Effect of coenzyme Q10 in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): Evaluation by noninvasive tissue oximetry

Abe, Kazuo; Matsuo, Yoshimi; Kadekawa, Jun; Inoue, Sumito; Yanagihara, Takehiko

doi:10.1016/s0022-510x(98)00296-2

Cited by 62 publications

(27 citation statements)

References 13 publications

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“…In this population, the most common therapeutic agent studied to detect the changes in metabolic function has been coenzyme Q 10 (CoQ 10 ), a cofactor in the electron transport chain (ETC) bridging complexes I and II to complex III of the ETC. In these studies, 31 P MRS has been serving as a popular tool of choice to monitor the effects of CoQ 10 in the electron facilitation and subsequent bioenergetic changes occurring within the mitochondria of skeletal muscle (144,145). Not all studies have been able to show improvements in mitochondrial function of MD patients with the aid of CoQ 10 .…”

Section: Mitochondrial Disease Mitochondrial Disease (Md)mentioning

confidence: 99%

Skeletal muscle energetics with PNMR: personal views and historic perspectives

Chance

Nioka

et al. 2006

NMR in Biomedicine

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This article reviews historical and current NMR approaches to describing in vivo bioenergetics of skeletal muscles in normal and diseased populations. It draws upon the first author's more than 70 years of personal experience in enzyme kinetics and the last author's physiological approaches. The development of in vivo PNMR jointly with researchers around the world is described. It is explained how non-invasive PNMR has advanced human exercise biochemistry, physiology and pathology. Further, after a brief explanation of bioenergetics with PNMR on creatine kinase, anerobic glycolysis and mitochondrial oxidative phosphorylation, some basic and controversial subjects are focused upon, and the authors' view of the subjects are offered, with questions and answers. Some of the research has been introduced in exercise physiology. Future directions of NMR on bioenergetics, as a part of system biological approaches, are indicated.

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Section: Mitochondrial Disease Mitochondrial Disease (Md)mentioning

confidence: 99%

Skeletal muscle energetics with PNMR: personal views and historic perspectives

Chance

Nioka

et al. 2006

NMR in Biomedicine

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“…Regarding treatment, symptomatic therapy and rehabilitation are the focus. Oral coenzyme Q10 and intravenous cytochrome preparation are administered [3,4] . However, multiple sclerosis sometimes causes dizziness and shows similar MRI findings as mitochondrial encephalomyopathy.…”

Section: Discussionmentioning

confidence: 99%

Repeated Attacks of Dizziness Caused by a Rare Mitochondrial Encephalomyopathy

et al. 2018

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Cases of dizziness caused by multiple sclerosis are commonly reported, but those caused by mitochondrial encephalomyopathy have been rarely reported. Particularly, the description of eye nystagmography (ENG) using caloric and optokinetic nystagmus tests has not been reported to date. We encountered the case of a 40-year-old woman with mitochondrial encephalomyopathy who visited us with the chief complaint of dizziness. At first, we considered multiple sclerosis based on the magnetic resonance imaging (MRI) findings and dizziness. Repeated attacks of dizziness and serum lactic acid levels suggested mitochondrial encephalomyopathy. A muscle biopsy confirmed the diagnosis. ENG findings suggested central vestibular disorder of the cerebellum and brainstem. This case suggests that we should not rule out the differential diagnosis of a very rare mitochondrial encephalomyopathy in patients who experience dizziness with MRI findings indicative of multiple sclerosis.

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“…Actualmente no hay tratamiento curativo, sin embargo se ha reportado que la suplementación con antioxidantes, sustratatos y cofactores de la cadena respiratoria en forma de vitaminas como la tiamina (B1), rivoflavina (B2), nicotinamida (niacinamida), coenzima Q10 (ubidecarenona), idebenona, succinato, citocromo C, menadiona (vitamina K3), ácido ascórbico, (vitamina C), ácido tioctico (ácido alfa lipoico), corticoides, coenzima Q puede ser clínica y bioquímicamente beneficiosa (8).…”

Section: Rev Neuropsiquiatr 78 (4) 2015unclassified

“…Los esquemas terapéuticos en MELAS se basan en antioxidantes, sustratatos y cofactores de la cadena respiratoria en forma de vitaminas como la tiamina (B1), rivoflavina (B2), nicotinamida (niacinamida), coenzima Q10 (ubidecarenona), idebenona, succinato, citocromo C, menadiona ( vitamina K3), ácido ascórbico, (vitamina C), ácido tioctico (ácido alfa lipoico), corticoides (8). En el caso de la paciente se usa coenzima Q 10, vitamina E y triptorelina, con evolución estable.…”

Section: Rev Neuropsiquiatr 78 (4) 2015unclassified