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

ICD-11 8A44 — Leukodystrophies

Group of rare progressive genetic diseases that are caused by mutations in genes that lead to destruction of white matter of the brain by disrupting development of the myelin sheath. More than 50 different leukodystrophies have been identified, including Alexander disease Canavan disease, cerebrotendinous xanthomatosis, metachromatic leukodystrophy, Pelizaeus-Merzbacher disease, and Refsum disease.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 8A44 contains 7 clarifying diagnoses:

1. 8A44.0 — Pelizaeus-Merzbacher disease
   It contains 1 clarifying diagnosis.
2. 8A44.1 — Adrenoleukodystrophy
3. 8A44.2 — Alexander disease
4. 8A44.3 — Certain specified leukodystrophies
   It contains 1 clarifying diagnosis.
5. 8A44.4 — Krabbe disease
6. 5C56.02 — Metachromatic leukodystrophy
7. 8A44.Z — Leukodystrophies, unspecified