E71.521

HCC Category Mapping

What This Code Means

A genetic disorder affecting males where nervous system deterioration begins in adolescence, causing progressive problems with movement, behavior, and cognitive function.

Coding Tips

• •Document age of onset in adolescence (typically 10-21 years) to distinguish from childhood form which presents earlier
• •This form generally has slower progression than childhood cerebral form; document specific neurological symptoms and functional decline

Clinical Significance

Adolescent X-linked adrenoleukodystrophy presents in males between ages 10-21 with similar but generally slower-progressing cerebral demyelination compared to the childhood form. Patients develop cognitive decline, behavioral changes, vision and hearing impairment, and progressive motor dysfunction. Like the childhood form, early stem cell transplantation may halt progression if detected before significant neurological damage occurs.

Documentation Requirements

• ✓Document age of symptom onset in adolescence, MRI findings and Loes score, very long chain fatty acid levels, ABCD1 gene mutation, adrenal function, and neurological assessment documenting rate of progression.
• ✓Record family history and carrier status testing of female relatives.

Commonly Confused Codes