A Case Report of Neuromyelitis Optica: An Effective Combination of Immunosuppressants, Corticosteroids and Plasmapheresis

Achyut Kanungo *

Department of Anaesthesiology and Intensive Therapy, IPGME&R, Kolkata-20, India.

Subhajit Das

Department of General Medicine, IPGME&R, Kolkata-20, India.

Rahul Debnath

Department of General Medicine, IPGME&R, Kolkata-20, India.

Jotideb Mukhopadhyay

Department of General Medicine, IPGME&R, Kolkata-20, India.

*Author to whom correspondence should be addressed.


Abstract

A girl in her teens presented with decreased sensation in the right half of the face and right half of the tongue associated with dysphagia, vomiting, and aphasia for 2 weeks with sudden onset flaccid quadriparesis for 7 days with diminished vision in both eyes and double vision for 5 days, with no history of fever, convulsions, diarrhoea, headache, palpitations, shortness of breath, loss of consciousness or features suggestive of bladder and bowel involvement with no history of trauma or similar episodes in the past.

MRI Brain revealed features favouring a demyelinating condition. MRI Spine, autoimmune encephalitis panel, ANA profile, and VEP were normal. NMOSD Screen (IIFD-EUROIMMUN) MOG & AQP-4 analysis showed a positive titre of Anti-NMO antibodies. She was treated with intravenous Methylprednisolone, oral prednisolone, and mycophenolate mofetil with 5 rounds of plasmapheresis. In subsequent follow-up, there was an improvement in power and gait with an improvement in vision.

A patient presenting with optic neuritis usually has a differential diagnosis of multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD) and Myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOG-AD). MS, the commonest entity, should be ruled out first. The other two diagnoses have less favourable prognosis and the clinician should be vigilant about the possibilities, complications, and different treatment modalities. NMOSD can present without ocular symptoms and this significantly expands the presentation and differential of NMOSD when the patient does not have ocular involvement.

Keywords: Neuromyelitis optica, immunosuppressants, corticosteroids, plasmapheresis


How to Cite

Kanungo , A., Das, S., Debnath, R., & Mukhopadhyay , J. (2023). A Case Report of Neuromyelitis Optica: An Effective Combination of Immunosuppressants, Corticosteroids and Plasmapheresis. Asian Journal of Case Reports in Medicine and Health, 6(1), 81–89. Retrieved from https://journalajcrmh.com/index.php/AJCRMH/article/view/160

Downloads

Download data is not yet available.

References

Estiasari R. Neuromielitis Optik. In: Aninditha T, Wiratman W, Eds. Buku Ajar Departemen Neurologi. Jakarta: Penerbit Kedokteran Indonesia. 2017;258-64.

Jasiak-Zatonska M, Kalinowska-Lyszczarz A, Michalak S, Kozubski W. The immunology of neuromyelitis optica- current knowledge, clinical implications, controversies and future perspectives. International Journal of Molecular Sciences. 2016;17(3):273. Available:https://doi.org/10.3390/ijms17030273

Broadley S, Khalili E, Heshmat S, et al. Review Article: Neuromyelitis Optica Spectrum Disorder. ACNR. 2017;17(1):11-14. Available:https://acnr.co.uk/articles/neuromyelitis-optica-spectrum-disorder/

Hundozi Z, Xhiha G, Rrustemi J, Gjikolli B, Jashari F. Neuromyelitis optica with a good response after low doses of corticosteroid therapy: A case report. Int J Drug Dev & Res. 2017;9:08-09.

Available:https://www.ijddr.in/drug-development/neuromyelitis-optica-with-a-good-response-after-low-doses-of-corticosteroid-therapy-a-case-report.pdf

Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177–189. Available:https://doi.org/10.1212/WNL.0000000000001729

Sherman E, Han MH. Acute and chronic management of neuromyelitis optica Spectrum Disorder. Current Treatment Options in Neurology. 2015;17(11):48. Available:https://doi.org/10.1007/s11940-015-0378-x

Trebst C, Jarius S, Berthele A, Paul F, Schippling S, Wildemann B, Borisow N, Kleiter I, Aktas O, Kümpfel T, Neuromyelitis Optica Study Group (NEMOS). Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS Trebst). Journal of Neurology. 2014; 261(1):1–16.

Available:https://doi.org/10.1007/s00415-013-7169-7

Pearce JM. Neuromyelitis optica. Spinal Cord. 2005;43(11):631–634. Available:https://doi.org/10.1038/sj.sc.3101758

Cree BC, Hauser SL. Neuromyelitis optica. Loscalzo J, Fauci A, Kasper D, Hauser S, Longo D, Jameson J(Eds.). Harrison's Principles of Internal Medicine, 21e. McGraw Hill; 2022.

Carnero Contentti E, Rojas JI, Cristiano E, et al. Latin American consensus recommendations for management and treatment of neuromyelitis optica spectrum disorders in clinical practice. Multiple Sclerosis and Related Disorders. 2020;45: 102428. Available:https://doi.org/10.1016/j.msard.2020.102428

Available:https://accessmedicine.mhmedical.com/content.aspx?bookid=3095&sectionid=2654493929392

Bonnan M, Cabre P. Plasma exchange in severe attacks of neuromyelitis optica. Multiple Sclerosis International. 2012; 787630. Available:https://doi.org/10.1155/2012/787630

Silva AR, Barros SV, Rotta NT, Ohlweiler L, Stone I, Mello LR. Doença de Devic: um relato de caso [Devic disease: A case report]. Jornal de pediatria. 2001;77(6): 522–524. Available:https://doi.org/10.2223/jped.356

Songthammawat T, Srisupa-Olan T, Siritho S, et al. A pilot study comparing treatments for severe attacks of neuromyelitis optica spectrum disorders: Intravenous methylprednisolone (IVMP) with add-on plasma exchange (PLEX) versus simultaneous IVMP and PLEX. Multiple sclerosis and related disorders. 2020;38: 101506.

Available:https://doi.org/10.1016/j.msard.2019.101506

Abboud H, Petrak A, Mealy M, et al. Treatment of acute relapses in neuromyelitis optica: Steroids alone versus steroids plus plasma exchange. Multiple sclerosis (Houndmills, Basingstoke, England). 2016;22(2):185–192.

Available:https://doi.org/10.1177/1352458515581438

Kleiter I, Gahlen A, Borisow N, Fischer K, et al. Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses. Annals of Neurology. 2016;79(2): 206–216. Available:https://doi.org/10.1002/ana.24554

Stiebel-Kalish H, Hellmann MA, Mimouni M, et al. Does time equal vision in the acute treatment of a cohort of AQP4 and MOG optic neuritis? Neurology(R) neuroimmunology & neuroinflammation. 2019;6(4):e572.

Available:https://doi.org/10.1212/NXI.0000000000000572

Mandler RN, Ahmed W, Dencoff JE. Devic's neuromyelitis optica: A prospective study of seven patients treated with prednisone and azathioprine. Neurology. 1998;51(4):1219–1220. Available:https://doi.org/10.1212/wnl.51.4.1219

Mealy MA, Shin K, John G, Levy M. Bevacizumab is safe in acute relapses of neuromyelitis optica. Clinical & experimental neuroimmunology. 2015;6(4): 413–418. Available:https://doi.org/10.1111/cen3.12239

Fox E, Lovett-Racke AE, Gormley M, et al. A phase 2 multicenter study of ublituximab, a novel glycol engineered anti-CD20 monoclonal antibody, in patients with relapsing forms of multiple sclerosis. Multiple sclerosis (Houndmills, Basingstoke, England). 2021;27(3):420–429. Available:https://doi.org/10.1177/1352458520918375

Mealy MA, Levy M. A pilot safety study of ublituximab, a monoclonal antibody against CD20, in acute relapses of neuromyelitis optica spectrum disorder. Medicine. 2019;98(25):e15944. Available:https://doi.org/10.1097/MD.0000000000015944