INVIVO MAGNETIC-RESONANCE SPECTROSCOPY OF BRAIN AND MUSCLE IN A TYPE OF MITOCHONDRIAL ENCEPHALOMYOPATHY (MERRF)

被引:35
作者
MATTHEWS, PM [1 ]
BERKOVIC, SF [1 ]
SHOUBRIDGE, EA [1 ]
ANDERMANN, F [1 ]
KARPATI, G [1 ]
CARPENTER, S [1 ]
ARNOLD, DL [1 ]
机构
[1] MCGILL UNIV,MONTREAL NEUROL INST,3801 UNIV ST,MONTREAL H3A 2B4,QUEBEC,CANADA
关键词
D O I
10.1002/ana.410290416
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Phosphorus magnetic resonance spectroscopy allows noninvasive measurement of the intracellular phosphate-containing metabolites and intracellular pH in localized volumes of human muscle and brain in vivo. This technique was used to study 8 patients with a mitochondrial cytopathy (myoclonus epilepsy with ragged red fibers). Phosphorus magnetic resonance spectroscopy of resting gastrocnemius muscle demonstrated significantly increased relative intracellular inorganic phosphate concentrations (p < 0.0005) and decreased phosphocreatine to inorganic phosphate concentration ratios (p < 0.01) in the patients, although only 3 had myopathic signs or symptoms. We propose, therefore, that phosphorus magnetic resonance spectroscopy of resting skeletal muscle is a useful clinical test in evaluation of progressive myoclonus epilepsy. In contrast to results from muscle, however, the relative phosphate metabolite concentrations and intracellular pH in central volumes of the brains of these patients were normal, despite evidence from our previous positron emission tomography studies suggesting that there is diffuse impairment of cerebral oxidative metabolism.
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页码:435 / 438
页数:4
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