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Brain Advance Access originally published online on October 22, 2008
Brain 2008 131(11):3072-3080; doi:10.1093/brain/awn240
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© 2008 The Author(s)
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/2.0/uk/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Antibody to aquaporin-4 in the long-term course of neuromyelitis optica

S. Jarius1,2, F. Aboul-Enein3, P. Waters1, B. Kuenz4, A. Hauser5, T. Berger4, W. Lang6, M. Reindl4, A. Vincent1 and W. Kristoferitsch3

1Neurosciences Group, Weatherall Institute of Molecular Medicine, and Department of Neurology, John Radcliffe Hospital, University of Oxford, UK, 2Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany, 3Department of Neurology, Sozialmedizinisches Zentrum Ost – Donauspital, Vienna, 4Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, 5Department of Laboratory Medicine, Sozialmedizinisches Zentrum Ost – Donauspital, Vienna and 6Department of Neurology, Hospital Barmherzige Brueder, Vienna, Austria

Correspondence to: Univ.-Doz. Dr Wolfgang Kristoferitsch, Department of Neurology, Sozialmedizinisches Zentrum Ost - Donauspital, Langobardenstraße 122, 1220 Vienna, Austria E-mail: wolfgang.kristoferitsch{at}wienkav.at

Neuromyelitis optica (NMO) is a severe inflammatory CNS disorder of putative autoimmune aetiology, which predominantly affects the spinal cord and optic nerves. Recently, a highly specific serum reactivity to CNS microvessels, subpia and Virchow–Robin spaces was described in patients with NMO [called NMO–IgG (NMO–immunoglobulin G)]. Subsequently, aquaporin-4 (AQP4), the most abundant water channel in the CNS, was identified as its target antigen. Strong support for a pathogenic role of the antibody would come from studies demonstrating a correlation between AQP4-Ab (AQP4-antibody) titres and the clinical course of disease. In this study, we determined AQP4-Ab serum levels in 96 samples from eight NMO–IgG positive patients (median follow-up 62 months) in a newly developed fluorescence-based immunoprecipitation assay employing recombinant human AQP4. We found that AQP4-Ab serum levels correlate with clinical disease activity, with relapses being preceded by an up to 3-fold increase in AQP4-Ab titres, which was not paralleled by a rise in other serum autoantibodies in one patient. Moreover, AQP4-Ab titres were found to correlate with CD19 cell counts during therapy with rituximab. Treatment with immunosuppressants such as rituximab, azathioprine and cyclophosphamide resulted in a marked reduction in antibody levels and relapse rates. Our results demonstrate a strong relationship between AQP4-Abs and clinical state, and support the hypothesis that these antibodies are involved in the pathogenesis of NMO.

Key Words: Devic syndrome; neuromyelitis optica; longitudinally extensive transverse myelitis; NMO-IgG; aquaporin-4 antibody; long-term follow-up

Abbreviations: Ab, antibody; AChR, acetylcholine receptor; AQP4, aquaporin-4; CNS, central nervous system; FIPA, fluorescence based immunoprecipitation assay; FU, fluorescence units; IgG, immunoglobulin G; IVMP, intravenous methylprednisolone; LETM, longitudinally extensive transverse myelitis; MRI, magnetic resonance imaging; NMO, neuromyelitis optica; TG, thyroglobulin; TPO, thyroid peroxidase

Received April 26, 2008. Revised September 1, 2008. Accepted September 1, 2008.


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