{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T02:28:34Z","timestamp":1792290514772,"version":"4.4.0"},"reference-count":31,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,10,8]],"date-time":"2004-10-08T00:00:00Z","timestamp":1097193600000},"content-version":"vor","delay-in-days":6431,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1987,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A new dietary regimen has been administered for periods ranging from 60 days to 1 1\/2 years in 34 patients with various forms of X\u2010linked adrenoleukodystrophy (ALD), as well as in 1 patient with neonatal ALD and 1 patient with infantile Refsum's disease. The diet combines the administration of a glyceryl trioleate oil (GTO) with the dietary restriction of very\u2010long\u2010chain fatty acids (VLFA), particularly hexacosanoic acid (C26:0). Reductions in the levels of plasma C26:0 and other VLFA were achieved in 25 of the 36 patients. Fifteen of these 25 patients were treated for more than 100 days. The mean reduction of the plasma C26:0 level was 53% (range, 22 to 73%) in these 15 patients. While the focus of the study was on biochemical variables, comparison of pre\u2010 and post\u2010diet studies of peripheral nerve function showed improvement in 1 patient with adrenomyeloneuropathy (AMN) and 1 heterozygote. In contrast, 2 patients with ALD onset in childhood developed new neurological deficits while on therapy. We conclude that it is possible to lower plasma VLFA levels in ALD patients. A clinical trial is indicated to test whether this approach can alter the neurological progression in patients with AMN or in symptomatic heterozygotes, and to determine whether it can prevent the onset of neurological disability in asymptomatic persons who have the biochemical defect of ALD.<\/jats:p>","DOI":"10.1002\/ana.410210305","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T17:41:27Z","timestamp":1104601287000},"page":"240-249","source":"Crossref","is-referenced-by-count":104,"title":["A new dietary therapy for adrenoleukodystrophy: Biochemical and preliminary clinical results in 36 patients"],"prefix":"10.1002","volume":"21","author":[{"given":"Ann B.","family":"Moser","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janet","family":"Borel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Augusto","family":"Odone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sakkubai","family":"Naidu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Cornblath","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald B.","family":"Sanders","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugo W.","family":"Moser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,10,8]]},"reference":[{"key":"e_1_2_1_2_2","volume-title":"ALD\/AMN Diet Cookbook","author":"Borel JS","year":"1986"},{"key":"e_1_2_1_3_2","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/S0022-2275(20)38153-0","article-title":"Metabolism of very long chain monounsaturated fatty acids (C22:1) and the adaptation to their presence in the diet","volume":"23","author":"Bremer J","year":"1982","journal-title":"J Lipid Res"},{"key":"e_1_2_1_4_2","first-page":"344","article-title":"Cerebrohepato\u2010renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids","volume":"151","author":"Brown FR","year":"1982","journal-title":"Johns Hopkins Med J"},{"key":"e_1_2_1_5_2","first-page":"164","article-title":"Adrenoleukodystrophy: effects of dietary restriction of very long chain fatty acids and of the administration of carnitine and clofibrate on clinical status and plasma fatty acids","volume":"151","author":"Brown FR","year":"1982","journal-title":"Johns Hopkins Med J"},{"key":"e_1_2_1_6_2","unstructured":"ChenWW WatkinsPA HashimotoT MoserHW:Peroxisomal beta\u2010oxidation enzymes in adrenoleukodystrophy. 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