5C50.70 Glycine encephalopathy

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01

Isolated nonketotic hyperglycinemia is an inborn disorder of glycine metabolism whose onset is generally neonatal with coma, severe hypotonia, myoclonic seizures, and microcephaly, usually progressing to severe intellectual deficit and tetrapyramidal syndrome.

synonyms

• Glycine encephalopathy
• Glycine cleavage deficiency
• NKH - [non-ketotic hyperglycinaemia]
• Non-ketotic hyperglycinaemia
• nonketotic hyperglycinaemia
• Neonatal glycine encephalopathy
• neonatal NKH - [non-ketotic hyperglycinaemia]
• neonatal non-ketotic hyperglycinaemia
• neonatal nonketotic hyperglycinaemia
• Infantile glycine encephalopathy
• infantile NKH - [non-ketotic hyperglycinaemia]
• infantile non-ketotic hyperglycinaemia
• infantile nonketotic hyperglycinaemia
• Atypical glycine encephalopathy

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