Adrenoleukodystrophy

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2018-05-05

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faculty of Basic Medical Science - Libyan International Medical University

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My report will have to discuss a type of neurological disease adrenoleukodystrophy refers to several different inherited condition that affect the nervous system and adrenal gland.The three major categories of ALD are childhood cerebral ALD ,adrenomyelopathy and Addison's disease.The gene that causes ALD was identified in 1993.According to the oncofertility consortium ALD occurs in about 1 in 20,000 to 50,000 people and mainly affects men.Women with the gene tend to be asymptomatic or mildly symptomatic ,meaning there are no symptoms or very few symptoms.The symptoms,treatmeants,and prognosis of ALD very depending on wich type is present.ALD is not curable,but doctors can sometimes sow its progression

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Adrenoleukodystrophy;is an X-Linked metabolic disorder characterized by the breakdowen or loss of the myelin sheath surrounding nerve cells in the brain and progressive dysfunction of the adrenal gland .Adrenaleukodystrophy is one of a group of genetic disorders called the leukodystrophies that cause damage to the myelin sheath of the nerve fibres in the brain.The myelin sheath is a fatty covering which acts as an electrical insulator(5).Without that sheath the neurons cannot conduct action potentials-in other words they stop telling the muscles of ther elements of the central nervous system what to do.This sequence of events appears to be related to an abnormal accumulation of saturated very long-chain fatty acids(VLCFA)in the serum and tissues of the central nervous system,which sets off an abnormal immune respose that leads to demylination it is unclear exactly how this chain of events works,but scientists do know that it has its roots in genetics

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http://repository.limu.edu.ly/handle/123456789/563

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Attribution 3.0 United States
Except where otherwised noted, this item's license is described as Attribution 3.0 United States