ArticleCNS neuroscience & therapeutics2017
Mitochondrial dysfunction and cerebral metabolic abnormalities in patients with mitochondrial encephalomyopathy subtypes: Evidence from proton MR spectroscopy and muscle biopsy.
Article in CNS neuroscience & therapeutics, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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9 citing papers in PubMed, 17 citations in OpenAlex.
- Multiparametric MRI detection of cerebral metabolism: a tool for early differentiation between MELAS and ischemic cerebral infarction.Quantitative imaging in medicine and surgery · 2025Article
- Sexual Dimorphism in Migraine. Focus on Mitochondria.Current pain and headache reports · 2025Review
- Multisystem clinicopathologic and genetic analysis of MELAS.Orphanet journal of rare diseases · 2024Article
- Magnetic resonance spectroscopy in MELAS syndrome: correlation with CSF and plasma metabolite levels and change after glutamine supplementation.Neuroradiology · 2024Observational
- ENT characteristics and therapeutic results in multisystemic disorders of mitochondrial encephalomyopathy.European journal of medical research · 2022Article
- Molecular and neurological features of MELAS syndrome in paediatric patients: A case series and review of the literature.Molecular genetics & genomic medicine · 2022Review
- Lactate peak in muscle disclosed by magnetic resonance spectroscopy in a patient with CPEO-plus syndrome.eNeurologicalSci · 2021Article
- Can MR spectroscopy and muscle biopsy findings be correlated with MELAS and CPEO?CNS neuroscience & therapeutics · 2017Article
- Mitochondrial dysfunction and cerebral metabolic abnormalities in patients with mitochondrial encephalomyopathy subtypes: Evidence from proton MR spectroscopy and muscle biopsy.CNS neuroscience & therapeutics · 2017Article
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9 authors at 1 institution in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
aimsAccumulated evidence indicates that cerebral metabolic features, evaluated by proton magnetic resonance spectroscopy (
methodsIn this study, cerebral metabolic features of pathologically diagnosed ME cases, that is, 19 mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS); nine chronic progressive external ophthalmoplegia (CPEO); and 23 healthy controls, were investigated using
resultsPatients with MELAS or CPEO exhibited a significantly higher Lac/Cr ratio and a lower NAA/Cr ratio compared with controls. The ROC curve of Lac/Cr ratio indicated prominent discrimination between MELAS or CPEO and healthy control subjects, whereas the NAA/Cr ratio may present diagnostic power in the distinction of MELAS from CPEO. Lower NAA/Cr ratio was associated with higher Lac/Cr in MELAS, but not in CPEO. Furthermore, higher ragged-red fibers (RRFs) percentages were associated with elevated Lac/Cr and reduced NAA/Cr ratios, notably in MELAS. This association was not noted in the case of mI/Cr ratio.
conclusionsMitochondrial cytopathy (lactic acidosis and RRFs on muscle biopsy) was associated with neuronal viability but not glial proliferation, notably in MELAS. Mitochondrial neuronopathy and neuronal vulnerability are considered significant causes in the pathogenesis of MELAS, particularly with regard to stroke-like episodes.
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