Adrenoleukodystrophy

January 26, 1993|By Jean Marbella

Adrenoleukodystrophy, or ALD, is a disorder resulting from an abnormal gene passed from mother to son. This genetic mutation causes substances called very long chain fatty acids to accumulate. That, in turn, damages the myelin, the material that coats nerve fibers in the brain much like the insulating material that protects telephone wire. The myelin damage -- which to date is irreversible -- is what causes the neurological system to break down.

While there are various forms of ALD, the most prevalent is the childhood cerebral form portrayed in "Lorenzo's Oil." It is particularly wrenching to watch -- seemingly normal little boys like the movie's Lorenzo Odone, usually from 4 to 10 years of age, suddenly and in rapid progression begin losing hearing, speech, sight and motor skills. Within two years, those with the most severe cases are helpless and eventually die.

Lorenzo's Oil, a form of erucic acid (a component of rapeseed oil), was so named to acknowledge the role Augusto and Michaela Odone played in developing it as a therapy for the disorder. The theory is that by introducing another fatty acid, the body becomes too busy to manufacture the ones destroying the myelin. Lorenzo's Oil indeed has had the effect of reducing very long chain fatty acids in the blood, but Dr. Hugo Moser, a Kennedy Krieger Institute researcher, warns that further study is needed to determine its role in treating ALD.

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