2026 ICD-10-CM Diagnosis Code A81.00Creutzfeldt-Jakob disease, unspecified

ICD-10-CM Codes›A00–B99›A80-A89›A81

ICD-10-CM A81.00
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

A81.00 is a billable ICD-10-CM diagnosis code for Creutzfeldt-Jakob disease, unspecified. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as cerebral degeneration due to Creutzfeldt-Jakob disease. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified CNS infection and poliomyelitis and Viral infection.

Code Identity

ICD-10-CM Code
A81.00
Billable Status
Yes — Valid for Submission
Code Describes
Creutzfeldt-Jakob disease, unspecified
Short Description
Creutzfeldt-Jakob disease, unspecified
Same as the full description in the CMS dataset.
Parent Code
A81.0
Creutzfeldt-Jakob disease

Code Classification

ChapterA00–B99Certain infectious and parasitic diseases
SectionA80-A89Viral and prion infections of the central nervous system
CategoryA81Atypical virus infections of central nervous system
This CodeA81.00Creutzfeldt-Jakob disease, unspecified

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Cerebral degeneration due to Creutzfeldt-Jakob disease
  • Creutzfeldt Jakob disease following graft of dura
  • Creutzfeldt-Jakob disease
  • Dementia due to Creutzfeldt Jakob disease
  • Dementia due to prion disease
  • Graft infection
  • Iatrogenic Jakob-Creutzfeldt disease

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Jakob-Creutzfeldt disease, unspecified

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Creutzfeldt-Jakob disease or syndrome(with dementia)
    • Dementia(degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety)
      • in (due to)
        • Creutzfeldt-Jakob disease

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR NVS003
Other specified CNS infection and poliomyelitis
Default principal diagnosis: inpatient Yes · outpatient Yes
CCSR INF008
Viral infection
Default principal diagnosis: inpatient No · outpatient No

Patient EducationClinical

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.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert A81.00 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
046.19 Creutzfldt-Jakob NEC/NOS
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About A81.00Overview

Is A81.00 (Creutzfeldt-Jakob disease) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report Creutzfeldt-Jakob disease, unspecified on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of A81.00?

Under the General Equivalence Mappings, Creutzfeldt-Jakob disease, unspecified converts to ICD-9-CM 046.19 (Creutzfldt-Jakob NEC/NOS). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.