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L-carnitine and creatine in Friedreich’s ataxia. A randomized, placebo-controlled crossover trial

Schöls, Ludger and Zange, Jochen and Abele, M. and Schillings, M. and Skipka, G. and Kuntz-Hehne, S. and van Beekvelt, Mireille C.P. and Colier, Willy N.J.M. and Müller, Klaus and Klockgether, Thomas and Przuntek, Hans and Vorgerd, Matthias (2005) L-carnitine and creatine in Friedreich’s ataxia. A randomized, placebo-controlled crossover trial. Journal of Neural Transmission, 112 (6), pp. 789-796.

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Impaired oxidative phosphorylation is a crucial factor in the pathogenesis of Friedreich’s ataxia (FA). L-carnitine and creatine are natural compounds that can enhance cellular energy transduction. We performed a placebo-controlled triple-phase crossover trial of L-carnitine (3 g=d) and creatine (6.75 g=d) in 16 patients with genetically confirmed FA. Primary outcome measures were mitochondrial ATP production measured as phosphocreatine recovery by <sup>31</sup>Phosphorus magnetic resonance spectroscopy, neurological deficits assessed by the international co-operative ataxia rating scale and cardiac hypertrophy in echocardiography. After 4 months on L-carnitine phosphocreatine recovery was improved compared to baseline (p<0.03, t-test) but comparison to placebo and creatine effects did not reach significance (p<0.06, F-test). Ataxia rating scale and echocardiographic parameters remained unchanged. Creatine had no effect in FA patients. L-carnitine is a promising substance for the treatment of FA patients, and larger trials are warranted.

Item URL in elib:https://elib.dlr.de/21039/
Document Type:Article
Title:L-carnitine and creatine in Friedreich’s ataxia. A randomized, placebo-controlled crossover trial
AuthorsInstitution or Email of AuthorsAuthor's ORCID iD
Schöls, LudgerUniversität TübingenUNSPECIFIED
Abele, M.Universität BonnUNSPECIFIED
Schillings, M.Universität BochumUNSPECIFIED
Skipka, G.Universität BochumUNSPECIFIED
Kuntz-Hehne, S.Universität BonnUNSPECIFIED
van Beekvelt, Mireille C.P.Universität Nijmegen, NLUNSPECIFIED
Colier, Willy N.J.M.Universität Nijmegen, NLUNSPECIFIED
Klockgether, ThomasUniversität BonnUNSPECIFIED
Przuntek, HansUniversität BochumUNSPECIFIED
Vorgerd, MatthiasUniversität BochumUNSPECIFIED
Journal or Publication Title:Journal of Neural Transmission
Refereed publication:Yes
Open Access:No
Gold Open Access:No
In ISI Web of Science:Yes
Page Range:pp. 789-796
Keywords:Friedreich’s ataxia, therapy, L-carnitine, oxidative phosphorylation, magnetic resonance spectroscopy
HGF - Research field:Aeronautics, Space and Transport (old)
HGF - Program:Space (old)
HGF - Program Themes:W FR - Forschung unter Weltraumbedingungen (old)
DLR - Research area:Space
DLR - Program:W FR - Forschung unter Weltraumbedingungen
DLR - Research theme (Project):W - no assignment (old)
Location: Köln-Porz
Institutes and Institutions:Institute of Aerospace Medicine > Space Physiology
Deposited By: Zange, Dr.rer.nat. Jochen
Deposited On:10 Jan 2006
Last Modified:27 Apr 2009 04:52

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