ICD-10-CM 2027
ICD-10 code for Creutzfeldt-Jakob disease or syndrome
From the official ICD-10-CM alphabetic index entry “Creutzfeldt-Jakob disease or syndrome (with dementia)”. Page updated September 29, 2026.
About coding Creutzfeldt-Jakob disease or syndrome
The ICD-10-CM code for Creutzfeldt-Jakob disease or syndrome is A81.00 (Creutzfeldt-Jakob disease, unspecified). The official index lists 5 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on A81.00.
Within A81.0, choose A81.00 (unspecified) only when documentation doesn't support a more specific option: variant Creutzfeldt-Jakob disease (A81.01) and other Creutzfeldt-Jakob disease (A81.09).
Use an additional code, if applicable, to identify: dementia with anxiety (F02.84, F02.A4, F02.B4, F02.C4), dementia with behavioral disturbance (F02.81-, F02.A1-, F02.B1-, F02.C1-), dementia with mood disturbance (F02.83, F02.A3, F02.B3, F02.C3), dementia with psychotic disturbance (F02.82, F02.A2, F02.B2, F02.C2), dementia without behavioral disturbance (F02.80, F02.A0, F02.B0, F02.C0) and 1 more.
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
More specific Creutzfeldt-Jakob disease or syndrome codes (official index)
Common questions about Creutzfeldt-Jakob disease or syndrome ICD-10 codes
What is the ICD-10 code for Creutzfeldt-Jakob disease or syndrome?
- A81.00 — Creutzfeldt-Jakob disease, unspecified. More specific codes apply when the documentation supports them: A81.09, A81.09, A81.09, A81.09 and others below.
What is the ICD-10 code for familial creutzfeldt-jakob disease or syndrome?
- A81.09 — Other Creutzfeldt-Jakob disease.
What is the ICD-10 code for iatrogenic creutzfeldt-jakob disease or syndrome?
- A81.09 — Other Creutzfeldt-Jakob disease.
What is the ICD-10 code for sporadic creutzfeldt-jakob disease or syndrome?
- A81.09 — Other Creutzfeldt-Jakob disease.
What is the ICD-10 code for variant creutzfeldt-jakob disease or syndrome?
- A81.01 — Variant Creutzfeldt-Jakob disease.
Is A81.00 billable?
- Yes. A81.00 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can A81.00 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict A81.00 as a principal diagnosis.
Is A81.00 a CC or MCC?
- A81.00 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.