Adolescent X-linked adrenoleukodystrophy

This code identifies the adolescent form of X-linked adrenoleukodystrophy (X-ALD), a genetic disorder affecting the white matter of the brain and the adrenal glands. It is characterized by demyelination and adrenal insufficiency, with onset typically occurring between ages 10 and 21.

Use this code for patients diagnosed with X-linked adrenoleukodystrophy where the clinical presentation and age of onset align with the adolescent subtype. Documentation should clearly specify the X-linked form and indicate an adolescent presentation, often supported by genetic testing and neurological imaging findings.

• Differentiate from cerebral (childhood) and adult-onset forms of X-ALD based on age of onset and clinical presentation.
• Ensure documentation specifies "X-linked" to avoid confusion with other leukodystrophies.
• Consider sequencing additional codes for associated manifestations, such as adrenal insufficiency (e.g., E27.40, E27.49) or neurological deficits.