Diseases

Adrenoleukodystrophy

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).

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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 Record

Classification

Broader headings

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MeSH Record

See Also

MeSH Record

Synonyms

18 entry terms
  • ALD (Adrenoleukodystrophy)
  • Addison Disease and Cerebral Sclerosis
  • Bronze Schilder Disease
  • Melanodermic Leukodystrophy
  • Schilder-Addison Complex
  • Siemerling-Creutzfeldt Disease
  • X-ALD
  • X-ALD (X-Linked Adrenoleukodystrophy)
  • X-Linked Adrenoleukodystrophy
  • Adrenoleukodystrophy, X-Linked
  • Leukodystrophies, Melanodermic
  • Leukodystrophy, Melanodermic
  • Schilder Addison Complex
  • Siemerling Creutzfeldt Disease
  • X ALD
  • X ALD (X Linked Adrenoleukodystrophy)
  • X Linked Adrenoleukodystrophy
  • Adrenomyeloneuropathy

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

Indexing Annotation

do not confuse with ADRENOLEUKODYSTROPHY, NEONATAL see PEROXISOMAL DISORDERS

MeSH Record

History Note

1991(1983)

MeSH Record

Previous Indexing

  • Adrenal Gland Hypofunction (1968-1982)
  • Cerebral Sclerosis, Diffuse (1966-1982)
  • Demyelinating Diseases (1966-1982)

MeSH Hierarchy

Tree Numbers

  1. C10w.228w.140w.163.100.084w
  2. C10w.228w.140w.163.100.362w.250w
  3. C10w.228w.140w.695w.625w.250w
  4. C10w.314w.400w.250w
  5. C10w.597.606.360w.455w.124w
  6. C16w.320w.322.500w.124w
  7. C16w.320w.400.525w.124w
  8. C16w.320w.565w.189.084w
  9. C16w.320w.565w.189.362w.250w
  10. C16w.320w.565w.663w.100w
  11. C18.452w.132.100.084w
  12. C18.452w.132.100.362w.250w
  13. C18.452w.648w.189.084w
  14. C18.452w.648w.189.362w.250w
  15. C18.452w.648w.663w.100w
  16. C19w.053w.500w.270w

MeSH Record

NLM Classification

QU 265.5.P4

AMA Style

References

  1. National Library of Medicine. Adrenoleukodystrophy. Medical Subject Headings (MeSH). 2026. Unique ID D000326. http://id.nlm.nih.gov/mesh/2026/D000326
  2. Adrenoleukodystrophy. In: Wikipedia. https://en.wikipedia.org/wiki/Adrenoleukodystrophy
  3. Adrenoleukodystrophy. In: Wikidata. https://www.wikidata.org/wiki/Q366964