Adrenoleukodystrophy (also known as Bronze Schilder Disease, Melanodermic Leukodystrophy): An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively i

NIH MeSH · D000326Diseases

Adrenoleukodystrophy

Also known asBronze Schilder Disease · Melanodermic Leukodystrophy · Leukodystrophies, Melanodermic · Leukodystrophy, Melanodermic · Siemerling-Creutzfeldt Disease · Siemerling Creutzfeldt Disease · Schilder-Addison Complex · Schilder Addison Complex · X-Linked Adrenoleukodystrophy · Adrenoleukodystrophy, X-Linked · X Linked Adrenoleukodystrophy · ALD (Adrenoleukodystrophy)

Definition

An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).

MeSH classification

  • C10.228.140.163.100.084
  • C10.228.140.163.100.362.250
  • C10.228.140.695.625.250
  • C10.314.400.250
  • C10.597.606.360.455.124
  • C16.320.322.500.124
  • C16.320.400.525.124
  • C16.320.565.189.084
  • C16.320.565.189.362.250
  • C16.320.565.663.100
  • C18.452.132.100.084
  • C18.452.132.100.362.250
  • C18.452.648.189.084
  • C18.452.648.189.362.250
  • C18.452.648.663.100
  • C19.053.500.270

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Related concepts

Alexander Disease

Rare leukoencephalopathy with infantile-onset accumulation of Rosenthal fibers in the subpial, periventricular, and subependymal zones of th…

Common questions about Adrenoleukodystrophy

What does Adrenoleukodystrophy mean in medicine?
An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS). This definition is taken from the National Library of Medicine's MeSH (Medical Subject Headings) 2026 release, which is the standard vocabulary used to index PubMed and most medical literature.
Is Adrenoleukodystrophy known by any other names?
Yes. Adrenoleukodystrophy is also referred to as Bronze Schilder Disease, Melanodermic Leukodystrophy, Leukodystrophies, Melanodermic, Leukodystrophy, Melanodermic in different clinical, research and patient-facing contexts. The MeSH descriptor groups all of these synonyms under a single canonical concept so research and records stay consistent.
Where does Adrenoleukodystrophy sit in the medical classification?
Adrenoleukodystrophy falls under the broader medical category "Diseases" in the MeSH hierarchy (tree numbers: C10.228.140.163.100.084, C10.228.140.163.100.362.250, C10.228.140.695.625.250). Browsing the related concepts on this page takes you to neighbouring topics in the same branch of medicine.
Where can I get a plain-language explanation of Adrenoleukodystrophy?
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Source: NIH MeSH 2026 (D000326) — National Library of Medicine, public domain. View official MeSH record ↗