ICD-10 Code A81.09 – Other Creutzfeldt-Jakob Disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code A81.09 – Other Creutzfeldt-Jakob disease
What it is
A81.09 identifies Creutzfeldt-Jakob disease that is not classified elsewhere. It refers to a rare, rapidly progressive prion disease affecting the brain.
Clinical signs
Clinical features vary; refer to documentation. Common findings include rapidly worsening dementia, myoclonus, ataxia, and other neurologic decline consistent with prion disease.
When to use this code
Use this code when the provider documents other or unspecified Creutzfeldt-Jakob disease and no more specific subtype is named. It may apply after neurologic evaluation, imaging, or specialist assessment supports the diagnosis. Code only when the record clearly identifies CJD.
Do not use for
Do not use this code for Alzheimer disease, other dementias, or prion disorders that are documented with a different, more specific ICD-10-CM code. Check documentation if the diagnosis is uncertain.
Coding tip
Confirm the exact CJD subtype in the assessment before assigning A81.09.