Document Detail

Effects of erucic acid therapy on Japanese patients with X-linked adrenoleukodystrophy.
MedLine Citation:
PMID:  7694994     Owner:  NLM     Status:  MEDLINE    
Ten Japanese boys with childhood adrenoleukodystrophy (ALD), one adult patient with adrenomyeloneuropathy (AMN), and two presymptomatic ALD boys were treated with dietary erucic acid (C22:1) for more than 12 months; except in a case of childhood ALD patient who died 7 months after beginning erucic acid therapy. During erucic acid therapy, the serum levels of very long-chain fatty acid (VLCFA) (C24:0/C22:0) decreased within 1-2 months in all patients, and these levels in four of the patients decreased to the normal range. Neurological examination and MRI findings in all 10 of the childhood ALD patients showed progression of the disease while they were receiving the dietary therapy. However, the mean interval between the onset of awkward gait and a vegetative state in diet-treated patients was significantly longer than that in the untreated patients. One AMN patient showed slight improvement of spastic gait and lessened pain in the lower limbs due to spasticity. The two presymptomatic ALD boys remained intact on clinical examination and on MRI findings for 38 and 23 months, respectively, after starting the diet.
J Asano; Y Suzuki; S Yajima; K Inoue; N Shimozawa; N Kondo; M Murase; T Orii
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Brain & development     Volume:  16     ISSN:  0387-7604     ISO Abbreviation:  Brain Dev.     Publication Date:    1994 Nov-Dec
Date Detail:
Created Date:  1995-04-27     Completed Date:  1995-04-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7909235     Medline TA:  Brain Dev     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  454-8     Citation Subset:  IM    
Department of Pediatrics, Gifu University School of Medicine, Japan.
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MeSH Terms
Adrenoleukodystrophy / drug therapy*
Age Factors
Child, Preschool
Diagnosis, Differential
Diet Therapy
Erucic Acids / therapeutic use*
Fatty Acids / metabolism
Follow-Up Studies
Magnetic Resonance Imaging
Motor Activity
Treatment Outcome
Reg. No./Substance:
0/Erucic Acids; 0/Fatty Acids

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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