ICD-11 classes
08 Diseases of the nervous system
Multiple sclerosis or other white matter disorders
8A44 — Leukodystrophies
8A44.4 — Krabbe disease

ICD-11 8A44.4 — Krabbe disease

Krabbe disease, also called globoid cell leukodystrophy, is a sphingolipidosis resulting from galactosylceramidase (or galactocerebrosidase) deficiency, a lysosomal enzyme that catabolizes a major lipid component of myelin. The disease leads to demyelination of the central and peripheral nervous system which is rapidly progressive from the first year of life, but juvenile, adolescent or adult onset forms have also been reported, with a more variable rate of progression.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Sphingolipidosis #4876