What is Rasmussen's Encephalitis?
Rasmussen’s encephalitis is a rare, chronic inflammatory disease that usually
affects only one hemisphere of the brain. It occurs in children under the age of
10 (and more rarely in adolescents and adults), and 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. Most individuals with Rasmussen’s encephalitis will experience
frequent seizures and brain damage over the course of the first 8 to 12 months,
and then enter a phase of permanent, but stable, neurological deficits.
Scientists currently think that Rasmussen’s encephalitis is an autoimmune
disease in which immune system cells enter the brain and cause inflammation and damage.
Is there any treatment?
Anti-epileptic drugs are usually not effective in controlling seizures.
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 and hemispherotomy, may reduce
the frequency of seizures and also improve behavior and cognitive abilities.
What is the prognosis?
The prognosis for individuals with Rasmussen’s encephalitis varies. Despite
the advances in medical treatment, none has yet been shown to halt the progress
of the disease in the long term. The disorder may lead to severe neurological
deficits or it may cause only milder impairments. For some children, surgery
decreases seizures. However, most individuals with Rasmussen’s encephalitis are
left with some paralysis, cognitive deficits, and problems with speech.
What research is being done?
The National Institute of Neurological Disorders and Stroke (NINDS) conducts
research related to Rasmussen’s encephalitis in its laboratories at the National
Institutes of Health (NIH), and also supports additional research through grants
to major medical institutions across the country. Much of this research focuses
on finding better ways to prevent, treat, and ultimately cure progressive
neurological disorders, such as Rasmussen’s encephalitis.
Organizations
American Autoimmune Related Diseases Association
22100 Gratiot Avenue
Eastpointe, MI 48201-2227
aarda@aarda.org
www.aarda.org
Tel: 586.776.3900 800.598.4668
Fax: 586.776.3903
Epilepsy Foundation
8301 Professional Place
Landover, MD 20785-7223
postmaster@efa.org
www.epilepsyfoundation.org
Tel: 301.459.3700 800.EFA.1000 (332.1000)
Fax: 301.577.2684
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
www.rarediseases.org
Tel: 203.744.0100 Voice Mail 800.999.NORD (6673)
Fax: 203.798.2291
Source: National Institutes of Health; The National Institute of Neurological Disorders and Stroke
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 2/7/2008...#6092
