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Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the CNS with severe involvement of the optic nerve and spinal cord. Highly specific serum IgG autoantibodies (NMO-IgG) that react with aquaporin-4 (AQP4), the most abundant CNS water channel protein, are found in patients with NMO. However, in vivo evidence combining the results of AQP4 antibody serum levels and brain pathology is lacking. We report a patient with NMO whose AQP4 antibody levels decreased simultaneously with clinical deterioration caused by the development of a tumor-like brain lesion. In the seminecrotic biopsied brain lesion, there was activated complement complex, whereas only very scattered immunoreactivity to AQP4 protein was detectable. The decrease in serum AQP4 antibody levels and the loss of AQP4 in the tumor-like lesion could represent a "serum antibody-consuming effect" during lesion formation.

Original publication

DOI

10.1097/NEN.0000000000000173

Type

Journal article

Journal

J Neuropathol Exp Neurol

Publication Date

03/2015

Volume

74

Pages

194 - 197

Keywords

Antibodies, Anti-Idiotypic, Aquaporin 4, Biomarkers, Brain Neoplasms, Female, Humans, Immunoglobulin G, Middle Aged, Neuromyelitis Optica