2022 ICD-10-CM Diagnosis Code A81.00

Creutzfeldt-Jakob disease, unspecified

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
  • A81.00 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2022 edition of ICD-10-CM A81.00 became effective on October 1, 2021.
  • 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
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to A81.00:
  • A00-B99
    2022 ICD-10-CM Range A00-B99

    Certain infectious and parasitic diseases

    • diseases generally recognized as communicable or transmissible
    Type 1 Excludes
    • certain localized infections - see body system-related chapters
    Type 2 Excludes
    • carrier or suspected carrier of infectious disease (Z22.-)
    • infectious and parasitic diseases complicating pregnancy, childbirth and the puerperium (O98.-)
    • infectious and parasitic diseases specific to the perinatal period (P35-P39)
    • influenza and other acute respiratory infections (J00-J22)
    Use Additional
    • code to identify resistance to antimicrobial drugs (Z16.-)
    Certain infectious and parasitic diseases
  • A80-A89
    2022 ICD-10-CM Range A80-A89

    Viral and prion infections of the central nervous system

    Type 1 Excludes
    • postpolio syndrome (G14)
    • sequelae of poliomyelitis (B91)
    • sequelae of viral encephalitis (B94.1)
    Viral and prion infections of the central nervous system
  • A81
    ICD-10-CM Diagnosis Code A81

    Atypical virus infections of central nervous system

      2016 2017 2018 2019 2020 2021 2022 Non-Billable/Non-Specific Code
    • diseases of the central nervous system caused by prions
    Use Additional
    • code to identify:
    • dementia with behavioral disturbance (F02.81)
    • dementia without behavioral disturbance (F02.80)
    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 v39.0):
  • 056 Degenerative nervous system disorders with mcc
  • 057 Degenerative nervous system disorders without mcc

Convert A81.00 to ICD-9-CM

Code 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

Diagnosis Index entries containing back-references to A81.00:
  • Creutzfeldt-Jakob disease or syndrome (with dementia) A81.00
  • Dementia (degenerative (primary)) (old age) (persisting) F03.90
    ICD-10-CM Diagnosis Code F03.90

    Unspecified dementia without behavioral disturbance

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code Adult Dx (15-124 years)
    Applicable To
    • Dementia NOS
    • in (due to)
      • Creutzfeldt-Jakob disease A81.00 - see also Creutzfeldt-Jakob disease or syndrome (with dementia)

ICD-10-CM Codes Adjacent To A81.00
A80.0 Acute paralytic poliomyelitis, vaccine-associated
A80.1 Acute paralytic poliomyelitis, wild virus, imported
A80.2 Acute paralytic poliomyelitis, wild virus, indigenous
A80.3 Acute paralytic poliomyelitis, other and unspecified
A80.30 Acute paralytic poliomyelitis, unspecified
A80.39 Other acute paralytic poliomyelitis
A80.4 Acute nonparalytic poliomyelitis
A80.9 Acute poliomyelitis, unspecified
A81 Atypical virus infections of central nervous system
A81.0 Creutzfeldt-Jakob disease
A81.00 …… unspecified
A81.01 Variant Creutzfeldt-Jakob disease
A81.09 Other Creutzfeldt-Jakob disease
A81.1 Subacute sclerosing panencephalitis
A81.2 Progressive multifocal leukoencephalopathy
A81.8 Other atypical virus infections of central nervous system
A81.81 Kuru
A81.82 Gerstmann-Sträussler-Scheinker syndrome
A81.83 Fatal familial insomnia
A81.89 Other atypical virus infections of central nervous system
A81.9 Atypical virus infection of central nervous system, unspecified

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.