ICD-11 classes
08 Diseases of the nervous system
Multiple sclerosis or other white matter disorders
8A44 — Leukodystrophies
8A44.1 — Adrenoleukodystrophy

ICD-11 8A44.1 — Adrenoleukodystrophy

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

The diagnosis is included in 3 other classes.

  • Zellweger syndrome (5C57.0)
  • Neonatal adrenoleukodystrophy (5A74.Y)
  • X-linked adrenoleukodystrophy (5C57.1)

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