Sphingolipidoses are lysosomal diseases which result from defective metabolism of sphingolipids.

Sphingolipids are important structural components of lipid membranes and are involved in cellular signalling.

The sphingolipidoses:

• show variable severity
• generally cause progressive peripheral and central nervous system disease

Variants of Gaucher and Niemann-Pick disease which do not affect the nervous system are termed non-neuronopathic.