ICD-10 Code E75.25 – Metachromatic leukodystrophy (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.25 – Metachromatic leukodystrophy
What it is
Metachromatic leukodystrophy is a rare inherited disorder that affects the brain and nervous system. It causes progressive loss of myelin, the protective covering around nerves.
Clinical signs
Clinical features vary; refer to documentation. Common findings include progressive motor decline, weakness, speech or swallowing difficulty, and cognitive or behavioral changes.
When to use this code
Use this code when the provider documents metachromatic leukodystrophy by name, including suspected or confirmed disease in the record. It may appear in pediatric or adult neurology notes, genetic evaluations, or problem lists. Check documentation if the record only describes leukodystrophy without specifying the type.
Do not use for
Do not use this code for other leukodystrophies or nonspecific white-matter disease unless metachromatic leukodystrophy is documented. Check documentation when the diagnosis is only being considered.
Coding tip
Code only when the condition is explicitly named; otherwise, query for the specific leukodystrophy type.