Fluorometric determination of pyruvate and alpha-ketoglutarate in cerebrospinal fluid and plasma of infants and children. A simple test that screen for metabolic disorders.

Levels of pyruvate and alpha-ketoglutarate in the cerebrospinal fluid (CSF) of 26 children, aged 4 months to 5 1/2 years, with febrile seizures and of 19 children, aged 4 months to 14 years, with the diagnosis of epilepsy were not different from values seen in 119 "normal" children 8 days to 14 years of age. The CSF samples from 24 adults, 24 to 81 years of age, suspected of having a herniated disk were also examined. In the pediatric age group, the data showed a highly significant downward trend of CSF and plasma alpha-ketoglutarate values with age; pyruvate values did not change. A correlation of the values of the two keto acids in the blood and CSF of 42 other children without apparent neurologic disease was also made. Findings in a child with thiamine deficiency suggest that CSF alpha-ketoglutarate may be a more sensitive indicator of deficiency than plasma alpha-ketoglutarate or pyruvate. Measurements of these keto acids in plasma and CSF may be diagnostically useful in a variety of metabolic disorders. Findings in 155 children from birth (20 minutes) to 17 years of age without neurologic disease are submitted as a standard of reference.