2027 ICD-10-CM Diagnosis Code A81.01Variant Creutzfeldt-Jakob disease

ICD-10-CM Codes›A00–B99›A80-A89›A81

ICD-10-CM A81.01
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

A81.01 is a billable ICD-10-CM diagnosis code for variant Creutzfeldt-Jakob disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 56 through 57. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 159 closely related codes. Coders also document this condition as dementia due to prion disease. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified CNS infection and poliomyelitis and Viral infection.

For Medicare Advantage risk adjustment, A81.01 maps to CMS-HCC Category 127 (Dementia, Mild or Unspecified) under the V28 model, adding a risk factor of about 0.341 for a community, non-dual, aged beneficiary in payment year 2027.

Code Identity

ICD-10-CM Code
A81.01
Billable Status
Yes — Valid for Submission
Code Describes
Variant Creutzfeldt-Jakob disease
Short Description
Variant Creutzfeldt-Jakob disease
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 CodeA81.01Variant Creutzfeldt-Jakob disease

Medicare Risk Adjustment (HCC)Billing

A81.01 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.

CMS-HCC V28 Category (Payment Model)
HCC 127— Dementia, Mild or Unspecified
Payment HCC · PY 2027 one of 65 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+0.341
community, non-dual, aged · ranges 0.000–0.438 across segments
Hierarchy
Superseded by HCC 125 and HCC 126
a more severe related category takes the payment when both are reported
Prior Model (CMS-HCC V24)
HCC 52
V24 retired V28 pays 100% of MA risk scores since PY 2026
Other CMS Models
ESRD (V21): HCC 52 · ESRD (V24): HCC 52
ESRD V21 weights: 0.045 dialysis, 0.000–0.328 functioning graft · ESRD V24 weights: 0.042 dialysis, 0.000–0.399 functioning graft
Part D (RxHCC)
RxHCC 112 — Dementia, Except Alzheimer's Disease
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Dementia due to prion disease
  • Dementia due to variant Creutzfeldt-Jakob disease
  • Variant Creutzfeldt-Jakob disease

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to A81.01: its own notes plus those printed at A81, block A80-A89, and Chapter 1. A note printed at a category, block or chapter applies to every code under it.

Includes

Further defines, or gives examples of, the content of the category.

From A81 Atypical virus infections of central nervous system applies to 10 codes
  • diseases of the central nervous system caused by prions
From Chapter 1 (A00-B99) Certain infectious and parasitic diseases applies to 1,068 codes
  • diseases generally recognized as communicable or transmissible

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • vCJD

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From Block A80-A89 Viral and prion infections of the central nervous system applies to 48 codes
  • postpolio syndrome (G14)
  • sequelae of poliomyelitis (B91)
  • sequelae of viral encephalitis (B94.1)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 1 (A00-B99) Certain infectious and parasitic diseases applies to 1,068 codes
  • carrier or suspected carrier of infectious disease (Z22.-)
  • certain localized infections - see body system-related chapters
  • 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

Add a second code, after this one, to fully describe the condition.

From A81 Atypical virus infections of central nervous system applies to 10 codes
From Chapter 1 (A00-B99) Certain infectious and parasitic diseases applies to 1,068 codes
  • code to identify resistance to antimicrobial drugs (Z16.-)

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name A81.01, its category, or a range that includes it.

Code First 2

These codes say to code first a condition named in the note, and A81.01 is one of the conditions named.

  • F02 Dementia in other diseases classified elsewhere
    Creutzfeldt-Jakob disease (A81.0-) names A81.0-, which includes this code
  • F02 Dementia in other diseases classified elsewhere
    Jakob-Creutzfeldt disease (A81.0-) names A81.0-, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

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

Clinical InformationClinical

  • Variant Creutzfeldt-Jakob Disease

    a form of creutzfeldt-jakob disease that is most commonly contracted after consuming meat from an animal suffering from bovine spongiform encephalopathy.

  • 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;339(27))

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 A81.01 to ICD-9-CMHistory

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

ICD-9-CM
046.11 Varnt Creutzfeldt-Jakob
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About A81.01Overview

What is the ICD-10 code for variant Creutzfeldt-Jakob disease?

The ICD-10-CM code for variant Creutzfeldt-Jakob disease is A81.01 (sometimes written as A8101). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is A81.01 (Creutzfeldt-Jakob disease) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report variant Creutzfeldt-Jakob disease on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does A81.01 group to?

When variant Creutzfeldt-Jakob disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 57, 56, with relative weights from 1.2835 to 2.2649 depending on complications. Higher weights mean higher Medicare reimbursement.

Is A81.01 a CC or MCC?

CMS lists A81.01 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 159 closely related codes in its exclusion list.

Can A81.01 be reported with G14?

Not as a rule. The Excludes1 note at block A80-A89, which applies to A81.01, lists "postpolio syndrome (G14)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

What HCC is A81.01?

A81.01 (variant Creutzfeldt-Jakob disease) maps to CMS-HCC Category 127 (Dementia, Mild or Unspecified), commonly written as HCC 127, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 52 under the retired V24 model. It also maps in the ESRD (V21) and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 112.

Does A81.01 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, A81.01 adds a risk adjustment factor of about 0.341 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.000 to 0.438 depending on the payment segment). A more severe related category (HCC 125 and HCC 126) supersedes it when both are reported. See the full factor table on the HCC 127 category page.