5C50.70

ICD-11 MMS 2026-01

Glycine encephalopathy

Description

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.

Index terms

  • Glycine encephalopathy
  • Glycine cleavage deficiency
  • NKH - [non-ketotic hyperglycinaemia]
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  • 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

Exclusions from above levels

  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
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  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C50.71 – Sarcosinaemia
  • 5C50.7Y – Other specified disorders of glycine metabolism
  • 5C50.7Z – Disorders of glycine metabolism, unspecified

Foundation entity

Glycine encephalopathy – http://id.who.int/icd/entity/1491869639

Part of: 5C50.7: Disorders of glycine metabolism › 5C50: Inborn errors of amino acid or other organic acid metabolism › 05: Endocrine, nutritional or metabolic diseases