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Acute Disseminated Encephalomyelitis

Acute Disseminated Encephalomyelitis
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Acute disseminated encephalomyelitis (ADEM) is a short and sudden bout of inflammation in the brain, spinal cord and, sometimes, the optic nerves. This inflammation damages the protective myelin (white fatty tissue that surrounds nerves) in the brain. ADEM is a demyelinating disorder, which is any condition that damages the myelin.

The inflammation usually occurs one to two weeks after a viral or bacterial infection, such as a sore throat or cough. ADEM very rarely occurs within three months after a child receives a vaccination for measles, mumps and rubella (MMR vaccine). The infection or vaccination triggers an autoimmune response, causing the body’s immune system to attack its own healthy myelin tissue.

The damaged myelin and resulting inflammation cause a variety of symptoms. Symptoms of acute disseminated encephalomyelitis include:

  • Fever
  • Weakness
  • Numbness or tingling
  • Headache
  • Confusion
  • Vomiting
  • Blurry vision
  • Changes in color perception
  • Altered mental state

ADEM is typically a one-time event that most often affects children younger than 10 years old, although it can recur within a few months of the initial episode. The condition affects 1 in every 125,000 to 250,000 individuals each year. The condition occurs most often in winter and spring.

The symptoms of ADEM are similar to other conditions, including pediatric multiple sclerosis , optic neuritis (inflammation in the bundle of nerves that sends signals from the eye to the brain), transverse myelitis (inflammation in the spinal cord) and recurrent ADEM (acute disseminated encephalomyelitis that returns).

If your child shows signs of ADEM, a neurologist will perform specific tests to make an accurate diagnosis. Most children with ADEM make a complete and full recovery. Recovery time may take only a few days, but some cases may take up to a year to heal. Some children may continue to have some symptoms, such as blurred vision, numbness or weakness.

Diagnosis of Acute Disseminated Encephalomyelitis

Doctors at Riley at IU Health perform the following exams and tests to diagnose acute disseminated encephalomyelitis:

  • Medical history. The neurologist will review your child’s medical history, especially any recent infections or vaccinations.
  • Neurological exam. The doctor will check your child’s balance and look for signs of weakness in the limbs, numbness and changes in vision.
  • Magnetic resonance imaging (MRI). The doctor will use MRI to look at the condition of the nerves in the brain and spinal cord.
  • Lumbar puncture. A sample of spinal fluid is removed and checked for signs of central nervous system infections like meningitis or encephalitis, inflammation, cell count, protein and immune markers.
  • Blood tests. A blood sample will be studied in the lab to check for infection triggers and other conditions similar to ADEM.

If repeated bouts of demyelinating symptoms occur, or if old and new lesions can be seen on an MRI exam, your child’s doctor may recommend further tests to check for other conditions, such as pediatric multiple sclerosis. The presence of older lesions on an MRI image may indicate multiple sclerosis, which can cause brain lesions before symptoms appear. A single episode of ADEM causes widespread myelin damage, while multiple sclerosis causes many attacks over time.

Treatments

Treatments

Treatments for acute disseminated encephalomyelitis focus on reducing inflammation around the nerves to stop symptoms. Treatments include:

  • High-dose steroids. Corticosteroids are given by intravenous injection (IV), usually during a three- to five-day course of treatment.
  • Oral steroids. After high-dose IV steroids, patients often receive a tapering steroid dose by mouth for a few days.

If ADEM recurs within a few months, corticosteroids are restarted.

Key Points to Remember

Key Points to Remember

  • Acute disseminated encephalomyelitis (ADEM) is a short and sudden bout of inflammation in the brain, spinal cord and, sometimes, the optic nerves. This inflammation damages the protective myelin (white fatty tissue that surrounds nerves) in the brain.
  • ADEM often occurs after a viral or bacterial infection. The MMR vaccine can very rarely trigger the condition.
  • ADEM is an autoimmune condition where the body’s immune system attacks its own healthy myelin tissue.
  • Treatment usually involves injections of corticosteroids for three to five days followed by a tapering dose of oral steroids for a few days.

Support Services & Resources

Support Services & Resources

View these links to discover support groups and more resources for ADEM.

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

Transverse Myelitis Association.

This association's website includes information on symptoms, diagnosis, treatment and prognosis of ADEM, a listing of clinical trials, a message forum and access to a physician network.

National Institute of Neurological Disorders and Stroke.

This National Institutes of Health website offers information about the symptoms, treatment and prognosis for patients with acute disseminated encephalomyelitis, as well as links to clinical trials.

National Multiple Sclerosis Society.

The National Multiple Sclerosis Society provides information on ADEM, including support groups and the latest research into the disorder.


Acute Disseminated Encephalomyelitis Research

Acute Disseminated Encephalomyelitis Research

Pediatric neurologists at Riley at IU Health work closely with researchers, neurologists and neuroscientists at the Stark Neurosciences Research Institute at the Indiana University School of Medicine and the Indiana Clinical and Translational Sciences Institute, where research studies related to acute disseminated encephalomyelitis are underway.

Departments Treating This Condition

Departments Treating This Condition

  • Neurology
  • Ophthalmology
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