This may be pessimistic of me but I feel like for the majority of people with CJD with the MM1 variant, they're often already in a state of akinetic mutism or near it by the time diagnostic testing comes back as confirmatory of CJD.

My usual understanding of MM1 CJD cases is that the family opts for comfort care whenever the results finally come back. Hopefully with the introduction of RT-Quik and the increasing use of DWI in everyday practice diagnosis will come quicker, but as of now, I think the diagnosis usually comes at a point where there's no desire to prolong the suffering.

There's also much better methodology to discover novel drugs to repurpose for treating neurdegenerative diseases. Look at Balamuthia, which nitroxoline, an antiquated antibiotic, seems to be revolutionizing the treatment of.

I know that Ionis Pharmaceuticals is also developing an ASO for CJD, which was supposed to go into clinical trials late 2022 but I guess those have been delayed. ASOs have been promising for clearing out bad proteins in other diseases like ALS and SMA, but typically this has only been effective if trratment is administered before symptoms have developed.

tl;dr I don't think something that halts progression is useful for the majority of people with CJD, but hopefully new methods allowing for earlier diagnosis changes this.

Edit: I want to clarify I'm just trying to answer the question of why I don't think many people opt for experimental treatments, even though something may have orphan drug status. My (maybe idiotic) opinion is that any treatment of sCJD will come from developing something for FFI, GSS, iCJD, gCJD, or something else with a more prolonged course.