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NEUROLOGY 1998;51:334
© 1998 American Academy of Neurology

Adrenoleukodystrophy

Increased plasma content of saturated very long chain fatty acids

Hugo W. Moser, MD, Ann B. Moser, AB, Karen K. Frayer, AB, Winston Chen, PhD, Joseph D. Schulman, MD, Brian P. O'Neill, MD and Yasuo Kishimoto, PhD

From the John F. Kennedy Institute (Drs. Moser, Chen, and Kishimoto, Ms. Moser, and Ms. Frayer), the Departments of Neurology and Pediatrics, Johns Hopkins University (Dr. Moser), the National Institutes of Health, Child Health and Human Development (Dr. Schulman), and the Department of Neurology, Mayo Clinic and Foundation (Dr. O'Neill), Rochester, MN.

Address correspondence and reprint requests to Dr. Moser, John F. Kennedy Institute, 707 N. Broadway, Baltimore, MD 21205.

Abstract.

With a new method we measured the saturated very long chain fatty acids in the plasma of adrenoleukodystrophy (ALD) hemizygotes, ALD heterozygotes, and controls. ALD hemizygotes showed increased levels of hexacosanoate (C26 fatty acid) which represented 0.081 ± 0.0066% (SEM) of total fatty acids, compared to 0.015 ± 0.0032% in the controls. C25, C24, and C23 fatty acids were also increased, but the C22 and C20 fatty acids were normal. C26 levels were also increased in most ALD heterozygotes, with a mean level 0.057± 0.0063% of total fatty acids. The technique can be used for diagnosis and carrier identification, and in the evaluation of therapy.

Footnotes

Supported in part by Grants HD 10981 and NS 10885 and 13513 from the Public Health Service and by Grant 6-271 from the National Foundation-March of Dimes.

Accepted for publication February 11, 1981.

American Academy of Neurology Dedicated to advancing the art and science of neurology, and thereby promoting the best possible care for patients with neurological disorders A Celebration of the First 50 Years of the American Academy of Neurology






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