Leigh disease is a rare neurometabolic disorder that prevents cells in the central nervous system from producing energy, leading to cell deterioration and death. It is a severe form of mitochondrial disease with no known cure, affecting both muscles and the brain. Treatment depends on severity, and dietary supplements may help.
Leigh disease, commonly called Leigh disease, is a rare neurometabolic disorder, which is a disease that prevents cells in the central nervous system from producing energy. Cells are unable to produce energy to grow and mature because they are unable to convert nutrients from the bloodstream into a form of energy that can be used by the cell for fuel. As cells are unable to produce the energy to grow and maintain themselves, energy depletion builds up and cells begin to deteriorate and die. Because Leigh’s disease strikes primarily at the time of maximum growth—from birth to early childhood—and because it affects the brain at a vital stage in development, the disease is invariably fatal.
Leigh’s disease was discovered in the early 1950’s by Dr. Denis Leigh and was at that time termed a “fatal” disease, due to its prevalence of deaths among the very young. It is characterized by a rapid failure of body development, along with convulsions and loss of coordination. The disease can also occur during adolescence or early adulthood, but when it does occur it is referred to as subacute necrotizing encephalomyelopathy (SNEM). Many medical specialists believe that the tendency towards cellular dysfunction is inherited from the maternal side, and it has been found to be linked to the mutation of cellular DNA, the genetic material of a cell that is passed on to newly created cells.
In neurology, an area of medicine that studies the brain and nervous system, Leigh’s disease is classified as one of the most severe forms of mitochondrial disease known to be caused by defective cell mitochondria, the part of the cell that produces energy. There are other less lethal forms, depending on how the cells’ energy production is affected. There may be smaller clusters of cells with abnormal genetic material, producing milder forms of mitochondrial disease, as higher numbers of normal energy-producing cells can mitigate the severity of the disease. Both nerve and muscle cells are particularly prone to mitochondrial disease, due to their high energy requirements, and the condition caused by the deterioration of muscle fiber cells is known as mitochondrial myopathy.
Leigh’s disease is actually a syndrome, as it affects both muscles and the brain, and is also called mitochondrial encephalomyopathy. There is no known cure for the disease and treatment depends on its severity. Dietary supplements have been tried in an attempt to help replace substances needed by the body that cannot be made by affected cells. Combined forms of natural enzymes and amino acids, such as creatine, L-carnitine and coQ10 supplements, have been used, and while they have not been shown to provide medical improvement in mitochondrial disease, they are generally thought to be helpful.
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