Document Detail

Biochemical and clinical effects of tyrosine and tryptophan in the Rett syndrome.
MedLine Citation:
PMID:  1693042     Owner:  NLM     Status:  MEDLINE    
In the Rett syndrome (RS), a progressive encephalopathy affecting girls, deficient neurotransmitter synthesis has been suggested as a pathogenetic mechanism. Nine girls with RS were treated with 0.3 g of tyrosine and 0.1 g of tryptophan per kg body weight for 2 to 17 weeks. This resulted in a median rise in the spinal fluid concentration of the dopamine metabolite homovanillic acid by 31%, and of the serotonin metabolite 5-hydroxyindoleacetic acid by 40%. This finding supports the hypothesis of a compromised neurotransmitter synthesis and indicates that it can be stimulated by supply of amino acid precursors. A double-blind cross-over trial including 11 girls did not show clinical improvement during a treatment period of 8 to 10 weeks.
J B Nielsen; H C Lou; J Andresen
Publication Detail:
Type:  Clinical Trial; Controlled Clinical Trial; Journal Article    
Journal Detail:
Title:  Brain & development     Volume:  12     ISSN:  0387-7604     ISO Abbreviation:  Brain Dev.     Publication Date:  1990  
Date Detail:
Created Date:  1990-06-28     Completed Date:  1990-06-28     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7909235     Medline TA:  Brain Dev     Country:  JAPAN    
Other Details:
Languages:  eng     Pagination:  143-7     Citation Subset:  IM    
Department of Neuropediatrics, John F Kennedy Institute, Glostrup, Denmark.
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MeSH Terms
Dopamine / metabolism
Double-Blind Method
Homovanillic Acid / cerebrospinal fluid*
Hydroxyindoleacetic Acid / cerebrospinal fluid*
Rett Syndrome / drug therapy*,  metabolism
Tryptophan / therapeutic use*
Tyrosine / therapeutic use*
Reg. No./Substance:
306-08-1/Homovanillic Acid; 54-16-0/Hydroxyindoleacetic Acid; 55520-40-6/Tyrosine; 73-22-3/Tryptophan

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

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