P11.49.B MOG antibody positive paraneoplastic brain stem encephalitis associated with cervical cancer: a rare case report. (5th September 2022)

• Record Type: Journal Article
• Authors: Bieler, L; Hecker, C; Machegger, L; Wolfrum-Ristau, P; Trinka, E; Kleindienst, W
• Is Part Of: Neuro-oncology. Volume 24(2022)Supplement 2
• Page Start: ii69
• Page End: ii69
• Publication Date: 2022-09-05
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noac174.238
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: Background: Paraneoplastic neurological syndromes are a rare complication of malignant tumors. Myelin oligodendrocyte glycoprotein (MOG) is an important marker on the surface of oligodendrocytes and is associated with many demyelinating diseases. There are only few reports of MOG antibody positive paraneoplastic syndromes. Here we present the first case of MOG-antibody positive brain stem encephalitis associated with cervical cancer. Material and Methods: Case report. Results: A 58-year-old female presented with headache, fever and acute cerebellar signs. 13 months earlier she was diagnosed with cervical cancer and ovarian metastases. Adnexectomy was performed and she received adjuvant chemo- and radiotherapy. At admission cerebrospinal fluid (CSF) analysis showed pleocytosis (90 cells/µl) with a mixed cell profile but no malignant cells could be detected. Protein and lactate were highly elevated. The patient was treated with aciclovir and ceftriaxone until serology and liquor PCR showed no evidence of systemic or cerebral infections. Initial magnetic resonance imaging (MRI) of the brain was unremarkable. The patient was symptomatically treated. CSF two weeks later showed 53 cells/µl, protein and lactate almost normal. Contrary to the mild improvement of CSF parameters, cerebellar symptoms worsened, and the patient complained about impaired vision and diplopia. MRI revealed gadolinium enhancing lesions of the left mesencephalon and the right N. opticus. Visual evoked potentials confirmed a demyelinating lesion of the right N. opticus. We then assumed paraneoplastic brain stem encephalitis and the patient received high dose methylprednisolone and immunoglobulins. Ultimately, lab results came back positive for anti-MOG-antibodies, while paraneoplastic panels were negative. At follow-up three weeks later, CSF analysis showed normal cell count and brain MRI was unremarkable again. MOG-antibodies however remained positive. Since then, the patient has received monthly immunoglobulins leading to a stable neurological status. Unfortunately, the cancer itself is progressive with a local relapse and metastases of liver, lung and bones. Conclusion: Our patient had an acute presentation of a rare brain stem encephalitis. However, infectious workup as well as all well-known onconeural antibodies showed negative results. This shows that pleocytosis in patients with a history of malignant disease is highly suggestive for paraneoplastic neurological syndromes even if common antibodies show negative results. Clinicians should be aware of other autoimmune antibodies like anti-MOG-antibodies and should extend workup if necessary. Vice versa it is worth considering tumor workup in patients with neurological symptoms and anti-MOG-positivity. Furthermore, the appearance of paraneoplastic neurological syndromes is always suspicious for progressive disease.