Know More About Leigh’s disease
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by: Saiera
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Leigh’s disease which is alternatively called Leigh’s syndrome denotes an uncommon inherited neurometabolic disorder that wrecks havoc on the central nervous system and adversely affects its metabolism. It most commonly affects the children aged between 3 months to 2 years. However, in some unusual cases, the teenagers and adults can also fall prey to this disease. Another variant of the ailment is named X-linked Leigh’s disease which is caused by the mutation of a gene located in the X chromosome of the affected person. The causes of Leigh’s disease The Mitochondria are tiny structures in animal cells that act as the power station of the cells. In other words, they supply the cells with the energy to function. In human body, the mitochondria’s basic function is to transfer the energy retrieved from the fatty acids and glucose to adenosine triphosphate or ATP. The energy stored in the ATP is subsequently implemented to execute the metabolic activities of the other cells. The Mitochondria are unique since they possess their own variant of DNA which is called mtDNA or mitochondrial DNA in physiological terms. When irregular mutations take place in the mtDNA, the mitochondria fail to work as it should. This makes a person vulnerable to a range of ailments which include Leigh’s disease. When the vital cells in a person’s brain stem get mutated mtDNA it leads to the formation of malfunctioning mitochondria and he contracts Leigh’s disease. If this happens, there is a constant deficiency of energy in the cells of his body which ultimately results in quick deterioration of the CNS. The motor skills of the person also degrade with progression of the disease. The boys can get the gene from their mother while the daughters may act as the careers of the gene. If both of the parents act as the career of the gene, there is 25% possibility that every child will get affected. The symptoms of Leigh’s disease The symptoms of Leigh’s disease start appearing in the childhood. The initial symptoms observed in the children are poor sucking ability and failure of acquired motor movement and skills. The disease is characterized by the gradual deterioration of a victim’s capability to regulate his movement. As the disease advances, more symptoms start appearing like fatigue and deficiency of muscle tone. Some patients face the build up of lactic acid in their brain. Subsequent problems are kidney failures and heart problems. The children tend to become more irritable with time. They also suffer from vomiting, seizures and continual crying incidents. The victims of this disease usually do not live for long after contracting the menace. Very few children afflicted with the disease can survive till their adolescence. If the disease attacks someone in his or her early adulthood then its progression is slow compared to other patients. The diagnosis of Leigh’s disease The diagnosis of this rare disease basically depends on factors like the irregularities in biochemistry, clinical characteristics etc. When the brain scan reports show symmetrical lesions, the doctors suspect its occurrence. The genetic tests can confirm the presence of the mutated gene in a person’s body. Another possible choice is prenatal screening. The treatment of Leigh’s disease This is indeed a very unusual disorder that impacts a minority of the population. Unfortunately, no specific cure is available for Leigh’s disease. The available treatments are not extensive in nature and they have been seen to be partially effective. The doctors try to make alterations to the diet of the affected person to cope with the malady. The inclusion of Vitamin B1 or thiamine is required in the diet of a patient suffering from this disease. The diet should ideally include foods that are low in carbohydrate and have high contents of fat. However, dietary changes in most instances succeed in prolonging the life span of the child by a few years but can not prevent the death. Other treatments include occupational, physical and speech therapy. For people who have developed serious movement problems or epilepsy, medication becomes imperative. |
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