Plasma amino acid levels in patients with amyotrophic lateral sclerosis.

Evidence for a generalized defect in glutamate in patients with amyotrophic lateral sclerosis (ALS), associated with widespread alterations in the central nervous system level of this excitatory amino acid. We measured fasting plasma amino acid in 10 ALS patients and 10 controls matched for age and sex. ALS patients had statistically significant elevations in plasma level of aspartate, glutamate, and glycine. The plasma levels of other amino acids were not significantly different from those found in controls. No correlation between ALS severity or activity and degree of abnormality in amino acids was established.