St. Luke's Hospital
Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Find a Physician Payment Options Locations & Directions
Follow us on: facebook twitter Mobile Email Page Email Page Print Page Print Page Increase Font Size Decrease Font Size Font Size
America's 50 Best Hospitals
Meet the Doctor
Spirit of Women
Community Health Needs Assessment
Home > Health Information

Multimedia Encyclopedia

    Print-Friendly
    Bookmarks

    Creutzfeldt-Jakob disease

    Transmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt disease

    Creutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease of mental function and movement.

    Causes

    CJD is thought to be caused by a protein called a prion. A prion causes normal proteins to fold abnormally. This affects the other proteins' ability to function.

    There are several types of CJD. The disorder is very rare, occurring in about 1 out of 1 million people. It usually first appears between ages 20 and 70. Symptoms usually start in the late 50s.

    CJD can be grouped into two types: classic or new variant disease.

    The classic types of CJD are:

    • Sporadic CJD makes up most cases. It occurs for no known reason. The average age at which it starts is 65.
    • Familial CJD occurs when a person inherits the abnormal prion from a parent (inherited CJD is rare)

    Classic CJD is not related to mad cow disease (bovine spongiform encephalitis).

    However, variant CJD (vCJD) is a form of the disease that is related to mad cow disease. The infection that causes the disease in cows is believed to be the same one that causes vCJD in humans.

    Variant CJD accounts for less than 1% of cases, and it tends to affect younger people. However, fewer than 200 people worldwide have had this disease.

    Variant CJD can be caused by exposure to contaminated products. Other vCJD cases have occurred when people were given corneal transplants, other tissues, or blood transfusions from infected donors. It may also have been caused by contaminated electrodes used in brain surgery (before instruments started to be properly disinfected).

    CJD may be related to several other diseases caused by prions, including:

    • Chronic wasting disease (found in deer)
    • Kuru (seen in New Guinea women who ate the brains of dead relatives as part of a funeral ritual)
    • Scrapie (found in sheep)
    • Other rare human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia

    Symptoms

    • Dementia that rapidly worsens over a few weeks or months
    • Blurred vision (sometimes)
    • Changes in gait (walking)
    • Confusion, disorientation
    • Hallucinations
    • Lack of coordination (for example, stumbling and falling)
    • Muscle stiffness
    • Muscle twitching
    • Myoclonic jerks or seizures
    • Nervous, jumpy feelings
    • Personality changes
    • Sleepiness
    • Speech impairment

    CJD is rarely confused with other types of dementia (such as Alzheimer's disease) because in CJD, the symptoms get worse much more quickly.

    Exams and Tests

    Early in the disease, a nervous system and mental examination will show memory problems and changes in otherintellectual functions. Later in the disease, a motor system examination may show:

    • Abnormal reflexes or increased normal reflex responses
    • Increase in muscle tone
    • Muscle twitching and spasms
    • Strong startle response
    • Weakness and loss of muscle tissue (muscle wasting)

    There is loss of coordination and changes in the cerebellum, the area of the brain that controls coordination (cerebellar ataxia). An eye examination shows areas of blindness that the person may not notice.

    Tests used to diagnose this condition may include:

    • Blood tests to rule out other forms of dementia and to look for markers that sometimes occur with the disease
    • CT scan of the brain
    • Electroencephalogram (EEG)
    • MRI of the brain
    • Spinal tap to test for a protein called 14-3-3

    The disease can only be confirmed with a brain biopsy or autopsy. It is very rare anymore for brain biopsy to be done to look for this disease.

    Treatment

    There is no known cure for this condition. Interleukins and other medications may help slow the disease. The person may need care early in the disease. Medications may be needed to control aggressive behaviors.

    Providing a safe environment, controlling aggressive or agitated behavior, and meeting the person's needs may require monitoring and assistance in the home or in a care facility. Family counseling may help the family cope with the changes needed for home care.

    Visiting nurses or aides, volunteer services, homemakers, adult protective services, and other community resources may help care for the person with CJD.

    People with this condition may need help controlling unacceptable or dangerous behaviors. This involves rewarding positive behaviors and ignoring negative behaviors (when it is safe). They may also need help getting oriented to their surroundings.

    Getting legal help with advance directives, powers of attorney, and other legal actions early in the disorder can make it easier to make ethical decisions about the CJD patient's care.

    Outlook (Prognosis)

    The outcome of CJD is very poor. Within 6 months or less after symptoms begin, the person will be unable to care for himself or herself.

    The disorder is fatal in a short time, usually within 8 months. However, a few people survive for as long as 1 or 2 years. The cause of death is usually infection, heart failure, or respiratory failure.

    • Infection
    • Loss of ability to interact with others
    • Loss of ability to function or care for oneself
    • Death

    When to Contact a Medical Professional

    CJD is not a medical emergency. However, getting diagnosed and treated early may make the symptoms easier to control, give patients time to make advance directives, and give families extra time to come to terms with the condition.

    Prevention

    Medical equipment should be treated to remove the proteins that may cause the disease. People who have a history of CJD should not donate a cornea or tissue.

    Most countries now have strict guidelines for managing infected cows to avoid transmitting CJD to humans.

    References

    Bosque PJ. Prion diseases. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 424.

    DeKosky ST, Kaufer DI, Hamilton RL, Wolk DA, Lopez OL. The dementias. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Butterworth-Heinemann Elsevier; 2008:chap 70.

    BACK TO TOP

    • Creutzfeldt-Jakob diseas...

      illustration

    • Central nervous system

      illustration

      • Creutzfeldt-Jakob diseas...

        illustration

      • Central nervous system

        illustration

      A Closer Look

        Talking to your MD

          Self Care

            Tests for Creutzfeldt-Jakob disease

              Review Date: 9/25/2013

              Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.

              The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
              adam.com

              A.D.A.M. content is best viewed in IE9 or above, Fire Fox and chrome browser.


              Back  |  Top
              About Us
              Contact Us
              History
              Mission
              Locations & Directions
              Quality Reports
              Annual Reports
              Honors & Awards
              Community Health Needs
              Assessment

              Newsroom
              Services
              Brain & Spine
              Cancer
              Heart
              Maternity
              Orthopedics
              Pulmonary
              Sleep Medicine
              Urgent Care
              Women's Services
              All Services
              Patients & Visitors
              Locations & Directions
              Find a Physician
              Tour St. Luke's
              Patient & Visitor Information
              Contact Us
              Payment Options
              Financial Assistance
              Send a Card
              Mammogram Appointments
              Health Tools
              My Personal Health
              mystlukes
              Spirit of Women
              Health Information & Tools
              Clinical Trials
              Health Risk Assessments
              Employer Programs -
              Passport to Wellness

              Classes & Events
              Classes & Events
              Spirit of Women
              Donate & Volunteer
              Giving Opportunities
              Volunteer
              Physicians & Employees
              For Physicians
              Remote Access
              Medical Residency Information
              Pharmacy Residency Information
              Physician CPOE Training
              Careers
              Careers
              St. Luke's Hospital - 232 South Woods Mill Road - Chesterfield, MO 63017 Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
              Copyright © St. Luke's Hospital Website Terms and Conditions  |  Privacy Policy  |  Patient Notice of Privacy Policies PDF Sitemap St. Luke's Mobile