Stroke, with mitochondrial encephalopathy

The clinical symptoms of mitochondrial diseases are highly varied and include seizures, vomiting, deafness, dementia, stroke-like episodes, and short stature. Although there are many types of mitochondrial disorders, four of the most common types are as follows Kearns-Sayre syndrome, Leber s hereditary optic atrophy, MELAS (mitochondrial encephalopathy, lactic acidosis and stroke-like episodes) and MERRE (myoclonic epilepsy with ragged red fibres). 

Al. Abe, K., Fujimura, H., Nishikawa, Y., Yorifuji, S., Mezaki, T., Hirono, N., Nishitani, N., and Kameyama, M., Marked reduction in CSF lactate and pyruvate levels after CoQ therapy in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Acta Neurol. Scand. 83, 356-359 (1991). 

K3. King, M. P., Koga, Y., Davidson, M., and Schon, E. A., Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNALe (UUR> mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. Mol. Cell. Biol. 12, 480-490 (1992). 

MELAS syndrome (mitochondrial encephalopathy lactic acidosis with stroke-like episodes)... 

Cll. Chomyn, A., Enriquez, J. A., Micol, V., Fernandez-Silva, P., and Attardi, G., The Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode syndrome-associated human mitochondrial 1 k A1 11 k i mutation causes aminoacylation deficiency and concomitant reduced association of mRNA with ribosomes. J. Biol. Chem. 275, 19198—19209 (2000). 

Mitochondrial cytopathy may present with stroke-like episodes often complicated by epilepsy and encephalopathy, a particular example of which is MELAS. Scanning with CT may show hypodensities, particularly in the occipital regions, and calcification of the... 

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