The importance of cerebrospinal fluid lactate in the evaluation of congenital lactic acidosis

In 27 of 28 children with congenital lactic acidosis, cerebrospinal fluid l actate was higher than venous blood lactate. The mean +/- SEM difference be tween these variables was 2.4 +/- 0.3 mmol/L (P =.0001). Girls or patients with pyruvate dehydrogenase deficiency had higher cerebrospinal fluid lacta te concentrations than boys or patients with respiratory chain defects or m itochondrial DNA mutations.