Medical Information

When you suspect a possible rapidly progressive dementia (RPD), there are certain tests you should order to help rule out or confirm a diagnosis. Many tests are needed for a complete work up, but a brain MRI including FLAIR (fluid attenuated inversion recovery) and DWI (diffusion weighted imaging) is the single most helpful tool in diagnosing CJD.

The disease course of CJD is highly variable and may mimic many other neurological disorders. The first step is to rule out alternative diagnoses.

Diagnostic criteria have been created to confirm a diagnosis of definite, probable and possible CJD for each form of CJD. These criteria are constantly revised as research and experience provide more data. California physicians should note that Creutzfeldt-Jakob disease (CJD) and other transmissible spongiform encephalopathies (TSEs) are reportable diseases.

While there is no proven cure or treatment for CJD, many of the symptoms can be managed either pharmaceutically or behaviorally.

Prions, the causative infectious proteins, are resistant to conventional chemical and physical sterilization and decontamination methods. They can usually be denatured or hydrolyzed, however, by soaking contaminated instruments in 1N sodium hydroxide for an hour or more and then autoclaving them in distilled water at 132-134 °C for at least an hour. It can be even more difficult to inactivate prions that have come into contact with materials such as metal or glass; when feasible, these instruments should be destroyed.

Genetic and paraneoplastic testing are available in the US.

When patients and families hear about prion disease, they are often scared, sad and angry. You will need to treat these emotional needs and reactions, as well as the neurological ones. Cultural differences may also affect attitudes and decision-making about health care, medications, surgery, experimental or alternative treatments or autopsy.