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Inflammatory Brain Disease

What is inflammatory brain disease?

Inflammatory brain disease, also referred to as inflammatory disease of the central nervous system (CNS), is a condition where the brain and/or spinal cord become inflamed. Inflammation in the brain causes irritation and swelling of brain tissue or blood vessels.

Brain inflammation can occur for a variety of reasons. By definition, all inflammatory brain diseases are due to primary processes in which inflammation occurs without a normal trigger. For example, immune system malfunction leads to unnecessary inflammation and continues with no resolution if not treated. This can lead to brain damage over the long term.

Examples of primary inflammation in the brain include:

  • CNS vasculitis, a condition marked by inflammation of the brain's blood vessels. CNS vasculitis is the most common inflammatory brain disease in children.
  • Antibody-mediated inflammatory brain diseases, conditions in which antibodies attack structures in the brain and lead to inflammation of surrounding brain tissue.
  • Demyelinating conditions, such as multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM), are often the result of inflammation in the brain. Demyelination is the destruction of myelin, the protective nerve sheath, resulting in impaired electrical conduction.
  • Very rare inflammatory diseases of the CNS, such as Rasmussen's encephalitis and neurosarcoidosis, are caused by particular immune cells functioning abnormally.

Secondary inflammation occurs second to another disease in the body. For example, inflammation occurs as part of the immune response to infection of the meninges (the membranes that surround the CNS) in meningitis.

Areas of the Brain Affected by Inflammatory Brain Disease
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Signs and symptoms of inflammatory brain disease

The signs and symptoms of inflammatory brain disease can be quite varied depending on what part of the brain is affected. When inflammation occurs at a distinct location in the brain, it results in focal deficits such as:

  • stroke: sudden onset of muscle weakness, headache, dizziness or lack of coordination
  • parathesia: the loss of normal sensation
  • vision loss
  • speech impairment

When inflammation occurs over a widespread area in the brain, it results in diffuse deficits such as:

  • memory loss.
  • decreased alertness.
  • changes in concentration and behavior.

Inflammation in the brain can also present with psychiatric symptoms such as:

  • hallucinations.
  • distortions in thought.
  • confusion.
  • mood swings.

Seizures and headaches are also quite common depending on the extent and location of brain inflammation.

How a doctor can help  

A physician will conduct various tests to help figure out what is causing your child's new symptoms. This might involve blood tests, a spinal tap​, an electroencephalogram (EEG) and different imaging procedures.

Treatment for inflammatory brain disease 

Treatment will vary depending your child's symptoms and the underlying cause of brain inflammation. The following are different types of therapies your child may be prescribed:

  • Inflammatory control: Therapies that control inflammation are usually first in line to prevent inflammation-induced organ destruction. Examples include immunosuppressants such as Prednisone, IVIG, and plasmapheresis.
  • Symptom control: Some medications are prescribed to control your child's symptoms. These medications include anti-seizure agents like Keppra or anti-clotting agents like Aspirin.
  • Side-effect control: Many treatments that attempt to control the immune system also come with some unfortunate side effects. As a result, medications may also be prescribed to promote bone health such as Vitamin D and calcium, or to prevent infection by using antibiotics.

Susanne Benseler, MD

Manisha Sickand, MSc, MD Candidate 2015

Marinka Twilt, MD