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Adrenoleukodystrophy

Abnormality of very long-chain fatty acids in erythrocyte membrane phospholipids

From the Department of Neurology (Drs. Antoku, Sakai, and Iwashita), National Chikugo Hospital, Chikugo City, and the Department of Neurology (Drs. Goto and Kuroiwa), Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka, Japan.

Long-chain fatty acids in erythrocyte membrane phospholipids were analyzed in three patients with adrenoleukodystrophy (ALD) and four diseased controls with other neurologic diseases by high performance liquid chromatography (HPLC). HPLC chromatograms showed the increase of a very long-chain fatty acid C_26:0 in glycerophospholipids of ALD erythrocyte membranes, which was not so prominent in sphingomyelin. The ratio of C_26:0 to C_22:0 increased in glycerophospholipids as well as sphingomyelin in ALD. These results suggest that ALD has a generalized abnormal metabolism of very long-chain saturated fatty acids.

Address correspondence and reprint requests to Dr. Antoku, Department of Neurology, National Chikugo Hospital, 515 Kurakazu, Chikugo City, 833 Japan.

Accepted for publication March 20, 1984.