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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Rasmussen encephalitis


Other Names for this Disease
  • RE
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Overview



What is Rasmussen encephalitis?

What causes Rasmussen encephalitis?

How might Rasmussen encephalitis be treated?


What is Rasmussen encephalitis?

Rasmussen encephalitis is a chronic inflammatory neurological disease that usually affects only one hemisphere of the brain. It most often occurs in children under the age of 10, although adolescents and adults may also be affected. Rasmussen encephalitis is characterized by frequent and severe seizures, loss of motor skills and speech, paralysis on one side of the body (hemiparesis), inflammation of the brain (encephalitis), and mental deterioration. While the cause of Rasmussen encephalitis is unknown, there is evidence that in many patients it is an autoimmune disorder.[1] Immune therapy and surgery may be used for treatment.[2]
Last updated: 3/22/2012

What causes Rasmussen encephalitis?

The underlying cause of Rasmussen encephalitis remains unknown. Current evidence suggests that autoimmune processes are responsible for the damage to the brain cells.[1][3] In most patients, the trigger of the abnormal immune response is unclear, although it may follow an otherwise minor bacterial or viral infection or head injury.[2]  Rasmussen encephalitis is a sporadic disease. It has not been associated with any particular environments or populations and cannot be caught from others.[3] Research is underway to help better understand the underlying cause.[1][3]
Last updated: 3/22/2012

How might Rasmussen encephalitis be treated?

Anti-epileptic drugs are usually not effective in controlling the seizures associated with Rasmussen encephalitis. Recent studies have shown some success with treatments that suppress or modulate the immune system, in particular those that use corticosteroids, intravenous immunoglobulin, or tacrolimus. Surgery to control seizures may be performed in later stages of the disease when neurological deficits stabilize. Surgical procedures, such as functional hemispherectomy (removal of half of the brain) and hemispherotomy (a surgical procedure for hemispheric disconnection), may reduce the frequency of seizures and also improve behavior and cognitive abilities.[1]   
Last updated: 3/22/2012

References
  1. NINDS Rasmussen's Encephalitis Information Page. National Institute of Neurological Disorders and Stroke (NINDS). December 19, 2011; http://www.ninds.nih.gov/disorders/rasmussen/rasmussen.htm. Accessed 3/22/2012.
  2. Hart I. Rasmussen Encephalitis . The Encephalitis Society. July 2005; http://www.encephalitis.info/Info/TheIllness/TypesEncephalitis/PostInfectious/Rasmussen.html. Accessed 3/22/2012.
  3. Frequently Asked Questions by Parents of Children with Rasmussen's Encephalitis (RE). RE Children's Project. Janurary 2012; http://rechildrens.org/images/stories/RE_FAQ_Jan_2012.pdf. Accessed 3/22/2012.