Disease Directory Adrenoleukodystrophy
Metabolic

Adrenoleukodystrophy

Also known as: ALD, X-ALD, X-linked adrenoleukodystrophy, ABCD1 deficiency, adrenomyeloneuropathy (AMN)

Prevalence

1-5 per 100,000 (Orphanet)

Onset

Childhood, Adolescent, Adult

Type

X-linked genetic

Gene

ABCD1

About Adrenoleukodystrophy

X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene encoding a peroxisomal membrane protein involved in very long-chain fatty acid (VLCFA) transport. Accumulation of VLCFAs, particularly in the adrenal glands and white matter of the brain and spinal cord, causes a spectrum of phenotypes from adrenocortical insufficiency to severe cerebral demyelination to progressive myeloneuropathy (AMN). Elivaldogene autotemcel (Skysona), a gene therapy, is approved for early cerebral ALD in boys.

Common Clinical Features

Adrenal insufficiency Progressive cerebral demyelination Behavioral and cognitive changes Spastic paraparesis Visual and hearing loss Peripheral neuropathy Seizures

Clinical Trial Eligibility Tips

What to know before applying to Adrenoleukodystrophy trials.

MRI Loes score is the critical cerebral ALD severity marker — gene therapy (Skysona) is approved for Loes score 0.5-9

Plasma VLCFA levels are the diagnostic biomarker required for trial documentation

Distinguish cerebral ALD from adrenomyeloneuropathy (AMN) — trials are phenotype-specific

Adrenal function testing (ACTH stimulation test) is required — adrenal insufficiency affects eligibility and requires corticosteroid management during trials

Patient Resources

Patient Organization

ALD Connect

Visit website ↗

Natural History Registry

ALD Connect Patient Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:43)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Adrenoleukodystrophy trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Adrenoleukodystrophy. Updated daily.

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