- Concept UI
- M0020298
- Scope Note
- A group of inherited metabolic disorders characterized by the intralysosomal accumulation of SPHINGOLIPIDS primarily in the CENTRAL NERVOUS SYSTEM and to a variable degree in the visceral organs. They are classified by the enzyme defect in the degradation pathway and the substrate accumulation (or storage). Clinical features vary in subtypes but neurodegeneration is a common sign.
- Terms
-
Sphingolipidoses
Preferred Term
Term UI
T038571
Date01/01/1999
LexicalTag
NON
ThesaurusID
NLM (1974)
-
Sphingolipidosis
Term UI
T038570
Date09/12/1990
LexicalTag
NON
ThesaurusID
NLM (1992)
-
Sphingolipid Storage Diseases
Term UI
T370372
Date11/03/1999
LexicalTag
NON
ThesaurusID
NLM (2000)