E71.522

HCC Category Mapping

What This Code Means

Adrenomyeloneuropathy is a rare inherited disorder that affects the nervous system and adrenal glands, causing progressive weakness and numbness in the legs, along with hormonal imbalances. It results from the body's inability to properly break down certain fatty acids, leading to their accumulation in nerve and adrenal tissue.

Coding Tips

• •This is a specific subtype of X-linked adrenoleukodystrophy (ALD); verify documentation specifies adrenomyeloneuropathy rather than other ALD variants to ensure accurate code selection
• •Look for associated conditions such as adrenal insufficiency, progressive neurological symptoms, or elevated very long chain fatty acids (VLCFA) in lab results, as these often accompany this diagnosis

Clinical Significance

Adrenomyeloneuropathy is the most common phenotype of X-linked adrenoleukodystrophy, typically presenting in adult males (20s-30s) with progressive spastic paraparesis, peripheral neuropathy, and adrenal insufficiency, predominantly affecting the spinal cord and peripheral nerves rather than cerebral white matter. Approximately 20% of patients eventually develop cerebral involvement. Female carriers may develop a milder myelopathy in later decades.

Documentation Requirements

• ✓Document age of onset, pattern of neurological involvement (predominantly spinal cord and peripheral nerves), adrenal function status, very long chain fatty acid levels, ABCD1 gene mutation, MRI findings (spinal cord vs cerebral involvement), and functional status assessment.
• ✓Record any adrenal hormone replacement therapy.

Commonly Confused Codes