ICD-10 Code E71.521 – Adolescent X-linked adrenoleukodystrophy (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Adolescent X-linked adrenoleukodystrophy is E71.521.
2026 ICD-10-CM Diagnosis Code E71.521 – Adolescent X-linked adrenoleukodystrophy

What it is

E71.521 identifies adolescent X-linked adrenoleukodystrophy, a genetic disorder that affects the nervous system and adrenal function. Use it when the record documents this specific adolescent form.

Clinical signs

Clinical features vary; refer to documentation. Commonly documented findings include progressive neurologic decline, behavioral or school changes, gait problems, and evidence of adrenal involvement in a male patient with X-linked disease.

When to use this code

Use this code when the clinician documents adolescent X-linked adrenoleukodystrophy as the confirmed diagnosis. It may appear in neurology, genetics, or metabolic disorder records, including follow-up visits after prior confirmation. If the note only says “adrenoleukodystrophy,” check documentation for the age-specific subtype.

Do not use for

Do not use this code for other leukodystrophies, nonspecific peroxisomal disorders, or adrenal insufficiency without documented X-linked adrenoleukodystrophy. Check documentation if the age group or subtype is unclear.

Coding tip

Code the documented subtype exactly; age and X-linked form matter here.

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