Yonsei Med J. 2000 Oct;41(5):676-679. English.
Published online Apr 07, 2009.
https://doi.org/10.3349/ymj.2000.41.5.676
Published online Apr 07, 2009.
https://doi.org/10.3349/ymj.2000.41.5.676
Copyright © 2000 The Yonsei University College of Medicine
Case Report
Transient improvement of pyruvate metabolism after coenzyme Q therapy in Kearns-Sayre syndrome: MRS study
Chulhee Choi,1,2
Il Nam Sunwoo,2
Hyun Sook Kim,3
and Dong Ik Kim3
Abstract
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.
Keywords
Kearns-Sayre syndrome; MRS; CoQ; lactate
MeSH Terms
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