ICD-10 Code E79.81 – Aicardi-Goutières Syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E79.81 – Aicardi-Goutières syndrome
What it is
Aicardi-Goutières syndrome is a rare inherited neuroinflammatory disorder that affects the brain and immune system. It is coded when documentation identifies this specific syndrome.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include early-onset neurologic impairment, developmental delay, irritability, and signs of chronic brain inflammation.
When to use this code
Use E79.81 when the provider documents Aicardi-Goutières syndrome as the confirmed diagnosis. Apply it for encounters focused on the syndrome itself, including evaluation of its neurologic or developmental manifestations.
Do not assign it for nonspecific encephalopathy, developmental delay, or genetic testing alone unless the syndrome is explicitly documented. Check documentation when the record only suggests a possible diagnosis.
Do not use for
Do not use this code for general leukodystrophy, congenital infection, or other causes of neurologic decline without a documented Aicardi-Goutières diagnosis. Avoid using it when the condition is only suspected.
Coding tip
Code the syndrome only when the chart clearly names Aicardi-Goutières syndrome; otherwise, query for clarification.