In vivo magnetic resonance spectroscopy of brain and muscle in a type of mitochondrial encephalomyopathy (MERRF)
Identifieur interne : 000F07 ( Istex/Corpus ); précédent : 000F06; suivant : 000F08In vivo magnetic resonance spectroscopy of brain and muscle in a type of mitochondrial encephalomyopathy (MERRF)
Auteurs : P. M. Matthews ; S. F. Berkovic ; E. A. Shoubridge ; F. Andermann ; G. Karpati ; S. Carpenter ; S. ArnoldSource :
- Annals of Neurology [ 0364-5134 ] ; 1991-04.
Abstract
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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DOI: 10.1002/ana.410290416
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<front><div type="abstract" xml:lang="en">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.</div>
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<p>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 (<i>p</i>
< 0.0005) and decreased phosphocreatine to inorganic phosphate concentration ratios (<i>p</i>
< 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.</p>
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<mods version="3.6"><titleInfo lang="en"><title>In vivo magnetic resonance spectroscopy of brain and muscle in a type of mitochondrial encephalomyopathy (MERRF)</title>
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<titleInfo type="abbreviated" lang="en"><title>In Vivo MRS in MERRF</title>
</titleInfo>
<titleInfo type="alternative" contentType="CDATA" lang="en"><title>In vivo magnetic resonance spectroscopy of brain and muscle in a type of mitochondrial encephalomyopathy (MERRF)</title>
</titleInfo>
<name type="personal"><namePart type="given">P. M.</namePart>
<namePart type="family">Matthews</namePart>
<namePart type="termsOfAddress">MD, DPhil</namePart>
<affiliation>Montreal Neurological Institute, McGill University, Montreal, Canada</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">S. F.</namePart>
<namePart type="family">Berkovic</namePart>
<namePart type="termsOfAddress">MD, FRACP</namePart>
<affiliation>Montreal Neurological Institute, McGill University, Montreal, Canada</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">E. A.</namePart>
<namePart type="family">Shoubridge</namePart>
<namePart type="termsOfAddress">PhD</namePart>
<affiliation>Montreal Neurological Institute, McGill University, Montreal, Canada</affiliation>
<role><roleTerm type="text">author</roleTerm>
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</name>
<name type="personal"><namePart type="given">F.</namePart>
<namePart type="family">Andermann</namePart>
<namePart type="termsOfAddress">MD, FRCP(C)</namePart>
<affiliation>Montreal Neurological Institute, McGill University, Montreal, Canada</affiliation>
<role><roleTerm type="text">author</roleTerm>
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</name>
<name type="personal"><namePart type="given">G.</namePart>
<namePart type="family">Karpati</namePart>
<namePart type="termsOfAddress">MD, FRCP(C)</namePart>
<affiliation>Montreal Neurological Institute, McGill University, Montreal, Canada</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
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<name type="personal"><namePart type="given">S.</namePart>
<namePart type="family">Carpenter</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Montreal Neurological Institute, McGill University, Montreal, Canada</affiliation>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="termsOfAddress">Dr</namePart>
<namePart type="family">Arnold</namePart>
<namePart type="termsOfAddress">MD, FRCP(C)</namePart>
<affiliation>Montreal Neurological Institute, McGill University, Montreal, Canada</affiliation>
<affiliation>Montreal Neurological Institute, McGill University, 3801 University St, Montreal, PQ, H3A‐2B4 Canada</affiliation>
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<dateIssued encoding="w3cdtf">1991-04</dateIssued>
<dateCaptured encoding="w3cdtf">1990-06-08</dateCaptured>
<dateValid encoding="w3cdtf">1990-09-30</dateValid>
<copyrightDate encoding="w3cdtf">1991</copyrightDate>
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<abstract lang="en">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.</abstract>
<relatedItem type="host"><titleInfo><title>Annals of Neurology</title>
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<titleInfo type="abbreviated"><title>Ann Neurol.</title>
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<subject><genre>article-category</genre>
<topic>Brief Communication</topic>
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<identifier type="ISSN">0364-5134</identifier>
<identifier type="eISSN">1531-8249</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8249</identifier>
<identifier type="PublisherID">ANA</identifier>
<part><date>1991</date>
<detail type="volume"><caption>vol.</caption>
<number>29</number>
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<detail type="issue"><caption>no.</caption>
<number>4</number>
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<extent unit="pages"><start>435</start>
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<accessCondition type="use and reproduction" contentType="copyright">Copyright © 1991 American Neurological Association</accessCondition>
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