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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.
Glycine acts as a chemical messenger that transmits signals in the brain. Glycine encephalopathy is caused by the shortage of an enzyme that normally breaks down glycine in the body, thus allowing excess glycine to build up in tissues and organs, particularly the brain.asic Information. 1  Genetics Home Reference (GHR) contains information on Glycine encephalopathy. 2  The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders.