800 engines. The LHTEC CTS800 was chosen due to commonality with the TAI/AgustaWestland T129. TUSAS Engine Industries, a Turkish Aero-engine design and production company has begun developing a next generation indigenous powerplant for the T625 named the TS1400; the T625 features a four-axis dual redundant automatic flight control system, along with an ASELSAN glass cockpit with two wide touchscreen Integrated Mission Displays and two touchscreen data entry Touch Command Control Units.
It is designed for IFR and VFR single pilot operations, night operations and flight in known icing conditions. The Turkish Presidency of Defense Industries has confirmed that it has obtained a multitude of patents for various sub-systems used in the T625. On 6 September 2018, registered TC-HLP, first flew at Ankara. Data from General characteristics Crew: 2 Capacity: 12 passengers Length: 15.87 m Max takeoff weight: 6,050 kg Powerplant: 2 × LHTEC CTS800-4A turboshaft engine, 1,024 kW each Main rotor diameter: 13.2 m Performance Maximum speed: 306 km/h Cruise speed: 278 km/h Range: 740 km + Endurance: 3 hours 48 minutes Service ceiling: 6,096 m Aircraft of comparable role and era Sikorsky S-76 AgustaWestland AW139 Airbus Helicopters H155 Airbus Helicopters H160 Kamov Ka-60 Harbin Z-9 Related lists List of helicopters List of utility helicopters TAI Reveals the New T625 Mutli-Role Helicopter
The Udukku known as Udukkai, is an Indian traditional percussion instrument of Tamil origin, once popular in Tamil Nadu Kerala, north and east parts of Sri Lanka, similar to Damaru and Edakka, larger than the former but smaller than the latter. Udukku is a smaller version of Edakka, shaped like an hour glass; the instrument is about 8 to 10 inches long with a girth of 6 to 8 inches on both ends and tapering towards the centre. The body of the instrument is traditionally made out of kiln fired clay but variants are made of wood, preferably from a single hollow block of Jackfruit wood. Brass bodies are used in some parts; the ends of the instrument is covered across the mouth with cured and dried animal hide, goat skin is the preferred leather. Hoops are placed on the edge of the instrument body and the skin is tightened using strings woven from end to end. Udukku is played on one side and the non playing side is provided with one or two metallic wire snares, enabling the player to generate more resonance.
A strap made of cloth is fixed to the middle of the instrument for clasping it. Coloured balls made of cotton strings are placed hanging from the instrument as an embellishment. Udukku is played as the percussion accompaniment in temple rituals or in folk culture; the Ayyappan Pattu performed at Sabarimala temple in Kerala to propitiate Lord Ayyappa, uses Udukku as the percussion accompaniment and is called Udukku Pattu. Udukku Kotti Pattu, is a traditional art form of Kerala where the vocal rendition of the song is accompanied by the Udukku rhythms, it has been reported that Udukku was popular in Jafna, Sri Lanka during the 9th century AD. Udukku is played holding it horizontally, only on one side the right side, while the left hand is used to clasp the instrument with the cloth strap; the fingers index and ring fingers, along with the inner palm are used for playing and the cloth strap is squeezed or released to adjust the tightness of the leather to attain pitch modulation. Fingers of the left hand are used to adjust the tightness by pulling the strings directly.
Kaliyamoorthy Poosari Karivelil Radhakrishnan Damaru Edakka "'Udukku' - Played by Mr. M. Thanigasalam, Point-Pedro, Sri Lanka". YouTube. 26 February 2012. Retrieved 18 November 2014. "Udukku performance". Video. YouTube. 10 September 2011. Retrieved 18 November 2014. "Folk song of Kerala - Udukku Kotti Pattu". Video. You Repeat. Retrieved 18 November 2014