ICD-10-CM 2027
ICD-10 code for familial creutzfeldt-jakob disease or syndrome
From the official ICD-10-CM alphabetic index entry “Creutzfeldt-Jakob disease or syndrome (with dementia) › familial”. Page updated September 29, 2026.
About coding familial creutzfeldt-jakob disease or syndrome
The ICD-10-CM code for familial creutzfeldt-jakob disease or syndrome is A81.09 (Other Creutzfeldt-Jakob disease).
Within A81.0, choose A81.09 (Other Creutzfeldt-Jakob disease) only when documentation doesn't support a more specific option: unspecified (A81.00) and variant Creutzfeldt-Jakob disease (A81.01).
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.
Common questions about familial creutzfeldt-jakob disease or syndrome ICD-10 codes
What is the ICD-10 code for familial creutzfeldt-jakob disease or syndrome?
- A81.09 — Other Creutzfeldt-Jakob disease.
Is A81.09 billable?
- Yes. A81.09 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can A81.09 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict A81.09 as a principal diagnosis.
Is A81.09 a CC or MCC?
- A81.09 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.