DESCRIPTION: Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder caused by an unknown organism that can be experimentally transmitted from one animal to another, as well as from human patients to other animals. CJD affects both men and women, and most often appears in people ages 50 to 75.

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 lapse into a coma.

Symptoms of CJD may be similar to those of other progressive neurological disorders such as Alzheimer's disease. NINDS scientists have recently developed a diagnostic test for CJD that will allow doctors to distinguish CJD from other neurological disorders.

The low random incidence of CJD indicates that person-to-person transmission probably does not occur. Spouses of patients and other household members appear to be at no higher risk of contracting the disorder than the general population.

There is no effective treatment for CJD. The disease usually leads to death within a year of onset.

British scientist recently found evidence possibly linking CJD to mad cow disease (bovine spongiform encephalopathy), a deadly brain disease -- similar to CJD -- found in cattle.

PRECAUTIONS: In a few cases, CJD has occurred among physicians, dentists, and other health-care workers, possibly after having been exposed to the agent in the course of their work. Health-care professionals should take precautions when handling blood and spinal fluid samples taken from patients with CJD.

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. Also, avoid contact between contaminated material and skin with cuts or abrasions.

For disinfection of CJD-contaminated material: Raise the autoclave temperature to 132-134 degrees C, use undiluted household bleach, or sodium hydroxide at 1N concentration. Wear disposable gloves, although simple contact of unbroken skin with infective material is not dangerous. To prevent subsequent contamination of the environment, however, swab the contaminated area of skin with undiluted bleach or 1N sodium hydroxide for 5 minutes, and then thoroughly wash the skin with water.

During an autopsy, pathologists and their assistants should wear gowns and gloves. Anyone using sharp instruments should use "chain mail" gloves to prevent accidental cuts to the hands. Masks and eye protectors may be worn if the skull is opened with a power saw, although there is no evidence of disease transmission by aerosol or nonpenetrating contact with conjunctival or nasal mucosal surfaces.

The autopsy (limited or complex) should be conducted as an in situ procedure. Place the body on a plastic/rubber sheet that covers the autopsy table. Arrange the sheet to confine all tissues and fluids. Place all organs removed from the body directly in containers filled with phenolsaturated formalin or 2N sodium hydroxide. Clearly mark the containers as infectious.

At the conclusion of the autopsy, swab any part of the body surface that required incision with undiluted household bleach and allow it to remain undisturbed for at least 10 minutes before wrapping the body for delivery to the mortician. The pathologists should tell the mortician that the internal body tissues and fluids may be infectious and that the body surface may be contaminated. Autoclave and discard the gowns, gloves, and sheet.

RESEARCH: The NINDS conducts and supports research on neurodegenerative brain disorders such as CJD. This research is aimed at finding ways to prevent, treat, and cure these disorders.

These articles, available from a medical library, are sources of in-depth information on CJD:

Hsich, G, Kenney, K, Gibbs, C, Lee, K, and Harrington, M. "The 14-3-3 Brain Protein in Cerebrospinal Fluid as a Marker for Transmissible Spongiform Encephalopathies." New England Journal of Medicine, 335:13;924-965 (September 26, 1996).

Pendlebury, W. "Central Nervous System Diseases Caused by Unconventional Transmissible Agents and Chronic Viral Infections." In Neurology in Clinical Practice, vol. II, Butterworth-Heinemann, Boston, pp. 1112-1116 (1991).

Brown, P. "Guidelines for High Risk Autopsy Cases: Special Precautions for Creutzfeldt-Jakob Disease." In Autopsy Performance and Reporting, College of American Pathologists, Northfield, IL, pp. 67-75 (1990).

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