Rasmussen's Encephalitis Health Article

Treatment

Early in the progress of Rasmussen's encephalitis, anticonvulsant drugs may help control seizures. Use of the anti-cytomegalovirus drug ganciclovir early in the syndrome produces improvement in some patients. Also, some patients have shown dramatic positive response to removal of anti-GluR3 antibodies from the blood by a process known as plasmapheresis. Currently, researchers are studying the hypothesis that drugs to prevent the formation of membrane-attack complexes might slow or halt the progression of Rasmussen's encephalitis as well as of other neurodegenerative diseases. However, the treatment of choice remained hemispherectomy, surgical removal of the affected half of the brain.

Remarkably, children may show little or no change in personality and no loss of intelligence or memory after having half their brain removed. Some children are irritable, withdrawn, or depressed immediately after surgery, but these symptoms are not permanent. So flexible is brain development that a child with a hemispherectomy may become fluent in one or more languages even if the left side of the brain, where the speech centers are usually located, is removed. Blindness or vision loss in one eye usually results from hemispherectomy, but normal hearing in both ears may be recovered. The older the patient is when the surgery is performed, however, the more likely they are to suffer permanent sensory, speech, and motor losses.

Recovery and rehabilitation

Rehabilitation begins immediately after hemispherectomy with passive range-of-motion exercises. Physical, occupational, and speech therapists are required. For children of school age, neuropsychological testing can help determine what academic setting or grade level is best. Children with hemispherectomies are often able to participate in school at the level appropriate for their age.

Prognosis

The prognosis for children below the age of 10 who are treated early in the course of the syndrome is good. This group can often achieve normal psychosocial and intellectual functioning. Without hemispherectomy, however, persons with Rasmussen's encephalitis eventually suffer near-continuous seizures, mental retardation, and death.

BOOKS

Graham, David I., and Peter L. Lantos. Greenfield's Neuropathology, 6th edition. Bath, UK: Arnold, 1997.

PERIODICALS

Cleaver, Hannah. "Girl Left with Half a Brain Is Fluent in Two Languages." Daily Telegraph (London, England), May 23, 2002.

Duke University. "Mild Injury May Render Brain Cells Vulnerable to Immune System Attack." Ascribe Higher Education News Service October 23, 2002.

Lilly, Donna J. "Functional Hemispherectomy: Radical Treatment for Rasmussen's Encephalitis." Journal of Neuroscience Nursing April 1, 2000.

Mercadante, Marcos T. "Genetics of Childhood Disorders: XXX. Autoimmune Disorders, Part 3: Myasthenia Gravis and Rasmussen's Encephalitis." Journal of the American Academy of Child and Adolescent Psychiatry (September 1, 2001).

Zuckerberg, Aaron. "Why Would You Remove Half a Brain? The Outcome of 58 Children after Hemispherectomy–The Johns Hopkins Experience: 1968–1996." Pediatrics (August 1, 1997).

OTHER

"NINDS Rasmussen's Encephalitis Information Page." National Institute of Neurological Disorders and Stroke. March 30, 2004 (June 2, 2004). <http://www.ninds.nih.gov/health_and_medical/disorders/rasmussn_doc.htm>.

ORGANIZATIONS

National Organization for Rare Disorders (NORD). P.O. Box 1968 (55 Kenosia Avenue), Danbury, CT 06813-1968. (203) 744-0100 or (800) 999-NORD; Fax: (203) 798-2291. orphan@rarediseases.org. <http://www.rarediseases.org>.

Larry Gilman, PhD