Reduction in Serum Aquaporin-4 Antibody Titers During Development of a Tumor-Like Brain Lesion in a Patient With Neuromyelitis Optica: A Serum Antibody-Consuming Effect?

被引:4
作者
Aboulenein-Djamshidian, Fahmy [1 ,3 ]
Hoeftberger, Romana [4 ]
Waters, Patrick [6 ]
Krampla, Wolfgang [2 ]
Lassmann, Hans [5 ]
Budka, Herbert [4 ]
Vincent, Angela [6 ]
Kristoferitsch, Wolfgang
机构
[1] SMZ Ost Donauspital, Dept Neurol, A-1220 Vienna, Austria
[2] SMZ Ost Donauspital, Dept Radiol, A-1220 Vienna, Austria
[3] Med Univ Vienna, Karl Landsteiner Inst Neuroimmunol & Neurodegene, Vienna, Austria
[4] Med Univ Vienna, Inst Neurol, Vienna, Austria
[5] Med Univ Vienna, Ctr Brain Res, Dept Neuroimmunol, Vienna, Austria
[6] Univ Oxford, John Radcliffe Hosp, Nuffield Dept Clin Neurosci, Neuroimmunol Grp, Oxford OX3 9DU, England
关键词
Aquaporin-4; Brain biopsy; Multiple sclerosis; Neuromyelitis optica; Serum level; Tumor-like lesion; MULTIPLE-SCLEROSIS; WHITE-MATTER; PATHOGENESIS; DISTINCTION; EXPRESSION; ABSENCE;
D O I
10.1097/NEN.0000000000000173
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:194 / 197
页数:4
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