期刊
NEUROSCIENCE LETTERS
卷 306, 期 3, 页码 169-172出版社
ELSEVIER SCI IRELAND LTD
DOI: 10.1016/S0304-3940(01)01892-4
关键词
Friedreich ataxia; magnetic resonance spectroscopy; idebenone; mitochondrial impairment; respiratory function; cardiomyopathy; controlled trial
Friedreich ataxia (FA), the most common form of degenerative ataxia, is thought to be caused by respiraton/deficiency due to mitochondrial iron accumulation and oxidative stress. Idebenone, a free-radical scavenger, protects mitochondrial function in in vitro models of FA. In a placebo-controlled crossover trial we studied the effect of idebenone on respiratory function in nine ambulant FA patients. P-31 magnetic resonance spectroscopy demonstrated mitochondrial impairment in vivo in skeletal muscle of all FA patients, but no recovery with idebenone. No effects were seen in clinical sco res. Echocardiography did not confirm a preliminary study reporting improvement of FA-associated cardiomyopathy with idebenone. (C) 2001 Elsevier Science Ireland Ltd. All rights reserved.
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