Creutzfeldt-Jakob disease is a rare progressive dementia caused by a transmissible agent which is resistant to 10% formalin, 70% alcohol, boiling and ultraviolet radiation, but is inactivated by a 5% sodium hypochloride, 2 normal sodium hydroxide, 0.03% permanganate, phenolic compounds and autoclaving. In cases of potential Creutzfeldt-Jakob disease the autopsy should be limited to the brain and lymphoid tissues only. In exceptional cases where the importance of clinical questions about the other organs outweighs the time required to safely examine them, additional tissues can be harvested. 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 Stryker saw while it is operation to incise the skull and any other bones. At the conclusion of the autopsy, the area of the incision and any other contaminated skin surfaces should be sponged with 5% sodium hypochloride, which is left on for 10 minutes before being washed off. Following the autopsy, any liquid on the autopsy table should be disinfected with an equal volume of 5% of sodium hypochloride or two normal sodium hydroxide. All instruments should be autoclaved for at least 30 minutes or soaked in 5% sodium hypochloride or two normal sodium hydroxide for 15 minutes. For steel instruments, 2 normal sodium hydroxide is preferable to a 5% sodium hypochloride. All gowns, gloves, plastic aprons and other disposables should be incinerated or autoclaved before disposal. The funeral home should be notified of the infectious nature of the case.
A one inch cube of frontal cortex and cerebellum should be frozen at -80oC and the remaining brain fixed in 10% buffered formalin.
Additional sections of the mid frontal cortex globus pallidus and cerebellum should be removed from the fresh brain and placed in 10% formalin for fixation from 2-7 days. The remaining brain can be immersion fixed in 10% formalin. After fixation, the scout blocks are fixed for one hour in 95% formic acid before a final fixation for two days in 10% formalin followed by embedding in paraffin. This fixation procedure essentially inactivates the agent and the blocks can be handled in a routine fashion. If the scout blocks reveal pathology compatible with Creutzfeldt-Jakob disease the brain stored in 10% formalin can be burned and container decontaminated as described above. If CJD is not found in the pilot blocks, the remaining tissue can be processed in a routine fashion for evaluation of possible Alzheimer's or other related dementing disorders.
Pathologists should consider taking these special precautions in all known CJD cases or cases where there is rapidly progressive dementia, a dementia with seizures especially myoclonic seizures, or dementia associated with cerebellar or lower motor neuron signs.