| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
From the Department of Neurology (Drs. Baranzini, Laxer, and Oksenberg, R. Saketkhoo, and M. Elkins), University of California at San Francisco; Northern California Comprehensive Epilepsy Center (Dr. Laxer), University of California at San Francisco; Department of Neurology (Dr. Parent), University of Michigan, Ann Arbor; and Neuromuscular Research Department (Dr. Mantegazza), Istituto Nazional Neurologico Carlo Besta, Milan, Italy.
Address correspondence and reprint requests to Dr. Jorge R. Oksenberg, Department of Neurology, University of California at San Francisco, 513 Parnassus Avenue, S-256, San Francisco, CA, 94143-043; e-mail: oksen{at}itsa.ucsf.edu
Background: Autoantibodies have been implicated in the development of chronic focal encephalitis (CFE) or Rasmussen’s disease, a progressive and intractable form of epilepsy characterized by uncontrollable unilateral focal seizures, brain atrophy, and inflammation.
Objective: To investigate the origin and characteristics of the B cell population that trigger or sustain brain inflammation in patients with CFE.
Methods: The authors used immunoglobulin (Ig) complementary determining region 3 (CDR3)-size spectratyping and DNA sequencing to examine the rearranged IgG heavy chain (IgGH) transcript repertoire in resected brain samples from four patients with CFE. They also performed Western blotting on human and rat brain homogenates and immunostaining on a human neuronal cell line to test the reactivity of sera from patients with CFE.
Results: The authors observed substantial perturbations from the normal, unstimulated repertoire of immunoglobulin genes. Sequencing of randomly selected clones confirmed the restricted profile and provided evidence for somatic mutation patterns characteristic of antigen-specific stimulation. They also observed IgGVH-CDR3 sequence diversity among patients. When sera were assayed from patients with CFE for specificity against rat and human brain homogenates, heterogeneous reactivity patterns were detected among patients. Immunostaining of postmitotic human neuronal cells demonstrated reactivity of some patients’ sera against neural antigens.
Conclusions: These findings support an important role for clonally expanded B lymphocytes in some forms of epilepsy, but also indicate a wide spectrum of reactivity characteristic of antigenic heterogeneity.
This article has been cited by other articles:
|
|
 |
|
  C. G. Bien, T. Granata, C. Antozzi, J. H. Cross, O. Dulac, M. Kurthen, H. Lassmann, R. Mantegazza, J.-G. Villemure, R. Spreafico, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement Brain, March 1, 2005; 128(3): 454 - 471. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Watson, Y. Jiang, I. Bermudez, L. Houlihan, L. Clover, K. McKnight, J. H. Cross, I. K. Hart, A. Roubertie, J. Valmier, et al. Absence of antibodies to glutamate receptor type 3 (GluR3) in Rasmussen encephalitis Neurology, July 13, 2004; 63(1): 43 - 50. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Bernasconi, B. Cipelletti, L. Passerini, T. Granata, C. Antozzi, R. Mantegazza, and R. Spreafico Similar binding to glutamate receptors by Rasmussen and partial epilepsy patients' sera Neurology, December 24, 2002; 59(12): 1998 - 2001. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. O. McNamara B cells and epilepsy: The odd couple Neurology, March 12, 2002; 58(5): 677 - 678. [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
