ICD-10-CM Code A81.0

Creutzfeldt-Jakob disease

Version 2020 Non-Billable Code

Not Valid for Submission

A81.0 is a "header" nonspecific and non-billable code code, consider using a code with a higher level of specificity for a diagnosis of creutzfeldt-jakob disease. The code is NOT valid for the year 2020 for the submission of HIPAA-covered transactions.

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

Consider the following ICD-10 codes with a higher level of specificity:

  • A81.00 - ... unspecified
  • A81.01 - Variant Creutzfeldt-Jakob disease
  • A81.09 - Other Creutzfeldt-Jakob disease

Clinical Information

  • PRION PROTEINS-. membrane glycosylphosphatidylinositol anchored glycoproteins that may aggregate into rod like structures. the prion protein prnp gene is characterized by five tandem repeat sequences that encode a highly unstable protein region of five octapeptide repeats. mutations in the repeat region and elsewhere in this gene are associated with creutzfeldt jakob disease; fatal familial insomnia; gerstmann straussler disease; huntington disease like 1 and kuru.
  • CREUTZFELDT JAKOB SYNDROME-. a rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. affected individuals may present with sleep disturbances personality changes ataxia; aphasia visual loss weakness muscle atrophy myoclonus progressive dementia and death within one year of disease onset. a familial form exhibiting autosomal dominant inheritance and a new variant cjd potentially associated with encephalopathy bovine spongiform have been described. pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of prions. from n engl j med 1998 dec 31;33927

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)

Code History

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

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

  • Sporadic CJD, which occurs for no known reason
  • Hereditary CJD, which runs in families
  • Acquired CJD, which occurs from contact with infected tissue, usually during a medical procedure

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


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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.
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