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
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