Lorenzo’s Oil and ALD
Disease still not fully understood
The movie "Lorenzo's Oil" brought a rare disorder called adrenoleukodystrophy (ALD). It is a progressive degenerative myelin disorder, meaning that myelin, the "insulation" around nerves, breaks down over time. Without myelin, nerves can't function normally, or at all. Unfortunately, the body can't grow replacement myelin, so the disorder is progressive -- it gets worse over time. Nerve Cell diagram:
What Causes ALD, And Who Carry It?
ALD is an inherited recessive genetic disorder linked to the X chromosome. Because of the way genetic inheritance works, only boys have the most severe form of ALD. Approximately 20 percent of females who carry the defective gene for ALD develop nervous system symptoms that resemble adrenomyeloneuropathy, but this begins later in life (age 35 years or later) and results in a milder disease than in affected males.
There are several forms that ALD can take. The most devastating type, what Lorenzo Odone had, is the childhood cerebral form, meaning that nerves in the brain are destroyed. About 35 to 40 percent of all cases of ALD are this type, which usually appears between 4 and 8 years of age in boys. These boys will usually become totally disabled in 6 months to 2 years, and die sometime after.
Unfortunately, there is as yet no complete cure for cerebral ALD. However, there are several methods being tried that seem to slow down the destruction of the disease. One method is the use of "Lorenzo's oil," made from olive and rapeseed oil, and a very low-fat diet. The oil, if started early in boys with ALD but no symptoms, is now known to have some benefit in preventing the childhood cerebral form of adrenoleukodystrophy. Researchers are still trying to understand the complex relationships among fats in the body, and how Lorenzo's oil could be further modified to be more effective.
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