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Leukodystrophy In The Curious Case Of Benjamin Button

845 Words4 Pages

By the looks of their home one would expect that Anthony and Christine Clark were raising two rambunctious toddlers. Toy cars and Monopoly pieces scattered on the floor and table while cartoons flickered on the television screen. Michael Clark teased his little brother by peeking from behind the curtain with Mr. Potato Head in his hand, and Matthew Clark ignored him while he cuddled close to their father on the sofa. These two brothers clearly had the mindset of young children, however they were two fully grown men who only shared the same behavior and attitude as little boys. Similar to the character portrayed in the 2008 film “The Curious Case of Benjamin Button” the two brothers were aging backwards. Not physically, but mentally regressing …show more content…

In this case Matthew and Michael Clark obviously carried the gene but managed to live healthy childhood and young adult lives, but develop Leukodystrophy later in their adult life. Adrenoleukodystrophy is an X-linked peroxisomal disorder which causes problems in the person's peroxisomes, which are tiny cellular structures involved in the breakdown of large molecules of fats into smaller ones that their body can use. The disease is caused by a mutation of the ABCD1 gene that is carried on the X chromosome. This gene produces the adrenal protein. Persons with ALD have peroxisomes that are unable to break down a type of fat called, ‘Very Long Chain Fatty Acid,’ or VLCFA. Because of this, VLCFA’s accumulate throughout the person’s body, particularly in their adrenal glands, blood, and myelin. Accumulation of VLCFA’s interferes with their adrenal gland’s ability to convert cholesterol into steroids, prompting deterioration of the myelin covering their nerve cells within the white matter of their brain and interfering with their nerve function. Fats that are usually produced through the breakdown of products of VLCFA’s also cannot be produced, and due to this the person’s nerve functioning is further compromised. Although this disorder was initially thought to occur only in childhood, it is now apparent that it can occur over a wide age spectrum with considerable clinical

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