What is acute disseminated encephalomyelitis (ADEM)?

ADEM (acute disseminated encephalomyelitis) is an immune mediated inflammatory demyelinating disorder of the central nervous system. The assault frequently causes injury to the myelin sheath, a protective layer of insulation that surrounds your nerve axon and facilitates communication between nerve cells.

Just as the plastic casing of some electric wires helps with conducting the electrical current, myelin insulates your nerve fibres so that messages can travel down them and pass to other nerve cells in different parts of the body. Symptoms such as difficulty with vision, and weakness of the muscles arise when you have an impairment in your myelin.

You might hear a healthcare provider call ADEM a “demyelinating” condition. It is an acute (sudden) condition. Most of the time, ADEM only occurs once; however it may recur.

What are the symptoms of ADEM?

ADEM typically occurs after viral or bacterial infection symptoms. Symptoms of granulomatous inflammation depend on the site in which this type of inflammatory finding will occur and these could be;

• Fever
• Headache
• Nausea or vomiting
• Confusion
• Fatigue

Signs and symptoms of neurological involvement may include:

• Loss of feeling or tingling in your arms and legs
• Difficulty swallowing
• Vision loss (optic neuritis)
• Muscle weakness
• Difficulty walking
• Seizures
• Symptoms of a STI can range from none, to mild or severe

What is the causes of ADEM?

The specific etiology of ADEM is not known. Studies have also indicated that an infection might cause your immune system to start attacking the threat improperly leading onto inflammation characteristic of ADEM. The first-line of defense for an immune system is to fight foreign invaders as soon as it recognizes them entering the body and making you sick.

For example, your immune system can believe that some parts of the central nervous system are similar to bacteria or virus and damage it by attacking it. This inflammation (swelling) leads to the process of ADEM.

Before developing ADEM symptoms, about 70 to 80% of individuals diagnosed with ADEM have experienced some type of infection or illness. Most cases start about seven to 14 days after the infection. Occasionally, children who have ADEM will develop one of the following infections:

• Flu (influenza)
• Measles
• Mumps
• Rubella
• Chickenpox
• Epstein Barr virus
• Cytomegalovirus
• Herpes simplex virus

Although a number of infectious agents, including bacteria and viruses cause ADEM the underlying infection does not seem to be due to any one bacterial or virus.

The list of causes that lead to ADEM is increasing, in particular among children because now they recognize MOGAD (myelin oligodendrocyte antibody-associated disease) as its own condition. This is considered an autoimmune disease and autoimmunally mediated central nervous system where ADEM can occasionally slip in.

How is ADEM diagnosed?

Your doctor will first conduct an in-depth medical history to know about your symptoms. They will be able to diagnose ADEM after doing a neurological and physical examination as well as testing.

Patients with ADEM may not normally have any causing factors for the attack, but testing should be done to exclude other underlying conditions that can present just like ADEM and potentially eliminate it from being considered in differential diagnosis such as MOGADHonolulu Photographer The first So these are the blood tests we would often order, but most of this is just going to tell us there's an inflammatory process at work.

• An MRI.
• Spinal fluid testing.

ADEM on MRI

This is actually an imaging test that uses magnetic resonance imagining (MRI). It searches for alterations all the way into your brain. A healthcare provider will look for changes in three areas of your central nervous system (brain, spinal cord and optic nerves) such as lesions or spots brought about by the effects of ADEM:

White Matter: This region is home to the nerves fibers. It is the white substance on basic MRI sequences and forms an insulating sheath around your nerve fibers.

Grey matter: contains cell bodies of nerves, and no myelin. This is why it has a darker appearance on routine MRI sequences.

Worried that the areas of your central nervous system may have changed over time, months after you first went through an MRI test last year?

Spinal fluid testing

Spinal Tap/Lumbar Puncture: This diagnostic test is done to determine the underlying reason for neurological symptoms. The neurological team will also test your cerebrospinal fluid (CSF) using a lumbar puncture.

CSF is a colourless fluid surrounding your brain and spinal cord that cushions these parts of the body (protecting them), providing nutrients for their purpose.

The spinal fluid is usually sent off for Cell counts, Chemistries and Infectious Testing that will often show white blood cells in your spinal column which are typically lymphocytes. These are basic units of your immune system.

Your healthcare provider might also obtain a sample of your cerebral spinal fluid (CSF) to determine how your cells respond to specific bacteria or viruses that are believed causes ADEM. It can also help determine whether a person has this disease or other neuroinflammatory diseases.

How is ADEM treated?

Treatment for ADEM is directed at decreasing the inflammation present in your brain and spinal cord. Treatment may include:

Steroids: The primary treatment for ADEM typically involves steroids given intravenously. Your doctor may require more than one infusion of solu-corticosteroids and you many also need to take oral corticosteroid medications.

IVIG (Intravenous Immune Globulin): Delivered as an intravenous infusion of antibodies from donor plasma

Plasma exchange: Plasma exchange or plasmapheresis involves circulating your blood through a device that filters out some parts of it so as to dampen the activity of your immune system. The process involves circulating blood from a vein in your arm into an apparatus and so back through another vein on the opposite arm. This treatment will take several hours to complete and you may need more than one session.

Researchers are continuously seeking a greater understanding of this disease and new treatment choices to help decrease enough symptoms for you to feel better.

Does the treatment have side effects?

Although the side effects of short-term use are relatively mild compared to long term, they can include:

• Raised blood sugar levels
• Low potassium levels
• Difficulty sleeping
• Irritability, Crying & Mood Swings
• Weight gain
• Flushed cheeks
• A metallic taste in the mouth

Side effects of IVIG include:

• An allergic reaction
• Infection
• Shortness of breath
• Blood clots
• Kidney injury
• Headaches

When will I start to feel better after treatment?

After receiving treatment, most people with ADEM make a full recovery within 3 to several days. Recovery on the other hand can take from six weeks to 6 months, thats if your symptoms were relatively mild. The rare case is when some people can not recover fully.

Frequently Asked Questions