8A44.1

ICD-11 MMS 2026-01

Adrenoleukodystrophy

Description

X-linked genetic disorder associated with accumulation of very-long-chain fatty acids in the brain and adrenal cortex due to a mutation in the ABCD1 gene causing defects in peroxisomal oxidation. Neurological symptoms can present in childhood or adulthood with almost all patients having concurrent adrenal insufficiency.

Index terms

  • Adrenoleukodystrophy
  • ALD - [adrenoleukodystrophy]
  • Addison-Schilder
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  • Adult-onset autosomal dominant leukodystrophy
  • ADLD - [adult-onset autosomal dominant leukodystrophy]
  • Autosomal dominant Pelizaeus-Merzbacher disease
  • Multiple sclerosis-like disorder

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

Sibling codes

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

Foundation entity

Adrenoleukodystrophy – http://id.who.int/icd/entity/1085655586

Part of: 8A44: Leukodystrophies › 08: Diseases of the nervous system