2024 ICD-10-CM Diagnosis Code A81.00

Creutzfeldt-Jakob disease, unspecified

  • A81.00 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2024 edition of ICD-10-CM A81.00 became effective on October 1, 2023.
  • This is the American ICD-10-CM version of A81.00 – other international versions of ICD-10 A81.00 may differ.

Applicable To

  • Jakob-Creutzfeldt disease, unspecified

The following code(s) above A81.00 contain annotation back-references

 that may be applicable to A81.00:

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

Approximate Synonyms

  • Dementia due to creutzfeldt jakob disease
  • Jakob creutzfeldt disease

Clinical Information

  • 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;339(27))
  • A rare transmittable degenerative disorder of the brain caused by prions. Morphologically it is characterized by spongiform degeneration of the cerebral and cerebellar cortex. Signs and symptoms include sleep disturbances, personality changes, aphasia, ataxia, muscle atrophy and weakness, visual loss, and myoclonus. It usually leads to death within a year from the onset of the disease.
  • A rare, incurable and often deadly brain 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.

ICD-10-CM A81.00 is grouped within Diagnostic Related Group(s) (MS-DRG v41.0):

  • 056 Degenerative nervous system disorders with mcc
  • 057 Degenerative nervous system disorders without mcc

Convert A81.00 to ICD-9-CMCode History

  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
  • 2024 (effective 10/1/2023): No change

Diagnosis Index entries containing back-references to A81.00:

  • Creutzfeldt-Jakob disease or syndrome (with dementia) A81.00
  • Dementia (degenerative (primary)) (old age) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety) F03.90
    • in (due to)
      • Creutzfeldt-Jakob disease A81.00 – see also Creutzfeldt-Jakob disease or syndrome (with dementia)