Mysterious presentation of autoantibody in a neuromyelitis optica spectrum disorder patient

Abstract

Background Treatment with interferon beta often leads to the development of relapses and expedites functional disability in neuromyelitis optica spectrum disorder patients. An accurate diagnosis frequently requires the presence of anti-aquaporin-4 (AQP4) antibodies when we encounter a patient who manifests atypical radiological findings. Case presentation A 35-year-old woman developed sudden vertigo and had lesions around her bilateral dentate nuclei, a part of which was enhanced with gadolinium. Soon, she developed optic neuritis. Other lesions in the central nervous system and anti-AQP4 antibody staining using cell-based assays were not detectable. During the treatment with interferon beta-1a, re-examination of the anti-AQP4 antibody staining led to a positive result; however, this was detectable by an enzyme-linked immunosorbent assay. The latent development of an intractable hiccup and a postrema lesion fulfilled the latest diagnostic criteria of neuromyelitis optica spectrum disorder, even if the patient did not have anti-AQP4 antibodies. Conclusions Anti-AQP4 antibodies detected only by enzyme-linked immunosorbent assay might be a false positive result. However, alteration in the immune balance between T-helper cell type 1 and T-helper cell type 2 were suspected during the treatment with interferon beta-1a.