Mitochondrial encephalomyopathy (MELAS): successful therapy with idebenone

Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q10 and idebenone.

Ihara Y, Namba R, Kuroda S, Sato T, Shirabe T.

Department of Neurology,
National Minamiokayama Hospital, Okayama, Japan.
J Neurol Sci 1989 May;90(3):263-71

ABSTRACT

Two patients with mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes (MELAS) in one family are reported. Pathological examination of case 1 showed ragged-red fibers, with 7% of the fibers being unstained by cytochrome C oxidase stain, peripheral nerve damage, multiple areas of softening in the cerebrum and midbrain, and spongy changes in the cerebrum, optic nerve and pons. Electron microscopic examination revealed abnormal accumulations of mitochondria in the skeletal muscle, smooth muscle and cardiac muscle. The activity of cytochrome C oxidase in the brain and liver showed a tendency to decrease. In case 2 (maternal aunt of case 1), muscular weakness and peripheral nerve damage improved by treatment with coenzyme Q10. By adding idebenone to the coenzyme Q10 therapy, the EEG and Wechsler's Adult Intelligence Scale (WAIS) improved. Furthermore, in the cerebral spinal fluid (CSF), the protein, lactate, and pyruvate decreased, and the monoamines and monoamine metabolites increased.

Idebenone - the ultimate anti-aging drug. (article)

Alzheimer's disease
Idebenone  in the treatment of Alzheimer's disease
Idebenone efficacy of idebenone in the treatment of Alzheimer's disease
Idebenone Nerve Growth Factor synthesis stimulators in Alzheimer's disease
Idebenone in the treatment of dementia of the Alzheimer type
Idebenone cognitive enhancement therapy for Alzheimer's disease

Friedreich's ataxia.
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia.
Idebenone causes improvement in mitochondrial cardiomyopathy
Idebenone cerebellar improvement in Friedreich's ataxia after 3 months
Idebenone   effect of idebenone on cardiomyopathy in Friedreich's ataxia
Idebenone Friedreich's ataxia and frataxin
Idebenone   Frataxin: Its role in iron metabolism and Friedreich's Ataxia
Idebenone   mitochondrial dysfunction in Friedreich's ataxia
Idebenone   helps heart function in Friedreich's ataxia

MELAS
Idebenone improves cerebral mitochondrial oxidative metabolism in MELAS
Idebenone in young patients with mitochondrial encephalomyopathy (MELAS)
Idebenone therapy in Mitochondrial encephalomyopathy (MELAS)
Idebenone and Mitochondrial encephalomyopathy (MELAS)
Idebenone successful therapy with idebenone in Mitochondrial encephalomyopathy (MELAS)
.
Cardiomyopathy
Idebenone improvement in mitochondrial cardiomyopathy following treatment with idebenone
Idebenone   effect of idebenone on cardiomyopathy in Friedreich's ataxia
.
Leber hereditary optic neuropathy (LHON)
Idebenone does it shorten the time to visual recovery in Leber hereditary optic neuropathy?
Idebenone improvement in a patient with Leber's hereditary optic neuropathy (LHON)

Misc.
Idebenone use in age-related cognitive disorders

Idebenone protects hippocampal neurons against beta amyloid neurotoxicity

Idebenone functions as a potent protective hepatocyte antioxidant

Idebenone neuroprotective effects of idebenone can be attributed to its antioxidant ability

Idebenone inhibition of brain mitochondrial swelling

Idebenone suppression of cold ischemia/reperfusion injury of liver endothelium

Idebenone ability to eliminate a nitroxide radical in the rat

Idebenone interaction with respiratory complexes of heart mitochondria

Idebenone effect on serotonin release and seroton receptors in rats

Idebenone protects hepatic microsomes against damage in organ preservation solutions

Idebenone protects against toxicity induced by low density lipoprotein

Idebenone protects against disorders due to cerebral hypoxia or ischemia

Idebenone protects against toxicity induced by low density lipoprotein

Idebenone attenuates neuronal degeneration induced by excitotoxins

Idebenone augments long-term potentiation in hippocampal slices in the guinea pig

Idebenoneeffects on lipid peroxidation in erythrocytes of stroke-prone, hypertensive rats

Idebenone brain distribution and its effect on local cerebral glucose utilization in rats

Idebenone effects on acetylcholine levels in the brains of rats with cerebral ischemia

Idebenone protective effect of idebenone against hypoxia in mice

Idebenone improves learning and memory impairment induced in rats

Idebenone effects of idebenone on neurological deficits in stroke-prone rats

Idebenone effects on memory induced impairment in rats

Idebenone effects of idebenone on cerebral blood flow in rats with cerebral ischemia

Idebenone inhibition of brain mitochondrial swelling by idebenone

Idebenone inhibition of platelet aggregation by idebenone

Idebenone effects of idebenone on monoamine metabolites of patients with dementia

Idebenone effects of idebenone on metabolism of monoamines and cyclic AMP formation

Idebenone effects on impairment of radial maze learning in cerebral embolized rats

Idebenone effect of idebenone on lipid peroxidation in rat brain homogenate

Idebenone effects on muricidal behavior in rats with raphe lesions

Idebenone inhibitory effect of idebenone on vascular lesions in hypertensive rats

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