Acute Disseminated Encephalomyelitis

Acute disseminated encephalomyelitis, known as ADEM, is a neurological disorder characterized by brief, intense attacks of swelling and inflammation in the brain and spinal cord. It typically causes symptoms that may be confused with those of multiple sclerosis, since both conditions are related to damaged myelin in the brain. While ADEM is more common in children than in adults, it can affect people of all ages. Unlike multiple sclerosis, ADEM typically only occurs once.

Symptoms

  • Blurry or double vision
  • Confusion
  • Difficulty swallowing
  • Drowsiness
  • Fever
  • Headache
  • Unsteadiness or an increased risk of falling
  • Weakness in the upper or lower extremities

In adults with ADEM, symptoms related to movement and the sensory system are also common.

Prevention

No specific way to prevent ADEM is known, but because it usually follows the onset of a virus or bacterial infection, basic hygiene may lower the risk. Wash your hands regularly, avoid touching unwashed hands to your face and disinfect commonly touched surfaces to lower the risk of illness.

Risk Factors

  • Bacterial infection
  • Viral infection
  • Young age (most commonly ages 10 and younger)

Diagnosis

  • Medical history and symptom review. Because ADEM often follows an acute infection, it may be discovered as part of a follow-up medical visit. At this visit, your medical provider will carefully review your symptoms, along with any treatment you received for the viral or bacterial infection. Your complete medical history and lifestyle habits will also be discussed.
  • Physical examination. A basic physical exam will be performed to help review your symptoms and determine their severity. During this exam, your provider will also be able to rule out other potential causes of your symptoms.
  • Additional tests. As with most conditions related to the brain and spinal cord, additional testing will be required to help a provider make a definitive diagnosis. Testing will likely include an MRI scan to look for changes to the white matter in the brain, along with a lumbar puncture to analyze spinal fluid.

Treatment

  • ADEM typically follows a viral or bacterial infection. If symptoms of the infection are lingering, your provider may recommend additional treatment, including prescription medications, to fully eradicate symptoms.
  • To reduce the swelling related to ADEM, high-dose corticosteroids are administered intravenously, typically for up to five days.
  • If this regimen of IV medication is successful in reducing inflammation, it may be followed by a short, tapering dose of oral steroids for a few days.
  • If this regimen of IV medication is not successful in reducing inflammation, your medical provider may recommend plasmapheresis. During this procedure, your blood is circulated through a machine that withdraws components of the immune system from the blood, which reduces inflammation. This therapy requires several hours and is typically done every other day for up to two weeks.
  • In severe cases, IV immune globulin or a chemotherapy medication called cyclophosphamide may also be used to lower the immune system’s inflammation response.

Follow-up Care

  • After swelling is alleviated, recovery is typically fairly rapid. Since a viral infection or bacterial infection typically precedes ADEM, it may take some period of time for your body and immune system to fully recover. Your medical provider may recommend a period of rest to help.
  • In some cases, patients with ADEM may experience lingering issues with cognition, weakness and vision. In that case, your provider may prescribe rehabilitation and adaptive devices to help you recover abilities.
  • After treatment with IV and oral steroids, it’s important to monitor your blood glucose and potassium levels. Your provider will give you a plan of follow-up care, including checkups.