Nutrition of patients with adrenoleucodystrophy (ALD) and adrenomyeloneuropathy (AMN)

Kathrin Jürgens, Jana Kraft und Gerhard Jahreis, Jena

Adrenoleucodystrophy is a rare x-linked, recessive disorder, characterized by an abnormal accumulation of saturated very long-chain fatty acids (VLCFA) in body tissues and plasma, associated with a progressive demyelinization of the central nervous system accompanied by adrenal insufficiency (M. Addison). The symptoms range from slowly progressing gait disorders to severe – often fast progressing and fatal – cerebral deficits such as blindness, deafness and dementia.

To date, effective therapies are not available. VLCFA plasma levels may be normalized by a diet low in fat and VLCFA in combinatiom with intake of Lorenzo’s oil®, a mixture of monounsaturated oleic acid (C18:1) and erucic acid (C22:1 n-9) which competitively inhibit the endogenous synthesis of VLCFA. Major aim of the present study were reliable dietary recommendations for German ALD patients in view of the fact that existing advices are often unclear and to some extent incorrect. VLCFA contents of 68 common German food items were analyzed.

ALD patients should generally prefer a low-fat diet and avoid food containing high amounts of VLCFA such as peanuts and peanut oil. Intake of high-fat dairy products, bananas, whole-grain based bread and other products containing moderate amounts of VLCFA should be reduced. VLCFA intake may be reduced (by about 65 %) by an appropriate choice of food items. A mixture of special plant oils was developed which could be a promising and less expensive alternative to the high-priced Lorenzo’s oil®.

Key words: adrenoleucodystrophy, adrenomyeloneuropathy, erucic acid, Lorenzo´s oil, oleic acid, very long chain fatty acids

Sie finden den Artikel in deutscher Sprache in Ernährungs Umschau 04/07 ab Seite 188.