Neonatal adrenoleukodystrophy

This code identifies a rare, inherited peroxisomal disorder affecting the adrenal glands and nervous system, presenting in the neonatal period. It is characterized by severe neurological deterioration, adrenal insufficiency, and often leads to early death. The condition is distinct from the more common X-linked form.

Assign this code when documentation explicitly states a diagnosis of "neonatal adrenoleukodystrophy." This diagnosis is typically made based on clinical presentation, biochemical testing (e.g., very long-chain fatty acids), and genetic analysis. It applies to infants diagnosed with this specific, severe form of adrenoleukodystrophy.

• Ensure the documentation specifies "neonatal" adrenoleukodystrophy to differentiate it from other forms.
• Do not code this alongside E71.42- (X-linked adrenoleukodystrophy) due to the Excludes1 note.
• Look for supporting documentation of elevated very long-chain fatty acids (VLCFAs) and neurological symptoms consistent with a severe, early-onset presentation.