What are the universal precautions for suspected Creutzfeldt-Jakob disease (CJD)?

Updated: May 20, 2019
  • Author: Jeffrey S Nine, MD; Chief Editor: Kim A Collins, MD, FCAP  more...
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Answer

Autopsy of a patient with suspected Creutzfeldt-Jakob disease (CJD) requires unique, special procedures. [13] CJD is a rare, progressive dementia caused by a transmissible agent that is resistant to 10% formalin, 70% alcohol, phenolic compounds, boiling, and ultraviolet radiation; it is inactivated by 5% sodium hypochlorite, 2 normal (2N) sodium hydroxide, 90% formic acid, 0.03% permanganate, and autoclaving at 134°C (273.2ºF) for 20 minutes or longer.

In cases in which CJD may be present, the autopsy should be limited to the brain and lymphoid tissues. In exceptional cases in which the importance of clinical questions about other organs necessitates that those organs be examined before it is safe to do so, additional tissues may be harvested. Pathologists should consider taking these special precautions in all known cases of CJD, as well as in cases in which there is rapidly progressive dementia, dementia with seizures (especially myoclonic seizures), or dementia associated with cerebellar or lower motor neuron signs.

During the autopsy, all tissues and fluids, including running water, should be confined to the autopsy tables. A plastic bag should be placed over the mechanical saw while it is being used to incise the skull and any other bones. At the conclusion of the autopsy, the area of the incision and any other areas of contaminated skin surfaces should be sponged with 5% sodium hypochlorite; the sodium hypochlorite solution should be left on the skin for 10 minutes before being washed off.

After the autopsy, any liquid on the autopsy tables should be disinfected with an equal volume of 5% sodium hypochlorite or 2N sodium hydroxide. All instruments should be autoclaved for at least 30 minutes or soaked in 5% sodium hydrochloride or 2N sodium hydroxide for 15 minutes. For steel instruments, 2N sodium hydroxide is preferable to 5% sodium hypochlorite. All gowns, gloves, plastic aprons, and other disposable supplies should be incinerated or autoclaved before disposal. The funeral home should be notified of the high-risk nature of the case.

Scout blocks of the midfrontal cortex, the globus pallidus, and the cerebellum should be removed from the fresh brain and placed in 10% formalin for fixation; the tissues should remain in 10% formalin for a period of 2 to 7 days. The remaining brain may be fixed by immersion in 10% formalin. After fixation, the scout blocks should be decontaminated for 1 hour in 95% formic acid before a final fixation for 2 days in 10% formalin; they should then be embedded in paraffin. This fixation procedure essentially inactivates the agent, and the blocks may then be handled in a routine fashion.

If the scout blocks reveal pathology consistent with CJD, the brain, which has been stored in 10% formalin, may be burned and the container decontaminated, as described above. If CJD is not found in the scout blocks, the remaining brain tissue may be processed in a routine fashion for evaluation of possible Alzheimer disease or other dementing disorders.


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