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8A44.1 Adrenoleukodystrophy

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

code elsewhere

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

synonyms

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

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