Experience with Phosphoryl Transferase Inhibition in Subacute Necrotizing Encephalomyelopathy

Abstract

A diagnostic test for urinary inhibition of thiamine pyrophosphate-adenosine triphosphate phosphoryl transferase uncovered five definite and three probable cases of subacute necrotizing encephalomyelopathy in a period of one year, suggesting that the disease is not as rare as originally believed. In addition, the distribution of thiamine phosphates in a sample of brain from another patient who died of the disease was examined. As in a previous study, thiamine triphosphate was not present in the brain, in contrast to four control samples. This result substantiates our contention that patients with subacute necrotizing encephalomyelopathy elaborate a factor found in blood and urine that inhibits the synthesis of thiamine triphosphate in brain.