what is Glycine encephalopathy
Glycine encephalopathy is sometimes referred to as nonketotic hyperglycinemia (NKH), as a reference to the biochemical findings seen in patients with the disorder, and to distinguish it from the disorders that cause ketotic hyperglycinemia (seen in propionic acidemia and several other inherited metabolic disorders).lycine encephalopathy has an estimated incidence of 1 in 60,000, making it the second most common disorder of amino acid metabolism, after phenylketonuria. It is caused by a defect in the glycine cleavage system (GCS), which is made up of four protein subunits.