Transient improvement of pyruvate metabolism after coenzyme Q therapy in Kearns-Sayre syndrome: MRS study

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Coenzyme Q therapy has been used to support metabolic derangements in patients with mitochondrial encephalomyopathies. Biochemical analysis of the living human brain can be performed by magnetic resonance spectroscopy (MRS). We report upon a KSS patient who was serially imaged with localized proton MRS to monitor the efficacy of CoQ treatment. A 17-year-old girl with KSS was serially imaged with localized proton MRS performed on a GE 1.5 T SIGNA MRI/MRS system. The elevated lactate contents of lesions decreased after one month of CoQ therapy but were re-elevated 10 months after:treatment. We conclude that MRS presents us with a powerful tool for monitoring the effects of therapeutic trials in mitochondrial encephalomyopathies.
Publisher
YONSEI UNIV COLLEGE MEDICINE
Issue Date
2000-10
Language
English
Article Type
Article
Keywords

MITOCHONDRIAL MYOPATHIES; ENCEPHALOMYOPATHIES; SPECTROSCOPY

Citation

YONSEI MEDICAL JOURNAL, v.41, no.5, pp.676 - 679

ISSN
0513-5796
URI
http://hdl.handle.net/10203/69946
Appears in Collection
BiS-Journal Papers(저널논문)
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