U.S. flag
        An official website of the United States government
          Here’s how you know

      Official websites use .gov
                A
                .gov
                website belongs to an official government organization in the United States.
          Secure .gov websites use HTTPS
                A lock
                  (
                  
                  )
                   or
                  https://
                   means you’ve safely connected to the .gov website. Share sensitive information only on official, secure websites.Search
                Menu
    
                      Información en Español
                      Search NINDSSearch NINDS
            

            Main navigation
  

        
              Health Information
                                     Toggle Health Information Dropdown
                                Disorders
                Clinical Trials
                Patient & Caregiver Education
                Public Education
                Support Resources
                Funding
                                     Toggle Funding Dropdown
                                Find Funding Opportunities
                About Funding
                Preparing Your Application
                After You Submit
                Determining Your Funding Likelihood
                Manage Your Award
                NINDS Small Business Program
                Training & Career Development
                Current Research
                                     Toggle Current Research Dropdown
                                Coronavirus and NINDS
                Focus on Disorders
                Focus on Tools & Topics
                Research Funded by NINDS
                Research at NINDS
                Scientific Resources
                Trans-Agency Activities
                News & Events
                                     Toggle News & Events Dropdown
                                Events
                News
                Director's Messages
                About NINDS
                                     Toggle About NINDS Dropdown
                                Who We Are
                What We Do
                Job Opportunities 
 Home
                                Health Information
SHARE: Creutzfeldt-Jakob Disease
On this page

            What is Creutzfeldt-Jakob disease?

Creutzfeldt-Jakob disease (CJD) is a rare, rapidly worsening brain disorder that causes unique changes in brain tissue and affects muscle coordination thinking, and memory. There are about 350 cases per year in the U.S.

The two main symptoms of CJD are:

Severe mental deterioration and dementia
	Involuntary (unwanted) muscle jerks (myoclonus) or muscle movementEarly symptoms of the disease may include:

Lack of coordination
	Problems with walking and balance
	Impaired thinking, memory, and judgment
	Behavior changes
	Depression, mood swings, and anxiety
	Confusion
	Insomnia or changes in sleeping patterns
	Unusual sensations
	Changes in visionAs CJD progresses, symptoms may include:

Weakness of the arms and legs
	Blindness
	Loss of ability to move or speak
	Pneumonia and other infections
	ComaSome symptoms of CJD can be similar to symptoms of other progressive (continuing to worsen) neurological disorders, such as Alzheimer's and Huntington's disease. However, these symptoms tend to worsen faster in CJD than in Alzheimer's disease or most other types of dementia.

There are three major categories of CJD, including:

Sporadic CJD—The disease develops in someone when the cause is unknown. This accounts for at least 85 percent of cases.
	Hereditary CJD—There may be a known gene mutation that occurs in a family. About 10 to 15 percent of cases of CJD in the United States are hereditary.
	Acquired CJD—Rarely, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures such as surgical grafts of dura mater (a tissue that covers the brain), transplanted corneas, and implantation of inadequately sterilized electrodes in the brain. Doctors call these iatrogenic cases. Fewer than one percent of cases have been acquired CJD.
	A type of CJD called variant CJD (or vCJD) can be acquired by eating meat from cattle affected by a disease similar to CJD called bovine spongiform encephalopathy (BSE, or “mad cow” disease). Variant CJD begins primarily with psychiatric symptoms, affects younger people than other types of CJD, and has a longer than usual duration from onset of symptoms to death. Fortunately, government regulations have basically eliminated this chance of transmission.Who is more likely to get Creutzfeldt-Jakob disease?

CJD usually appears in later life, typically around age 60, and quickly gets worse. About 70 percent of people having CJD die within one year.A person cannot get CJD through the air or by touching something like a doorknob or being close to someone who has CJD. Still, doctors recommend that people with suspected or confirmed CJD or who have a family history of CJD should not donate blood, organs, or tissue.

There is a very small risk that a surgeon or others who handle brain tissue following a brain biopsy or autopsy may become accidentally infected. Special surgical and disinfection procedures can markedly reduce this risk.There is no evidence that caregivers, healthcare workers, and those who prepare bodies for funerals and cremation have an increased risk of prion disease when compared to the general population.

CJD is caused by abnormal forms of proteins called prions. Proteins are long chains of amino acids that fold together into a unique shape or conformation to help a cell function in a particular way. The normal and harmless prion protein is found throughout the body but is most abundant in the nervous system. Its overall role is not fully understood. This abnormal folding or conformation is “transmitted” to normal prion proteins in surrounding nerve cells, causing them to become infective and changed to one that results in the disease. Once they are formed, abnormal prion proteins aggregate, or clump together, which may lead to the nerve cell loss and other brain damage seen in CJD.

CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases. The infective prion causes CJD and related disorders in people and TSEs in animals. Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges when examined under a microscope.

Human prion diseases include:

CJD and variant CJD
	Gerstmann-Straussler-Schenker Disease
	Fatal Familial Insomnia
	KuruAnimal prion diseases include:

Bovine spongiform encephalopathy (also known as “mad cow” disease)
	Mink encephalopathy
	Feline encephalopathy
	Scrapie (which affects sheep and goats)
	Chronic wasting disease (which affects elk and deer)The normal, harmless prion protein is usually designated as PrPC ("C" stands for cellular) and the abnormal, infectious form (which causes the disease) is PrPSc ("Sc" stands for prototypical prion disease–scrapie).

In the acquired form of the disease, the infectious PrPSc comes from the outside the body, for example, through contaminated meat as is seen in variant CJD.
	In the hereditary form, infectious prions can arise when a mutation occurs in the gene for the body's normal prion protein. As the mutated PrPCduplicates itself, it spontaneously changes shape into the infectious form. (Prions themselves do not contain genetic information and do not require genes to reproduce themselves.)  If the prion protein gene is changed in a person's sperm or egg cells, the changed gene can be transmitted to the person's children. Specific changes found in each family affects how often the disease appears and what symptoms are most noticeable. However, not all people with mutations in the prion protein gene develop CJD.
	In the sporadic form, the infectious prions are believed to be made by an error in the cell's machinery that makes proteins and controls their quality. These errors are more likely to occur with aging.How is Creutzfeldt-Jakob disease diagnosed and treated?

**Diagnosing CJD**

There are a few tests a doctor can use to help diagnose CJD:

Electroencephalography (EEG)—This test records the brain's electrical pattern and find a specific type of abnormality in major types of CJD.
	Cerebrospinal fluid-based tests—This testing looks for the 14-3-3 protein (a marker for some prion diseases, such as CJD) to detect the abnormal prion protein in the fluid that surrounds the brain and spinal cord.
	Magnetic resonance imaging (MRI)—This type of brain imaging is accurate in about 90 percent of cases.The only way to confirm a diagnosis of CJD is by brain biopsy or an autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the living person's brain so it can be examined by a neuropathologist. This procedure may be dangerous for the individual and is generally discouraged unless it is needed to rule out a treatable disorder. In an autopsy, the whole brain is examined after death.

**Treating CJD**

Currently there is no cure for CJD, although some drugs are being tested to control it. Today's treatments are aimed at making the person comfortable and easing symptoms. Medications may help relieve pain and muscle jerks. During later stages of the disease, intravenous fluids and machine feeding also may be used.

What are the latest updates on Creutzfeldt-Jakob disease?

Researchers are examining and characterizing the prions associated with CJD and other human and animal prion diseases. A better understanding of these diseases may help scientists discover factors that influence prion infectivity and transmission, and how the disorder damages the brain. Researchers are investigating the cellular mechanisms involved in abnormal prion formation and accumulation, as well as their replication by select cellular subsets in the brain. Other projects are examining how abnormal prions cross the protective blood-brain barrier and spread throughout the central nervous system, and tests that measure the biological activity of prions. Findings may identify new therapeutic targets to treat prion diseases.

Other NIH Institutes, including the National Institute of Allergy and Infectious Diseases, also conduct research on CJD. More information about CJD research supported by NINDS and other NIH Institutes and Centers can be found usingNIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies. RePORTER also includes links to publications and resources from these projects.

For research articles and summaries on CJD, searchPubMed, which contains citations from medical journals and other sites.

How can I or my loved one help improve care for people with Creutzfeldt-Jakob disease?

file-medical

Learn About Clinical Trials

Clinical trials are studies that allow us to learn more about disorders and improve care. They can help connect patients with new and upcoming treatment options.

Consider participating in a clinical trial so clinicians and scientists can learn more about CJD. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visitNIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with CJD atClinicaltrials.gov, a database of federal and privately funded trials and research results.

Scientists are conducting biochemical analyses of brain tissue, blood, spinal fluid, urine, and serum in the hope of determining the nature of the transmissible agent or agents causing CJD. To help with this research, they are seeking biopsy and autopsy tissue, blood, and cerebrospinal fluid from individuals with CJD and related diseases. The following investigators have expressed an interest in receiving such material:

**Dr. Brian Appleby, Director**National Prion Disease Pathology Surveillance CenterCase Western Reserve University
Phone: 216-368-0587

**Dr. Laura Manuelidis**Manuelidis TSE LaboratoryYale University School of Medicine
Phone: 203-785-4442

Where can I find more information about Creutzfeldt-Jakob disease?

Information may be available from the following organizations:

Centers for Disease Control and Prevention (CDC)Phone: 800-311-3435 or 404-639-3311

Creutzfeldt-Jakob Disease (CJD) FoundationPhone: 800-659-1991

MedlinePlus

National Institute of Allergy and Infectious Diseases (NIAID)

National Organization for Rare Disorders (NORD)
Phone: 800-999-6673
book-open

Order publications from the NINDS Catalog

The NINDS Publication Catalog offers printed materials on neurological disorders for patients, health professionals, and the general public. All materials are free of charge, and a downloadable PDF version is also available for most publications. Health Information
  
    

    Disorders
                                                              
                    
                                      Glossary of Neurological Terms
    
            Order PublicationsClinical Trials
                                                              
                    
                                      Clinical Trials in the Spotlight
    
            Find NINDS Clinical TrialsPatient & Caregiver Education
                                                              
                    
                                      Brain Attack Coalition
    
            Brain DonationPublic Education
                                                              
                    
                                      Brain Basics
                                                              
                    
                                      Know Your Brain
    
            Understanding Sleep
    
            Preventing Stroke
    
            The Life and Death of a Neuron
    
            Genes and the BrainKnow Stroke
                                                              
                    
                                      Health Professionals
                                                              
                    
                                      NIH Stroke ScalePatients and Caregivers
    
            Researchers
    
            Shareable Resources
                                                              
                    
                                      Recursos en EspañolMigraine Trainer® App
                                                              
                    
                                      Migraine Trainer® Shareable ResourcesMind Your Risks®
    
            NINDS Brain Educational Resources
        
  
              
    
            Support Resources
                                                              
                    
                                      Patient Organizations
    
            Professional Societies
    
            Government Resources

Did you find the content you were looking for?
  Yes, I did find the content I was looking for



No, I did not find the content I was looking forPlease rate how easy it was to navigate the NINDS website
  Very easy to navigate



Easy to navigate



Neutral



Difficult to navigate



Very difficult to navigateThank you for letting us know! Any other feedback?Submit

This site is protected by reCAPTCHA and the Google Privacy Policyand Terms of Serviceapply.

      Last reviewed on November 28, 2023
        Contact Us
  

        
              Contact NINDS
                Visitor Information
        Related Sites
  

        
              Site Map
                National Institutes of Health
                Department of Health & Human Services
                USA.gov
        Policies
  

        
              Accessibility
                Freedom of Information Act
                Linking Policy
                Privacy Statement
                Vulnerability Disclosure Policy
Follow NINDS On Social Media
          
          National Institutes of Health (NIH)
          9000 Rockville Pike, Bethesda, MD, 20892