Leukodystrophies

Description

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.

Exclusions from above levels

05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth and the puerperium
19 – Certain conditions originating in the perinatal period

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22 – Injury, poisoning or certain other consequences of external causes

Child codes

8A44.0 – Pelizaeus-Merzbacher disease
8A44.1 – Adrenoleukodystrophy
8A44.2 – Alexander disease
8A44.3 – Certain specified leukodystrophies
8A44.4 – Krabbe disease
8A44.Z – Leukodystrophies, unspecified

Foundation children elsewhere

5C56.02 – Metachromatic leukodystrophy

Sibling codes

8A40 – Multiple sclerosis
8A41 – Isolated demyelinating syndromes of the central nervous system
8A42 – Acute disseminated encephalomyelitis
8A43 – Neuromyelitis optica
8A45 – Secondary white matter disorders
8A46 – Central demyelination of corpus callosum
8A4Y – Other specified multiple sclerosis or other white matter disorders
8A4Z – Multiple sclerosis or other white matter disorders, unspecified

Foundation entity

Leukodystrophies – http://id.who.int/icd/entity/468040251

Part of: 08: Diseases of the nervous system