MOG-expressing teratoma followed by MOG-IgG-positive optic neuritis
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MOG expressing teratoma followed by MOG‑IgG‑positive optic neuritis Brigitte Wildemann1 · Sven Jarius1 · Jonas Franz2 · Klemens Ruprecht3 · Markus Reindl4 · Christine Stadelmann2 Received: 30 July 2020 / Revised: 4 October 2020 / Accepted: 5 October 2020 © The Author(s) 2020
Keywords Myelin oligodendrocyte glycoprotein (MOG) · Antibodies · Optic neuritis · Ovarian teratoma A paraneoplastic etiology has been reported in few patients with aquaporin-4 (AQP4)-IgG-seropositive neuromyelitis optica spectrum disorders (NMOSD), with lung and breast cancer being the most frequent associated malignancies [12]. Whether MOG encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease [MOGAD]), a rare autoimmune disease characterized by serum immunoglobulin G antibodies (IgG) against myelin oligodendrocyte glycoprotein (MOG) and overlapping clinical and radiological features with both NMOSD and multiple sclerosis (MS) [4–7, 11], may occur in a paraneoplastic context is less well known. We report on a patient with oligosymptomatic MOG-EM emerging several months after detection and resection of an ovarian teratoma. Histopathology revealed neural tissue expressing MOG protein and accompanying immune cell infiltration within the teratoma, suggesting a possible paraneoplastic origin of MOG-EM in this case. A 26-year-old woman with a history of Hashimoto thyroiditis developed right abdominal pain persisting for several weeks. A pelvic MRI showed a mass of 6 cm diameter Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00401-020-02236-5) contains supplementary material, which is available to authorized users. * Brigitte Wildemann [email protected]‑heidelberg.de 1
Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Germany
2
Institute of Neuropathology, University Medical Center Göttingen, Robert‑Koch‑Strasse 40, 37075 Göttingen, Germany
3
Department of Neurology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany
4
Clinical Department of Neurology, Medical University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria
adjacent to the right ovary displaying sebaceous components and calcifications. The right ovary was resected and histology confirmed a mature cystic teratoma. Eleven months later and within 3 weeks of an upper respiratory infection, the patient developed left-sided optic neuritis. Symptoms included mild eye pain, visual blurring, impaired color vision, along with a drop in visual acuity to 0.6. Cranial magnetic resonance imaging (MRI) revealed a normal optic nerve and a solitary small T2/fluid-attenuated inversion recovery lesion in the right frontal white matter; spinal MRI was unremarkable. CSF analysis disclosed normal cell and protein profiles and no oligoclonal bands. Serology for AQP4-IgG and NMDA-rece
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