Very High Plasma Leucine Concentrations Without Neurological Symptoms in a Patient with Classical Maple Syrup Urine Disease

Journal of Inherited Metabolic DiseaseVolume 23, Issue 5 p. 513-514 Article Very high plasma leucine concentrations without neurological symptoms in a patient with classical maple syrup urine disease D. Skladal, D. Skladal Universitätsklinik für Kinder- und Jugendheilkunde, Innsbruck, AustriaSearch for more papers by this authorG. Grissenauer, G. Grissenauer Universitätsklinik für Kinder- und Jugendheilkunde, Innsbruck, AustriaSearch for more papers by this authorV. Konstantopoulou, V. Konstantopoulou Universitätsklinik für Kinder- und Jugendheilkunde, Innsbruck, AustriaSearch for more papers by this authorS. Felber, S. Felber Universitätsklinik für Neurologie, Innsbruck, AustriaSearch for more papers by this authorW. Sperl, W. Sperl Kinderspital, Salzburg, AustriaSearch for more papers by this author D. Skladal, D. Skladal Universitätsklinik für Kinder- und Jugendheilkunde, Innsbruck, AustriaSearch for more papers by this authorG. Grissenauer, G. Grissenauer Universitätsklinik für Kinder- und Jugendheilkunde, Innsbruck, AustriaSearch for more papers by this authorV. Konstantopoulou, V. Konstantopoulou Universitätsklinik für Kinder- und Jugendheilkunde, Innsbruck, AustriaSearch for more papers by this authorS. Felber, S. Felber Universitätsklinik für Neurologie, Innsbruck, AustriaSearch for more papers by this authorW. Sperl, W. Sperl Kinderspital, Salzburg, AustriaSearch for more papers by this author First published: 01 July 2000 https://doi.org/10.1023/A:1005620515996AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. REFERENCES 1Felber SR, Sperl W, Chemelli A, Murr C., Wendel U (1993) Maple syrup urine disease: metabolic decompensation monitored by proton magnetic resonance imaging and spectroscopy. Ann Neurol, 33: 396–401. 10.1002/ana.410330412 PubMedWeb of Science®Google Scholar 2Korein J, Sansaricq C, Kalmijn M, Honig J, Lange B (1994) Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity. Int J Neurosci, 79: 21–45. 10.3109/00207459408986065 CASPubMedGoogle Scholar 3Treacy E, Clow CL, Reade TR, Chitayat D, Mamer OA, Scriver CR (1992) Maple syrup urine disease: interrelations between branched-chain amino-, oxo-and hydroxyacids; implications for treatment; associations with CNS dysmyelination. J Inherit Metab Dis, 15: 121–135. 10.1007/BF01800354 CASPubMedWeb of Science®Google Scholar Volume23, Issue5July 2000Pages 513-514 ReferencesRelatedInformation