Leukodystrophies

Definition

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.

6 subordinate codes

• 8A44.0Pelizaeus-Merzbacher disease
• 8A44.1Adrenoleukodystrophy
• 8A44.2Alexander disease
• 8A44.3Certain specified leukodystrophies
• 8A44.4Krabbe disease
• 4B4ZDiseases of the immune system, unspecified

Nearby in Multiple sclerosis or other white matter disorders

• 8A40Multiple sclerosis
• 8A41Isolated demyelinating syndromes of the central nervous system
• 8A42Acute disseminated encephalomyelitis
• 8A43Neuromyelitis optica
• 8A45Secondary white matter disorders
• 8A46Central demyelination of corpus callosum
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified