Background

The first question to ask when hearing the words "prion disease" (also known as transmissible spongiform encephalopathies) is exactly what it is. In an extremely simplified definition, "prions" are pathogenic agents that cause misfolded prion proteins that spread by connecting their shape to other proteins. What does this mean? Well, in the world of proteins, your shape defines your characteristics and abilities. Some proteins can connect to others while others cannot. When prions appear, they will begin changing protein shapes and eventually, your body will have a massive problem to deal with: proteins that no longer work for you, and in some cases, even against you. Normally, functional prion proteins positively regulate neural precursor proliferation during developmental and adult neurogenesis. They create neurons throughout your life, and if these are misfolded, well, nothing good happens. Now, what is important to understand is that these proteins have no mind. They have no goal for nutrition, so the way that TSEs kill you is simply by getting rid of your functionality. What makes this even scarier is that your immune system does not give a response to prions, which means if you get one, you essentially don't have a defense.

Description: as seen on the right, the misfolded protein

has an abundance of β-pleated sheets, a type of protein

motif that helps the structure of the protein. However,

the abundance of all these sheets gives the prion protein

a strange tendency to group up a lot of homologous proteins

which can cause damage to internal and external tissue.

Etymology

So what does the name mean? Well let's take a deeper look into it: "Transmissible Spongiform Encephalopathy." Obviously, "transmissible" denotes that prion disease is passed on from one creature to another. Now, what does "spongiform" mean? It is pretty simple too, where the word itself means "to have the consistency of a sponge." In context, however, "spongiform" alludes to the material in your brain. Gray matter, for example, has this consistency. Spongiform in this case can also be defined as any degenerative disease in the brain characterized by the development of porous spongelike lesions in brain tissue. And now the scariest word, "encephalopathy." Encephalopathy is actually simpler than it seems. It is a form of disease where the brain is affected by some agent or condition. These can include brain tumors, dementia, and more.

Cures and Treatments

Now that you probably get the general gist of these scary pathogenic agents, how can you treat them? The short answer is: you can't. What make prions so lethal is the fact that there is no way to stop them from killing you. You can slow them down with treatments, but sooner or later, they will inflict death. There is no effective therapy either.

So, then what? If we can't stop death, what can we do? Multiple breakthroughs in immunotherapy have allowed humans to slow down the fatality of non-curable diseases and a few other tough curable diseases like cancer. Like everything else, immunotherapy is a lot simpler than it sounds. Primarily, immunotherapy is used for diseases like cancer because it helps immune cells find enemies easier. However, scientists have spent lots of effort trying to fight off other encephalopathies like Alzheimer's Disease and Parkinson's Disease. Prions are, like Alzheimer's and Parkinson's, neurodegenerative diseases, meaning similarities could be exploited and prions could have a harder time expanding their kill count.

Immunotherapy: Yes or No?

Then begs the question, should this immunotherapy be used? This is a question with a personal answer, but for me, I think it is a good idea to use this weapon against prions. Immunotherapy can be used extremely specifically and has very little side effects, which is probably why it is also a viable solution to fighting cancer.

The reason why this is not yet implemented too much is that it is too soon to see just how good it is. Recent trials have provided evidence for success, but the blood-brain barrier is a fragile place and any mistake can do a lot of things worse than just death (like nerve damage). Another difficulty that researchers will face is actually finding a way to target these pathogenic agents. As previously mentioned, there is no humoral immune response to prions, which means there still is no clear path to defeating prions. Immunotherapy, although it doesn't have a lot of side effects, can still cause the immune system to attack healthy cells, problems in the GI tract, difficulties in the muscles and skeleton, and a few other side effects that make people a little worried about these.

Conclusion

Right now, it seems a little too early to decide on whether or not immunotherapy is good for treating diseases especially in the brain. Recent research has shown some positive results for it, but speculation and inductive reasoning has put this treatment on reserve. From what we have seen from other diseases like cancer, immunotherapy can be useful, but it can also hurt the body as well. We will most likely have to play the waiting game to see if immunotherapy is truly effective against neurodegenerative diseases.