Adrenoleukodystrophy and Myelin Essay -- Biology Essays Research Paper

Adrenoleukodystrophy and Myelin: Where's the Connection? Adrenoleukodystrophy. Also known as ALD. Ten years ago many people would not be familiar with this extremely rare childhood disease. But now awareness has increased as a result of the 1992 MCA Universal film, "Lorenzo's Oil". This true story movie shows the struggles of a family whose son is dying from this disease and their fight to try to save him and children like him. After seeing this movie myself I became very interested in ALD and the possible ways to cure or treat it. In the last decade there has been some amazing research done in conjunction with this disease and the few others who, like it, occur as a result from the breakdown or loss of myelin in the brain. The first step in understanding adrenoleukodystrophy is to ask what it is and how it works. According to the fact sheet put out by the National Institute of Health in Bethesda, MD in 1997, "ALD is a rare, genetic disorder characterized by the breakdown or loss of the myelin sheath surrounding nerve cells in the brain and progressive dysfunction of the adrenal gland" (1). This disorder affects fatty acid metabolism which causes the dysfunction of the adrenal glands, the nervous system, and the testes (2). This leads to progressive neurological disability and death (3). Physical symptoms include, but are not limited to, visual loss, deafness, learning disabilities, seizures, dysarthria (poorly articulated speech), dysphagia (difficulty swallowing), strabismus (crossed eyes), fatigue, disturbance in gait and coordination, vomiting, paralysis, blindness, melanoderma, and dementia (1) (2) (4). But this disease does not stop with merely physical changes, there are many behavioral changes which are parti... ...gs are almost never what you think they are. WWW Sources 1)Adrenoleukodystrophy, http://www.ninds.nih.gov/HEALINFO/DISORDER/Adrenoleukodystrophy/adrenoleuko.html 2)Adrenoleukodystrophy, http://www.healthanswers.com/database/ami/converted/001182.html 3)NCBI: Adrenoleukodystrophy, http://www.ncbi.nlm.nih.gov/disease/ALD.html 4) ADRENOLEUKODYSTROPHY , http://www.kid-med.org/pedbase/adrenole.htm 5)The Myelin Project , http://www.myelin.org/ 6)TMP Newsletter , http://www.myelin.org/112698pr.html 7)Virtual Hospital: Radiology Resident Case of the Week Adrenal Leukodystrophy (ALD) , http://www.vh.org/adult/provider/radiology/RCW/012696/012696.html 8) Neurodegenerative Disorders of Childhood- Dr. Greene's House Calls , http://www.drgreene.com/21_525.html 9) Metachromatic Leukodystrophy , http://www.duke.edu/~pdrh/MLD.html