ICD-10 Code A81.01 – Variant Creutzfeldt-Jakob Disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code A81.01 – Variant Creutzfeldt-Jakob disease
What it is
Variant Creutzfeldt-Jakob disease is a rare, fatal prion disease that affects the brain and causes rapidly progressive neurological decline. Use this code when documentation specifically identifies the variant form of CJD.
Clinical signs
Typical features include rapidly worsening cognitive decline, psychiatric or behavioral changes, and progressive neurologic impairment. Clinical features vary; refer to documentation.
When to use this code
Use A81.01 when the provider documents variant Creutzfeldt-Jakob disease, vCJD, or confirms the diagnosis in the record. Apply it for encounters focused on evaluation, monitoring, or care related to this specific prion disorder. Check documentation if the note only says suspected prion disease or CJD without the variant type.
Do not use for
Do not use this code for classic Creutzfeldt-Jakob disease or other dementing illnesses. If the record does not clearly identify the variant form, check documentation.
Coding tip
Code the diagnosis only when the variant form is explicitly stated in the provider record.