A syndrome consisting of hyperuricema, mental retardation, choreoathetosis and self-
destructive biting has been described in two brothers aged five and eight years. The uric
acid pools in these patients were found to be similar in size to those reported for gouty adult
subjects; their rates of turnover were greater than any previously reported. The daily
excretion of uric acid in the urine was considerably higher than those of control patients and
approximated total values found in gouty adult “hyperexcretors.” The formation of uric acid …