Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder that can be experimentally transmitted from one animal to another, as well as from human patients to other humans and animals. It affects about one person in every one million people each year worldwide. The low random incidence of CJD indicates that person-to-person transmission probably does not occur through normal contact. Spouses and other household members of people with sporadic CJD appear to be at no higher risk of contracting the disorder than the general population. CJD affects both men and women, and most often appears in people aged 50 to 75. The majority of cases occur on a sporadic basis via unknown mechanisms. In a few rare families (7-10% of all cases), the disease is hereditary. In a smaller number of cases (<1% of all cases) it is transmitted iatrogenically through contaminated surgical instruments (e.g. implanted EEG depth electrodes) and human material (e.g. human growth hormone, dura mater or corneal transplants).
Symptoms of CJD may be similar to those of other progressive dementias such as Alzheimer’s disease, but it tends to progress much more rapidly, usually culminating in death over the course of a few months to a year. In the early stages of CJD, individuals may have failing memory, changes in behavior, lack of coordination, or visual disturbances. As the disease progresses, mental deterioration becomes pronounced; involuntary movements, especially muscle jerks, appear; and the patient may become blind, develop weakness in the arms or legs, and ultimately become comatose. There is currently no effective treatment for CJD, but clinical trials of potential therapies may begin soon. Information about clinical trials may be available at http://clinicaltrials.gov.
NINDS scientists have developed a test for CJD that detects a protein in cerebrospinal fluid that can aid in the diagnosis of the disease. See a press release about this test. However, the only way to absolutely confirm a diagnosis of CJD is by brain biopsy or autopsy.
Scientists found evidence linking an outbreak of a variant form of CJD (vCJD), largely in Britain, to mad cow disease (bovine spongiform encephalopathy or BSE), a deadly brain disease- similar to CJD-that affects cattle. While there is still no definitive evidence for how this transmission took place, it is believed that transmission occurred through the ingestion of beef contaminated by the BSE agent.
To protect themselves, health-care professionals should employ universal precautions when handling blood and spinal fluid samples from patients with CJD. When performing medical/surgical procedures and post-mortem examinations, the most important safety rule is to avoid self-induced injury from instruments used in the course of removing and processing tissues for pathological examination. In particular, avoid contact between contaminated material and skin with cuts or abrasions.
Normal sterilization procedures such as boiling or irradiating materials do not prevent transmission of CJD. Whenever possible, contaminated instruments and other materials should be discarded as medical pathological waste or destroyed by incineration. When this is not possible, special disinfection methods may be employed. Re-used instruments and materials should be kept moist until they can be appropriately decontaminated and cleaned. Detailed guidelines for infection control during patient care, for disinfection and disposal of CJD-contaminated material, and for performing an autopsy and embalming are available in the document, "WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies." The URL for this document is http://www.who.int/csr/resources/publications/bse/WHO_CDS_CSR_APH_2000_3/en/ . To receive a printed copy of this report, contact the World Health Organization at the following address:
Communicable Disease Surveillance and
World Health Organization
CH-1211 Geneva 27
Fax: +41 22 791 4198
Email: email@example.com, Attention: Publications
The NINDS is one of several NIH Institutes that support research on CJD. This research is aimed at finding ways to prevent, treat, and cure these disorders. Scientists are examining and characterizing the prions associated with various diseases, and are using animal models to identify risk factors that influence susceptibility to the disease and that govern when in life the disease appears.
Where can I get more information?
For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and Information Network (BRAIN) at:
P.O. Box 5801
Bethesda, MD 20824