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Science 29 July 1994: Vol. 265. no. 5172, pp. 648 - 651 DOI: 10.1126/science.8036512
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Articles
Science, Vol 265, Issue 5172, 648-651
Copyright © 1994 by American Association for the Advancement of Science
Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis
SW Rogers,
PI Andrews,
LC Gahring,
T Whisenand,
K Cauley,
B Crain,
TE Hughes,
SF Heinemann,
and
JO McNamara
Salt Lake City Geriatric Research Education and Clinical Center, Veterans Affairs Medical Center, UT.
Rasmussen's encephalitis is a progressive childhood disease of unknown cause characterized by severe epilepsy, hemiplegia, dementia, and inflammation of the brain. During efforts to raise antibodies to recombinant glutamate receptors (GluRs), behaviors typical of seizures and histopathologic features mimicking Rasmussen's encephalitis were found in two rabbits immunized with GluR3 protein. A correlation was found between the presence of Rasmussen's encephalitis and serum antibodies to GluR3 detected by protein immunoblot analysis and by immunoreactivity to transfected cells expressing GluR3. Repeated plasma exchanges in one seriously ill child transiently reduced serum titers of GluR3 antibodies, decreased seizure frequency, and improved neurologic function. Thus, GluR3 is an autoantigen in Rasmussen's encephalitis, and an autoimmune process may underlie this disease.
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