ICD-10 Code E71.52 – X-linked adrenoleukodystrophy (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.52 – X-linked adrenoleukodystrophy
What it is
E71.52 identifies X-linked adrenoleukodystrophy, an inherited metabolic disorder that affects the nervous system and adrenal glands. It is caused by impaired breakdown of very long-chain fatty acids.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include progressive neurologic decline, behavioral or cognitive changes, and adrenal insufficiency. Some patients present in childhood, while others have later-onset disease.
When to use this code
Use E71.52 when the provider documents X-linked adrenoleukodystrophy, adrenomyeloneuropathy due to the same disorder, or a confirmed history of this diagnosis. Code it from the documented diagnosis, not from a nonspecific leukodystrophy mention alone. Check documentation when the chart is unclear.
Do not use for
Do not use this code for other leukodystrophies, unspecified peroxisomal disorders, or adrenal insufficiency without documented X-linked adrenoleukodystrophy. If the diagnosis is uncertain, check documentation.
Coding tip
Confirm that the record states X-linked adrenoleukodystrophy explicitly, because similar neurologic or adrenal findings alone are not enough.