2022 ICD-10-CM Code A81.0

Creutzfeldt-Jakob disease

Version 2021

Not Valid for Submission

ICD-10:A81.0
Short Description:Creutzfeldt-Jakob disease
Long Description:Creutzfeldt-Jakob disease

Code Classification

  • Certain infectious and parasitic diseases (A00–B99)
    • Viral and prion infections of the central nervous system (A80-A89)
      • Atypical virus infections of central nervous system (A81)

A81.0 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of creutzfeldt-jakob disease. The code is not specific and is NOT valid for the year 2022 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

Specific Coding for Creutzfeldt-Jakob disease

Non-specific codes like A81.0 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10 codes with a higher level of specificity when coding for creutzfeldt-jakob disease:

  • BILLABLE CODE - Use A81.00 for Creutzfeldt-Jakob disease, unspecified
  • BILLABLE CODE - Use A81.01 for Variant Creutzfeldt-Jakob disease
  • BILLABLE CODE - Use A81.09 for Other Creutzfeldt-Jakob disease

Clinical Information

Information for Patients


Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disorder. Symptoms usually start around age 60. Memory problems, behavior changes, vision problems, and poor muscle coordination progress quickly to dementia, coma, and death. Most patients die within a year.

The three main categories of CJD are

Cattle can get a disease related to CJD called bovine spongiform encephalopathy (BSE) or "mad cow disease." There is concern that people can get a variant of CJD from eating beef from an infected animal, but there is no direct proof to support this.

NIH: National Institute of Neurological Disorders and Stroke


[Learn More in MedlinePlus]

Prion disease

Prion disease represents a group of conditions that affect the nervous system in humans and animals. In people, these conditions impair brain function, causing changes in memory, personality, and behavior; a decline in intellectual function (dementia); and abnormal movements, particularly difficulty with coordinating movements (ataxia). The signs and symptoms of prion disease typically begin in adulthood and worsen with time, leading to death within a few months to several years.


[Learn More in MedlinePlus]

Code History

  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)