8A44.4 Krabbe disease

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

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.

synonyms

• Krabbe disease
• Diffuse globoid body sclerosis
• Galactocerebrosidase deficiency
• Galactosylceramide beta-galactosidase deficiency
• Globoid cell leukodystrophy
• Krabbe brain sclerosis
• Krabbe's leukodystrophy
• Infantile Krabbe disease
• Classic Krabbe disease
• Congenital Krabbes syndrome
• Early-onset Krabbe disease
• Late-infantile or juvenile Krabbe disease
• Adult Krabbe disease

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