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Disease Spotlight: Acute Disseminated Encephalomyelitis

Next up on our tour of all-things-autoimmune is acute disseminated encephalomyelitis, otherwise known as ADEM. While this autoimmune disease doesn’t have as many pseudonyms as some of the other ones that we’ve spotlighted, it sure is a mouthful to say!

It is also a disease full of other complexities. 

ADEM is very rare. ADEM is episodic or “acute”, which means that patients affected by the disease may recover completely, unlike the many autoimmune diseases that are chronic. Because it is so rare and is an acute autoimmune disease, the prevalence has been hard for researchers to establish. 

Typically, ADEM affects younger patients. In fact, 80% of ADEM patients are under the age of 10. Most of the rest are under age 20. (It is considered to be very rare in those over 20 years old.)

A lot of juvenile acute disseminated encephalomyelitis patients contract the disease post-vaccination. This isn’t a reason to avoid the pediatrician or join the anti-vaccination movement — it can occur after an infection, too. A bacterial or viral infection is usually the trigger in young patients, although ADEM can sometimes come along without an apparent causative factor. 

Parents with a child or teen who has ADEM usually discover the condition after taking them to the doctor for symptoms mimicking an array of other diseases. Symptoms include confusion, drowsiness, loss of balance, general shakiness or unsteadiness, falling, blurry vision or double vision, generalized malaise, pain, trouble swallowing, limb weakness, and even seizures. These symptoms are frightening and they describe a wide variety of neurological conditions and infectious diseases. Many doctors initially mistake it for multiple sclerosis. 

So what exactly is ADEM? Like many autoimmune diseases, ADEM involves disruption of the immune system that causes it to destroy healthy cells, organs, or tissue, and also leads to inflammation.  In ADEM the immune system attacks a myelin, a protein-based membrane that surrounds the brain and the spinal cord. Like multiple sclerosis (which is also an autoimmune disease) ADEM can also affect the optic nerve and disturb or impair vision. 

Doctors don’t know why this acute immune disorder mostly affects younger patients rather than adults. What researchers have found, however, is that boys are more likely to develop ADEM than girls, and that ethnicity does not seem to play a role.

Diagnosing a rare disease like ADEM presents many challenges to physicians. Because it so closely mimics MS and even shares likenesses with systemic migraines, it may take some outside-the-box thinking on the doctors’ part to make an accurate ADEM diagnosis. Patients undergo a physical exam along with MRIs, blood tests, and a spinal tap. Other neurological and infectious conditions must be ruled out before a correct ADEM diagnosis can be made.

Unlike some autoimmune diseases that are labeled as rare when they are not, ADEM appears to rightfully fall into the rare disease category. 

But even diseases that are so-called “zebras” in the medical community deserve to be studied —and patients with these diseases need to connect with others like themselves to realize they are not alone. 

This kind of progress begins with you. So we ask that you get involved. We need donations to our nonprofit organization. We also need patients to fill out our survey — and for patient advocacy groups (PAGs) to begin sharing information both with us and about us. 

We’re looking for guest bloggers, too, to share the ADEM patient experience so that you have a place to go for information and commiseration.

If you live with ADEM or are an ADEM blogger, patient advocate, or advocacy group, we’d love to hear your story. Please email ashley@autoimmuneregistry.org if you wish to contribute a guest blog about life with acute disseminated encephalomyelitis. 

In the meantime, please visit the Transverse Myelitis Association or the Acute Disseminated Encephalomyelitis Support Group for more information. You may also acquire info from the National Multiple Sclerosis Society. Please check out our ADEM fact sheet, here

… And don’t forget to join our free registry, so we can learn more about what life is like for you as an autoimmune patient.