Lugarics disease

The motor neuron disease (MND) is a collective term used for a range of Motor Neuron Disease: Amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), Progressive bulbar palsy (PBP) and progressive lateral sclerosis (PLS). MND is the term used internationally whilst ALS is often in the United States (where it) is also known as Lou Gehrig's disease, the famous patient to all forms of MND. It was first described by Jean-Martin Charcot, French neurologist, in 1869 and in France, because the disease is known as Maladie de Charcot (Charcot 's disease). Signs and symptoms of onset of symptoms is usually between 40-70 years (mean 55). Disorder is characterized by progressive loss of voluntary muscle contraction due to the destruction of nerve cells in the brain and spinal cord that are responsible for the promotion of voluntary muscles. During the first symptoms of Lugarics disease are subtle, the disease causes progressive physical disabilities. Mental function and physical sensation is spared, although many patients show symptoms of depression, as they deteriorate. Diagnosis of the Lugarics disease - The diagnosis is both clinical and is based also on grounds, and electromyography (EMG) analysis to show the spread mandatory loss of nerve stimulation of the muscles in the limbs, face and abdomen.Clinically, it can find signs of motor neuron damage (eg, spasticity, lively reflexes and Babinski sign), while the lower motor neurons show weakness and wasting. Bulbar muscle weakness can also be seen (difficulty breathing or swallowing, coughing and speaking). Neuroimaging tests are usually performed to rule out other causes, such as a mass in the upper part of the spinal cord. Causes of Lugarics disease - Most cases of MND are "sporadic ", although it indicated that the occurrence is triggered by viral infections. At least 4 genes are known to be associated with ALS in combination: SOD1, ALS2, NEFH and VAPB. Only ~ 10% of the cases were identified genetic - inherited causes for the remaining 90% are not known. SOD1 gene codes for an enzyme superoxide dismutase, reduced oxidative stress in nerve cells. Thus far, were found more than 100 different mutations in the SOD1 gene, which is any form of ALS.
Similar findings have led researchers assume that nerve cell death caused by an excess of free radicals in the cell. This hypothesis has developed one of the many others to describe the etiology of ALS / MND and is waiting to be reliably determined. Skeletal muscle stimulated groups of neurons (lower motor neurons) are projected onto the front of the spinal cord to the muscle cells are, and these nerve cells) are a group of neurons (upper motor neurons that project from an area called the motor area in the frontal lobe is stimulated. This projection is as corticospinal tract. Shrink in MNS, nerve cells and pathways and to die. On macroscopic pathology of Lugarics disease, the degeneration of the spinal anterior horn and atrophy of the anterior roots (shrunken appearance. Similarly, degeneration of motor neurons seen in the brainstem. Loss of neurons and astrogliosis is developing well. Degeneration of corticospinal paths as well as in the motor neurons of the cerebral cortex Strip, is known. It is a role in excitotoxicity and oxidative stress, probably secondary mitochondrial dysfunction. In animal models, has, death by apoptosis have been identified (tranlocation of Bax to mitochondria, cytochrome c and the relocation) in the cytoplasm. MND has three major subgroups called primary lateral sclerosis (PLS, only the upper motor neurons are affected), progressive muscular atrophy (PMA, only the lower motor neurons are affected) and ALS (both are affected). PCS and PMA each represent about 5% of cases, with most diagnoses ALS. The incidence of MND is approximately 1-5 of 100,000 people, and men are 20% higher than the number of cases in women. Approximately 5,600 cases in the U.S. are diagnosed each year (Lugarics disease). The disease is usually a grave prognosis and 50% of patients die within the first 3 years, 20% of the population live survive 5 or more years ago, only 10% for 10 or more years. There are documented cases of people who live over 35 years after diagnosis, Stephen Hawking lives more than 42 years since his (1963) Lugarics disease diagnosis. MND rarer subgroups have a better prognosis than ALS, PMA people are more likely to "long survivors ', ie> 5 years, while people with PLS actually have a normal life. ALS and PMA, mortality is usually caused by the weakness of the respiratory complications of prolonged paralysis. Treatment of Lugarics Disease is essentially supportive and symptomatic. Many patients may require modifications to their environment as they progressively become more physically impaired. Riluzole, an inhibitor of glutamate, can the progression of ALS in selected patients. In the meantime, using some experimental drugs in the setup process, of which) many disturbances in the oxidative stress in cells (presumed mechanism, but only limited success. Some current promising research to cure is through gene therapy and the use of stem cells, ethical and legal problems, harvesting stem cells is slowing progress, focusing especially in the United States. Etymology Amyotrophic comes from the Greek language. -Means "no ", myo refers to "muscles and trophic " food "means, amyotrophic therefore means " no muscle nourishment, "which describes the typical sufferer atrophication 's not used muscle tissue. side are the areas in a person 's spinal cord, where are the nerve cells that are affected by Lugarics Disease. As this area degenerates, leading to scarring or hardening "sclerosis " in this region. History and prominent of Lugarics disease patients, Lou Gehrig brought national and international attention for the disease in 1939, when he abruptly from baseball after he is diagnosed with ALS / MND. Former guitar virtuoso Jason Becker, a theoretical physicist Stephen Hawking and former soccer player Jimmy Johnstone Celtic also suffer from the disease. Founder and nursing Leonard Cheshire VC, Rangers footballer Sam English, Hall of Fame pitcher Jim "Catfish " Hunter, a blues singer and guitarist Leadbelly, jazz giant Charles Mingus, Hollywood actor David Niven, the legendary Leeds United manager Don Revie, American soap opera veteran Michael Zaslow and Chinese Chairman Mao Zedong died of the Lugarics disease.