NEONATAL ENCEPHALOPATHY WITH A PUNGENT BODY ODOR

A neonate had transient unexplained bleeding into the gut, severe ence phalopathy, and an abnormal pungent body odour. An inherited metabolic defect was excluded. The malodour was due to methanethiol and hydroge n sulphide, identified in urine. These sulphur compounds may have cont ributed to encephalopathy. Colonic bacteria were the probable source.