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Open Access Short report

Treatment of an adrenomyeloneuropathy patient with Lorenzo's oil and supplementation with docosahexaenoic acid-A case report

Gisella Terre'Blanche1*, Mietha M van der Walt1, Jacobus J Bergh1 and Lodewyk J Mienie2

Author Affiliations

1 Pharmaceutical Chemistry, Unit for Drug Research and Development, School of Pharmacy, North-West University, Private Bag X6001, Potchefstroom, 2520, South Africa

2 Biochemistry, School of Physical and Chemical Sciences, North-West University, Private Bag X6001, Potchefstroom 2520, South Africa

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Lipids in Health and Disease 2011, 10:152  doi:10.1186/1476-511X-10-152

Published: 26 August 2011

Abstract

This is a case report of adrenomyeloneuropathy (AMN), the adult variant of adrenoleukodystryphy (ALD). The diagnoses in the patient, aged 34, was confirmed via increased serum very long chain fatty acid concentration (VLCFA). Treatment started with the cholesterol lowering drug, atorvastatin, followed by add-on therapy with Lorenzo's oil (LO) and finally supplementation with docosahexaenoic acid (DHA). The magnetic resonance imaging (MRI) scan of the AMN patient before DHA treatment, already showed abnormal white matter in the brain. Although the MRI showed no neurological improvement after 6 months of DHA treatment, no selective progression of demyelination was detected in the AMN patient. Contrary to what was expected, LO failed to sustain or normalize the VLCFA levels or improve clinical symptoms. It was however, shown that DHA supplementation in addition to LO, increased DHA levels in both plasma and red blood cells (RBC). Additionally, the study showed evidence that the elongase activity in the elongation of eicosapentaenoic acid (EPA) to docosapentaenoic acid (DPA) might have been significantly compromised, due to the increased DHA levels.

Keywords:
Adrenoleukodystrophy; Adrenomyeloneuropathy; Lorenzo's oil; Docosahexaenoic acid; polyunsaturated fatty acids