2025 ICD-10-CM Diagnosis Code A81.00

Creutzfeldt-Jakob disease, unspecified

ICD-10-CM Code:: A81.00
ICD-10 Code for:: Creutzfeldt-Jakob disease, unspecified
Is Billable?: Yes - Valid for Submission
Chronic Condition Indicator: ^[1]: Chronic
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A81.00 is a billable diagnosis code used to specify a medical diagnosis of creutzfeldt-jakob disease, unspecified. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2024 through September 30, 2025.

Unspecified diagnosis codes like A81.00 are acceptable when clinical information is unknown or not available about a particular condition. Although a more specific code is preferable, unspecified codes should be used when such codes most accurately reflect what is known about a patient's condition. Specific diagnosis codes should not be used if not supported by the patient's medical record.

Table of Contents

Code Information
Approximate Synonyms
Clinical Classification
Tabular List of Diseases and Injuries
Index to Diseases and Injuries References
Convert to ICD-9 Code
Patient Education
Other Codes Used Similar Conditions
Code History

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

Approximate Synonyms

The following list of clinical terms are approximate synonyms, alternative descriptions, or common phrases that might be used by patients, healthcare providers, or medical coders to describe the same condition. These synonyms and related diagnosis terms are often used when searching for an ICD-10 code, especially when the exact medical terminology is unclear. Whether you're looking for lay terms, similar diagnosis names, or common language alternatives, this list can help guide you to the correct ICD-10 classification.

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

Clinical Classification

Clinical Classifications group individual ICD-10-CM diagnosis codes into broader, clinically meaningful categories. These categories help simplify complex data by organizing related conditions under common clinical themes.

They are especially useful for data analysis, reporting, and clinical decision-making. Even when diagnosis codes differ, similar conditions can be grouped together based on their clinical relevance. Each category is assigned a unique CCSR code that represents a specific clinical concept, often tied to a body system or medical specialty.

Other specified CNS infection and poliomyelitis

CCSR Code: NVS003

Inpatient Default: Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.

Outpatient Default: Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.

Viral infection

CCSR Code: INF008

Inpatient Default: N - Not default inpatient assignment for principal diagnosis or first-listed diagnosis.

Outpatient Default: N - Not default outpatient assignment for principal diagnosis or first-listed diagnosis.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.

Inclusion Terms

Jakob-Creutzfeldt disease, unspecified

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

- Creutzfeldt-Jakob disease or syndrome (with dementia) - A81.00

- Dementia (degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety) - F03.90
- - in (due to)
  - - Creutzfeldt-Jakob disease - See Also: Creutzfeldt-Jakob disease or syndrome (with dementia); - A81.00

Convert A81.00 to ICD-9-CM

Below are the ICD-9 codes that most closely match this ICD-10 code, based on the General Equivalence Mappings (GEMs). This ICD-10 to ICD-9 crosswalk tool is helpful for coders who need to reference legacy diagnosis codes for audits, historical claims, or approximate code comparisons.

Creutzfldt-Jakob NEC/NOS

ICD-9-CM: 046.19

Approximate Flag - The approximate mapping means this ICD-10 code does not have an exact ICD-9 equivalent. The matched code is the closest available option, but it may not fully capture the original diagnosis or clinical intent.

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Patient Education

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

[Learn More in MedlinePlus]

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.