ADEM: Symptoms, Causes, Treatment and Diagnosis

Overview

Acute disseminated encephalomyelitis, or ADEM, is a rare neurological condition. Anyone can get affected by ADEM, but it mostly affects children. It usually develops after a bacterial or viral infection. 

People diagnosed with acute disseminated encephalomyelitis experience brain and spinal cord inflammation. The condition is known to damage myelin – the protective coating of the nerve fibres. ADEM may cause severe symptoms that are treatable. It is important to note more than 85% patients fully recover without sequelae .

The blog explains acute disseminated encephalomyelitis, its symptoms, causes, diagnosis, and treatment options. 

What is an Acute Disseminated Encephalomyelitis?

As mentioned earlier, acute disseminated encephalomyelitis is a rare neurological disorder that causes brain and spinal cord inflammation. The inflammation damages the protective layers of the nerve fibers known as myelin. It primarily affects children, but adults are susceptible too. It develops after a viral or bacterial infection. 

In rare cases, it also affects the optic nerve. The disorder is most common in the winter and spring months. Each year at least one in 2,50,000 people get affected by ADEM.

What are the Symptoms of an Acute Disseminated Encephalomyelitis?

You may develop acute disseminated encephalomyelitis two to three weeks after a viral or bacterial upper respiratory tract infection or another form of infection in the body. 

The onset of the symptoms is sudden and worsens quickly. The following are the symptoms:

1. Fever
2. Headache
3. Stiffness in the neck
4. A sensation of weakness, numbness, and tingling in the arms and legs
5. Problems in the balance
6. Lethargy
7. Optic neuritis (you may experience blurred or doubled vision as the disorder causes inflammation of the optic nerve)
8. Swallowing and speaking problems
9. Issues in the bladder and bowel
10. Changes in behavior, such as confusion and crankiness
11. In rare cases, seizures or coma
12. Paralysis on one side of the body

What Causes Acute Disseminated Encephalomyelitis?

Viral and bacterial infection of some kind causes acute disseminated encephalomyelitis. In nearly 70% of the cases, the disease develops after a viral or bacterial infection, mainly a sore throat or cough. Several different bacteria, viruses, and other infections are associated with ADEM. However, the disease does not occur due to any one infectious agent. Most cases of ADEM start nearly a week or 14 days after the infection.

Most experts believe acute disseminated encephalomyelitis is an immune reaction to the infection. Children may develop this disease soon after getting inoculated with certain vaccines, such as measles, mumps, and rubella. However, experts are yet to find a tangible link between the two. 

When to Seek Medical Help for Acute Disseminated Encephalomyelitis?

If you or your child is experiencing the symptoms of ADEM, you should immediately contact your healthcare provider. 

Request an appointment at Apollo Hospitals   

Who is at Risk of Developing Acute Disseminated Encephalomyelitis?

You may be at higher risk of developing ADEM if you have the following risk factors:

1. Age: Children under 10 are more susceptible to ADEM – nearly 80% of cases. 
2. Sex: Boys are at a higher risk of developing ADEM than girls
3. Time of the year: The cases increase during winter or spring

How do Doctors Diagnose an Acute Disseminated Encephalomyelitis?

When there is a close link between infection and the occurrence of more than one neurological symptom, often accompanied by headaches, fever, and a changed mental state, the doctor may suspect ADEM. These symptoms may worsen over a few days. However, no one test can determine the disease. So, doctors conduct the following series of tests to assess ADEM accurately:

1. MRI scans: Magnetic Resonance Imaging (MRI) scanning helps identify any changes in the brain. If your doctor suspect of ADEM, they notice widespread, multiple changes in the deep areas of your brain, known as the white matter. This white matter contains nerve fibers and is also a part of the brain and spinal cord. 

It is called white matter as it contains the protective layer known as myelin while the grey matter contains nerve cells. Sometimes, the grey matter is also affected by lesions. These changes can occur due to ADEM and numerous other health conditions such as multiple sclerosis, direct brain infection, and in rare cases, tumours. 

2. Lumbar puncture: It is a necessary test for ADEM as it rules out any direct infections or other health issues similar to ADEM. The doctor tests your cerebrospinal fluid for several other neurological diseases too. If you are suffering from ADEM, your spinal fluid shows an increase in white blood cells, usually lymphocytes. Lymphocytes are an integral part of your immune system. Also, certain proteins that are typical in multiple sclerosis would be absent in ADEM .

What are the Treatment Options Available for Acute Disseminated Encephalomyelitis?

Your doctor may have to devise a single or combined treatment plan depending on your age, overall health, symptoms, and medical health. However, the main goal of the treatment plan is to minimize the inflammation in your central nervous system.

The first line of defense is administering steroid medications, such as methylprednisolone, intravenously for five days to one week. An additional oral steroid may be required for a short duration to help with your symptoms. During the steroid treatment, your doctor will closely monitor you for side effects, such as a metallic taste, swelling of the face, flushing, sudden weight gain, and problems sleeping. 

Another option is intravenous immune globulin (IVIg) is administered intravenously for close to five days. Again, your doctor monitors you for possible side effects, such as infections, allergic reactions, and breathlessness.

In severe cases, you may have to undergo plasmapheresis. Hospitalization is necessary as doctors filter your blood to remove harmful antibodies. This treatment is repeated several times during your hospital stay. When you do not respond to medicines, the doctors may consider chemotherapy treatment. 

Once you have completed the treatment, a follow-up MRI is necessary to ensure the inflammation is under control.

Conclusion

Acute disseminated encephalomyelitis affecting your children may not a challenging experience. But understanding that recovery is possible and is a slow process is essential. Children recover faster than adults but may take a year to recover completely. 

In rare cases, children may have a lasting impairment, such as vision damage or muscle weakness. ADEM develops only once. It is essential to get an early diagnosis to prevent any lasting side effects. 

Frequently Asked Questions (FAQs)

Is ADEM preventable?

As the cause of ADEM is unclear, there are no ways to prevent it. However, seeking medical help for  any neurological issue early is essential to avoid possible long-term and severe complications.