What is Leigh's disease?
Leigh disease, commonly referred to as Leigh's disease, is a rare neurometabolic disorder, a disorder that prevents cells in the central nervous system in energy production. Cells are unable to produce energy for growth and maturation because they are unable to convert nutrients from the bloodstream into an energy form that the cell can use as fuel. Because cells are unable to produce energy for growth and maintaining themselves, energy exhaustion accumulates and the cells begin to deteriorate and die. As Leigh disease affects mainly at the time of maximum growth - from birth to early childhood - and because it affects the brain in a major period of development, the disease is always fatal.
Leigh's disease was discovered in the early 1950s Dr. Denis Leigh and at that time were called "deadly" diseases because of his prevalence of death among very young people. It is characterized by rapid body failure, along with seizures and loss of coordination. The disease may also occur during adultsEntry or first adults, but when they do so, then it is called subacute necrotizing encephalomyelopathy (dream). The tendency towards cellular dysfunction believes that many medical specialists are inherited on the mother side and found that it is associated with cellular mutation, a cell genetic material that is transmitted to newly formed cells.
In neurology, the area of medicine, which studies the brain and the nervous system, Leigh disease is classified as one of the most serious forms of known mitochondrial diseases caused by defective cellular mitochondria - part of the cell that produces energy. There are other less deadly forms depending on how cell energy is affected. There may be smaller groups of cells with unusual genetic material and creating milder forms of mitochondrial disease because higher number of normal energy cells can alleviate weightsthe disease of the disease. Both nerve and muscle cells are subject to mitochondrial disease, because of their high energy requirements and the condition caused by deteriorating muscle fiber cells is called mitochondrial myopathy.
Leigh disease is actually a syndrome in that it affects muscles and brain and is also called mitochondrial encephalomyopathy. There is no known disease for the disease and treatment depends on its severity. Food supplements have been tested in an effort to help replace the substances needed by the body that are unable to be made by affected cells. Combined forms of natural enzymes and amino acids have been used are creatin, L-carnitine and CoQ10 supplements-and although they have been shown to improve the medical improvement of mitochondrial disease, it is generally considered beneficial.