ICD-10 Code A81.00 – Creutzfeldt-Jakob disease, unspecified (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code A81.00 – Creutzfeldt-Jakob disease, unspecified
What it is
A81.00 identifies Creutzfeldt-Jakob disease when the record does not specify a subtype. It is a rare, fatal prion disease that causes rapidly progressive brain degeneration.
Clinical signs
Typical features include rapidly worsening dementia, confusion, ataxia, myoclonus, and other neurologic decline. Clinical features vary; refer to documentation.
When to use this code
Use this code when the provider documents Creutzfeldt-Jakob disease but does not name variant, type, or etiology details. It also fits confirmed cases where the chart only supports an unspecified diagnosis. If a more specific CJD form is documented, code that instead.
Do not use for
Do not use it for other prion diseases, dementia without CJD, or suspected cases without confirmation. Check documentation if the record names a specific subtype.
Coding tip
Look for the exact wording in the assessment and final diagnosis; unspecified codes are appropriate only when no subtype is documented.