ICD-10 Code E75.27 – Pelizaeus-Merzbacher Disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.27 – Pelizaeus-Merzbacher disease
What it is
E75.27 identifies Pelizaeus-Merzbacher disease, a rare inherited disorder affecting myelin formation in the central nervous system. Use it when the record documents this specific leukodystrophy.
Clinical signs
Clinical features vary; refer to documentation. Standard references describe early-onset nystagmus, hypotonia, developmental delay, spasticity, and progressive motor impairment.
When to use this code
Use E75.27 when the clinician documents Pelizaeus-Merzbacher disease by name, or clearly confirms the diagnosis in the assessment. It may also fit genetic or neurologic evaluations that explicitly identify this leukodystrophy. If the note only says leukodystrophy or white matter disease, check documentation.
Do not use for
Do not assign this code for nonspecific developmental delay, cerebral palsy, or other leukodystrophies unless Pelizaeus-Merzbacher disease is documented. Check documentation when the diagnosis is uncertain.
Coding tip
Code the condition only when the provider states the diagnosis clearly; otherwise, query for specificity before selecting E75.27.