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We investigated the biochemical and clinical efficacy of dietary erucic acid(C22:1)therapy for X-linked adrenoleukodystrophy(ALD).In a double- blind crossover study of patients who were on chronic oleic acid(C18:1) therapy,addition of erucic acid to the diet led to a further reduction in plasma hexacosanoic acid(C26:0)concentration.We treated 12 newly diagnosed ALD patients with a diet enriched with erucic acid and oleic acid for 2 to 19 months.Mean plasma C26:0 concentration decreased to normal by 4 weeks, and the C26:0 composition of plasma sphingomyelin and phosphatidyclcholine became normal by 4 months on therapy.Fatty acid analysis of postmortem tissues from 1 boy treated for 10 months suggested that dietary erucic acid entered the heart,liver,adrenal gland,and brain.Eight patients remained on treatment long enough(mean,12+/-3 months)to evaluate their clinical response;6 of these patients with moderate to advanced disease deteriorated neurologically or showed progression of white matter disease on brain magnetic resonance imaging whereas 2 mildly affected patients remained clinically stable after 10 and 19 months.No adverse effects of the diet occurred.We conclude that dietary erucic acid therapy is effective in lowering plasma C26:0 to normal in ALD patients,and may prevent further demyelination in some mildly affected boys. |
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