ICD-10 Code A81.0 – Creutzfeldt-Jakob Disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code A81.0 – Creutzfeldt-Jakob disease
What it is
Creutzfeldt-Jakob disease is a rare, fatal prion disease that affects the brain and causes rapidly progressive neurologic decline. Code A81.0 identifies this specific diagnosis in ICD-10-CM.
Clinical signs
Typical findings include rapidly worsening dementia, confusion, myoclonus, ataxia, and other neurologic deficits. Clinical features vary; refer to documentation.
When to use this code
Use A81.0 when the provider documents Creutzfeldt-Jakob disease, including suspected or confirmed cases that are being managed as this diagnosis. It is appropriate for classical presentations and documented variants when the record clearly names CJD. If the note only describes unexplained rapidly progressive neurologic decline, check documentation before assigning this code.
Do not use for
Do not use this code for other dementia disorders, encephalopathy, or nonspecific neurologic symptoms without documentation of CJD. Check documentation if the record mentions prion disease but does not specify Creutzfeldt-Jakob disease.
Coding tip
Assign A81.0 only when the provider explicitly documents Creutzfeldt-Jakob disease, and query if the diagnosis is implied but not stated.