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July 1981

Correction of Increased Plasma Pyruvate and Plasma Lactate Levels Using Large Doses of Thiamine in Patients With Kearns-Sayre Syndrome

Author Affiliations

Department of Neurology Roskilde Hospital 4000 Roskilde, Denmark

Arch Neurol. 1981;38(7):469. doi:10.1001/archneur.1981.00510070103027

To the Editor.—  In a previous report, I and my colleague demonstrated that Kearns-Sayre syndrome (progressive external ophthalmoplegia, ophthalmoplegia-plus) is accompanied by deficient pyruvate-lactate metabolism (Archives 1976;33:455-456). This finding has since been confirmed by several authors, and it may have important pathogenetic implications.1 Recently, we repeated the determinations of plasma pyruvate and plasma lactate levels in three of the original four patients. (The oldest patient was unavailable for follow-up.) By this time, all three displayed characteristic chorioretinal atrophy, ptosis, and external ophthalmoplegia, as well as ragged red fibers in muscle biopsy specimens stained with Gomori's trichrome stain and abnormal mitochondria on electron microscopy. Venous plasma pyruvate and plasma lactate measurements were performed under standardized conditions after three hours of fasting and 30 minutes of rest, avoiding venous stasis, and with immediate precipitation of the samples in iced perchloric acid followed by chemical analysis using the method of Lundholm et

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