Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri

Melike Doğan Ünlü; Serpil Demirci

doi:10.46332/aemj.1303107

Research Article

CLINICAL AND RADIOLOGICAL FEATURES OF PATIENTS DIAGNOSED WITH ANTI-AQUAPORIN 4 AND ANTI-MYELIN OLIGODENTROSIDE GLYCOPROTEIN ANTIBODY-RELATED NEUROMYELITIS OPTICA SPECTRUM DISEASE

Year 2024, Volume: 8 Issue: 1, 94 - 103, 29.04.2024

Melike Doğan Ünlü Serpil Demirci

https://doi.org/10.46332/aemj.1303107

Abstract

Purpose: We aimed to present the clinical, imaging and laboratory findings of patients which diagnosed Neuromyelitis Optica Spectrum Disease (NMOSD) and accompanied by antibody positivity.

Materials ve Metods: This retrospective observational study included 15 patients diagnosed with NMOSH.

Results: Eleven of our patients were female and four were male. The mean age at onset was 51.27±12.26 years. The first clinical attack was simultaneous optic neuritis (ON) and myelitis in 3 patients, myelitis in 6 patients and ON in 5 patients. Ten patients had positive aquaporin-4 (AQP-4) IgG antibody and 5 patients had positive myelin oligodentrocyte glycoprotein (MOG) IgG antibody. Six patients had elevated cerebrospinal fluid (CSF) protein. CSF oligoclonal band of eight patients was Type 1 negative, 1 patient was Type 2 positive, and 1 patient was Type 3 positive. Eight patients had normal cranial magnetic resonance imaging, while 1 patient had lesions similar to multiple sclerosis (MS). Two patients had a previous diagnosis of Sjögren's Syndrome (SS) and 3 patients were diagnosed with SS in our clinic. Intravenous methylprednisolone (1000 mg/day) and thoropathic plasma exchange were given as treatment for the attack, and oral steroids, azathioprine, rituximab and cyclophosphamide were given as prophylactic treatment.

Conclusion: NMOSH has a more severe prognosis than MS. It is most frequently associated with SS among autoimmune diseases. It has been observed that patients with MOG IgG antibody positivity have better response to attack and prophylactic treatment than patients with AQP-4 IgG antibody positivity.

Keywords

aquaporin 4, myelin oligodentrocyte glycoprotein, neuromyelitis optica, optic neuritis, transverse myelitis

Project Number

References

1. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53(5): 1107-1114.
2. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 2005;202(4):473-477.
3. Sellner J, Boggild M, Clanet M, et al. EFNS guidelines on diagnosis and management of neuromyelitis. Eur J Neurol. 2010;17(8):1019-1032.
4. Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364(9451):2106-2112.
5. Lee DH, Linker RA. The role of myelin oligodendrocyte glycoprotein in autoimmune demyelination: a target for multiple sclerosis therapy? Expert Opin Ther Targets. 2012;16(5):451-462.
6. Hamid SH, Whittam D, Mutch K, Linaker S, Solomon T, Das K. What proportion of AQP4- IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients. J Neurol. 2017;264(10):2088-2094.
7. Wingerchuk DM. Diagnosis and Treatment of Neuromyelitis Optica. The Neurologist. 2007;13(1):2-11.
8. Wingerchuk DM, Pittock SJ, Lennon VA, et al. Neuromyelitis optica diagnostic criteria revisited: validation and incorporation of the NMOIgG serum autoantibody. Neurology. 2006;66(10):1485-1489.
9. Trebst C, Jarius S, Berthele A, et al. Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS). J Neurol. 2014;261:11-16.
10. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6(9):805-815.
11. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2): 177-189.
12. Sahraian MA, Radue EW, Minagar A. Neuromyelitis Optica Clinical Manifestations and Neuroimaging Features. Neurol Clin. 2013;31(1):139-152.
13. Sato, DK, Callegaro D, Lana-Peixoto MA, et al. Distinction between MOG antibody positive and AQP4 antibody-positive NMO spectrum disorders. Neurology. 2014;82(6):474-481.
14. Ambrosius W, Michalak S, Kozubski W, Kalinowska A. Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Current Insights into the Disease Pathophysiology, Diagnosis and Management. Int. J. Mol Sci. 2021;22(1):100.
15. Brilot F, Dale RC, Selter RC, et al. Antibodies to Native Myelin Oligodendrocyte Glycoprotein in Children with Inflammatory Demyelinating Central Nervous System Disease. Ann. Neurol. 2009;66(6):833-842.
16. Jurynczyk M, Messina S, Woodhall MR, et al. Clinical Presentation and Prognosis in MOG-Antibody Disease: A UK Study. Brain. 2017;140(12):3128-3138.
17. Armangue T, Olivé-Cirera G, Martínez-Hernandez E, et al. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: A multicentre observational study. Lancet Neurol. 2020;19(3):234-246.
18. Cobo-Calvo A. Ruiz A, Maillart E, et al. Clinical Spectrum and Prognostic Value of CNS MOG Autoimmunity in Adults: The MOGADOR Study. Neurology. 2018;90(21):e1858-e1869.
19. Ciotti J, Eby N, Wu G, Naismith R, Chahin S, Cross A. Clinical and laboratory features distinguishing MOG antibody disease from multiple sclerosis and AQP4 antibody-positive neuromyelitis optica. Mult. Scler. Relat. Disord. 2020;45:102399.
20. Netravathi M, Venkappayya Holla V, Nalini A, et al. Myelin oligodendrocyte glycoprotein-antibody-associated disorder: A new inflammatory CNS demyelinating disorder. J. Neurol. 2021;269(4):1419-1433.
21. Jarius S, Ruprecht K, Kleiter I, et al. MOG-IgG in NMO and Related Disorders: A Multicenter Study of 50 Patients. Part 2: Epidemiology, Clinical Presentation, Radiological and Laboratory Features, Treatment Responses, and Long-Term Outcome. J. Neuroinflamm. 2016;13(1):1-45.
22. Akman Demir G. Nöromiyelitis optika: Klinik ve İmmünolojik Özellikler. Türkiye Klinikleri J Neurol-Special Topics. 2009;2(4):37-40.
23. Dutra BG, da Rocha AJ, Nunes RH, Junior AC. Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. Radiographics. 2018;38(1):169-193.
24. Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG. Brain abnormalities in neuromyelitis optica. Arch Neurol. 2006;63(3):390-396.
25. Lana-Peixoto M. Talim N. Neuromyelitis optica spectrum disorder and anti-mog syndromes. Biomedicines. 2019;7(2):42.
26. Ringelstein M, Kleiter I, Ayzenberg I, et al. 2013 Visual evoked potentials in neuromyelitis optica and its spectrum disorders. Mult Scler. 2014;20(5):617-620.
27. Wandinger KP, Stangel M, Witte T, et al. Autoantibodies against aquaporin-4 in patients with neuropsychiatric systemic lupus erythematosus and primary Sjögren’s syndrome. Arthritis Rheum. 2010;62(4):1198-1200.
28. Önerli Yener M. Sjögren sendromunda nörolojik manifestasyonlar. Öztekin ZN. Romatolojik Hastalıkların Nörolojik Yönleri. 1. Baskı. Ankara: Türkiye Klinikleri; 2021;48-54.
29. Wingerchuk DM, Weinshenker BG. Neuromyelitis optica: clinical predictors of a relapsing course and survival. Neurology. 2003;60(5):848-853.
30. Magraner MJ, Coret F, Casanova B. The effect of intravenous immunoglobulin on neuromyelitis optica. Neurologia. 2013;28(2):65-72.
31. Chihara N, Aranami T, Sato W, et al. Interleukin 6 signaling promotes anti-aquaporin 4 autoantibody production from plasmablasts in neuromyelitis optica. Proc Natl Acad Sci USA. 2011;108(9):3701-3706.
32. Araki M, Aranami T, Matsuoka T, Nakamura M, Miyake S, Yamamura T. Clinical improvement in a patient with neuromyelitis optica following therapy with the anti-IL-6 receptor monoclonal antibody tocilizumab. Mod Rheumatol. 2013;23:827-832.
33. Kieseier BC, Stu O, Dehmel T, et al. Disease amelioration with tocilizumab in a treatment-resistant patient with neuromyelitis optica: implication for cellular immune responses. JAMA Neurol. 2012;70(3):390-393.
34. Fitzgerald S. Three new treatment options for neuromyelitis optica spectrum disorder. Neurology Today. 2019;19(11):7-10.

Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri

Year 2024, Volume: 8 Issue: 1, 94 - 103, 29.04.2024

Melike Doğan Ünlü Serpil Demirci

https://doi.org/10.46332/aemj.1303107

Abstract

Amaç: Nöromiyelitis Optika Spektrum Hastalığı (NMOSH) tanısı alan ve antikor pozitifliği eşlik eden hastaların klinik, görüntüleme ve laboratuvar bulgularını sunmayı amaçladık.

Araçlar ve Yöntem: Bu retrospektif gözlemsel çalışmaya NMOSH tanısı alan 15 hasta dahil edildi.

Bulgular: Hastalarımızın 11’i kadın, 4’ü erkekti. Hastalığın ortalama başlangıç yaşı 51.27±12.26 idi. İlk klinik atak 3 hastada eş zamanlı optik nörit (ON) ve miyelit iken, 6 hastada miyelit, 5 hastada ON idi. On hastanın aquaporin-4 (AQP-4) IgG antikoru, 5 hastanın miyelin oligodentrosit glikoprotein (MOG) IgG antikoru pozitifti. Altı hastanın beyin omirilik sıvısı (BOS) proteini yüksekti. Sekiz hastanın BOS oligoklonal bantı Tip 1 negatif, 1 hastanın Tip 2 pozitif, 1 hastanın Tip 3 pozitifti. Sekiz hastanın kranial manyetik rezonans görüntülemesi normal iken 1 hastanın multipl skleroz (MS)’a benzer lezyonları vardı. İki hastanın önceden bilinen Sjögren Sendromu (SS) tanısı vardı, 3 hastaya da kliniğimizde SS tanısı koyuldu. Atak tedavisi olarak intravenöz metilprednizolon (1000 mg/gün) ve töropatik plazma değişimi, profilaktik tedavi olarak oral steroid, azatioprin, ritüksimab ve siklofosfamid tekli veya kombinasyon tedavileri verildi.

Sonuç: NMOSH’ı prognoz olarak MS’den daha ağır seyretmektedir. Otoimmün hastalıklardan en sık SS ile birliktelik gösterir. MOG IgG antikor pozitifliği olanların AQP-4 IgG antikor pozitifliği olanlara göre atak ve profilaktik tedaviye yanıtları daha iyi olduğu görülmüştür.

Keywords

aquaporin 4, miyelin oligodentrosit glikoprotein, nöromiyelitis optika, optik nörit, transvers miyelit

Supporting Institution

yoktur

Project Number

Thanks

Çalışmada yer alan hastalarımıza teşekkür ederiz.

References

1. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53(5): 1107-1114.
2. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 2005;202(4):473-477.
3. Sellner J, Boggild M, Clanet M, et al. EFNS guidelines on diagnosis and management of neuromyelitis. Eur J Neurol. 2010;17(8):1019-1032.
4. Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364(9451):2106-2112.
5. Lee DH, Linker RA. The role of myelin oligodendrocyte glycoprotein in autoimmune demyelination: a target for multiple sclerosis therapy? Expert Opin Ther Targets. 2012;16(5):451-462.
6. Hamid SH, Whittam D, Mutch K, Linaker S, Solomon T, Das K. What proportion of AQP4- IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients. J Neurol. 2017;264(10):2088-2094.
7. Wingerchuk DM. Diagnosis and Treatment of Neuromyelitis Optica. The Neurologist. 2007;13(1):2-11.
8. Wingerchuk DM, Pittock SJ, Lennon VA, et al. Neuromyelitis optica diagnostic criteria revisited: validation and incorporation of the NMOIgG serum autoantibody. Neurology. 2006;66(10):1485-1489.
9. Trebst C, Jarius S, Berthele A, et al. Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS). J Neurol. 2014;261:11-16.
10. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6(9):805-815.
11. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2): 177-189.
12. Sahraian MA, Radue EW, Minagar A. Neuromyelitis Optica Clinical Manifestations and Neuroimaging Features. Neurol Clin. 2013;31(1):139-152.
13. Sato, DK, Callegaro D, Lana-Peixoto MA, et al. Distinction between MOG antibody positive and AQP4 antibody-positive NMO spectrum disorders. Neurology. 2014;82(6):474-481.
14. Ambrosius W, Michalak S, Kozubski W, Kalinowska A. Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Current Insights into the Disease Pathophysiology, Diagnosis and Management. Int. J. Mol Sci. 2021;22(1):100.
15. Brilot F, Dale RC, Selter RC, et al. Antibodies to Native Myelin Oligodendrocyte Glycoprotein in Children with Inflammatory Demyelinating Central Nervous System Disease. Ann. Neurol. 2009;66(6):833-842.
16. Jurynczyk M, Messina S, Woodhall MR, et al. Clinical Presentation and Prognosis in MOG-Antibody Disease: A UK Study. Brain. 2017;140(12):3128-3138.
17. Armangue T, Olivé-Cirera G, Martínez-Hernandez E, et al. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: A multicentre observational study. Lancet Neurol. 2020;19(3):234-246.
18. Cobo-Calvo A. Ruiz A, Maillart E, et al. Clinical Spectrum and Prognostic Value of CNS MOG Autoimmunity in Adults: The MOGADOR Study. Neurology. 2018;90(21):e1858-e1869.
19. Ciotti J, Eby N, Wu G, Naismith R, Chahin S, Cross A. Clinical and laboratory features distinguishing MOG antibody disease from multiple sclerosis and AQP4 antibody-positive neuromyelitis optica. Mult. Scler. Relat. Disord. 2020;45:102399.
20. Netravathi M, Venkappayya Holla V, Nalini A, et al. Myelin oligodendrocyte glycoprotein-antibody-associated disorder: A new inflammatory CNS demyelinating disorder. J. Neurol. 2021;269(4):1419-1433.
21. Jarius S, Ruprecht K, Kleiter I, et al. MOG-IgG in NMO and Related Disorders: A Multicenter Study of 50 Patients. Part 2: Epidemiology, Clinical Presentation, Radiological and Laboratory Features, Treatment Responses, and Long-Term Outcome. J. Neuroinflamm. 2016;13(1):1-45.
22. Akman Demir G. Nöromiyelitis optika: Klinik ve İmmünolojik Özellikler. Türkiye Klinikleri J Neurol-Special Topics. 2009;2(4):37-40.
23. Dutra BG, da Rocha AJ, Nunes RH, Junior AC. Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. Radiographics. 2018;38(1):169-193.
24. Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG. Brain abnormalities in neuromyelitis optica. Arch Neurol. 2006;63(3):390-396.
25. Lana-Peixoto M. Talim N. Neuromyelitis optica spectrum disorder and anti-mog syndromes. Biomedicines. 2019;7(2):42.
26. Ringelstein M, Kleiter I, Ayzenberg I, et al. 2013 Visual evoked potentials in neuromyelitis optica and its spectrum disorders. Mult Scler. 2014;20(5):617-620.
27. Wandinger KP, Stangel M, Witte T, et al. Autoantibodies against aquaporin-4 in patients with neuropsychiatric systemic lupus erythematosus and primary Sjögren’s syndrome. Arthritis Rheum. 2010;62(4):1198-1200.
28. Önerli Yener M. Sjögren sendromunda nörolojik manifestasyonlar. Öztekin ZN. Romatolojik Hastalıkların Nörolojik Yönleri. 1. Baskı. Ankara: Türkiye Klinikleri; 2021;48-54.
29. Wingerchuk DM, Weinshenker BG. Neuromyelitis optica: clinical predictors of a relapsing course and survival. Neurology. 2003;60(5):848-853.
30. Magraner MJ, Coret F, Casanova B. The effect of intravenous immunoglobulin on neuromyelitis optica. Neurologia. 2013;28(2):65-72.
31. Chihara N, Aranami T, Sato W, et al. Interleukin 6 signaling promotes anti-aquaporin 4 autoantibody production from plasmablasts in neuromyelitis optica. Proc Natl Acad Sci USA. 2011;108(9):3701-3706.
32. Araki M, Aranami T, Matsuoka T, Nakamura M, Miyake S, Yamamura T. Clinical improvement in a patient with neuromyelitis optica following therapy with the anti-IL-6 receptor monoclonal antibody tocilizumab. Mod Rheumatol. 2013;23:827-832.
33. Kieseier BC, Stu O, Dehmel T, et al. Disease amelioration with tocilizumab in a treatment-resistant patient with neuromyelitis optica: implication for cellular immune responses. JAMA Neurol. 2012;70(3):390-393.
34. Fitzgerald S. Three new treatment options for neuromyelitis optica spectrum disorder. Neurology Today. 2019;19(11):7-10.

There are 34 citations in total.

Details

Primary Language	Turkish
Subjects	Clinical Sciences
Journal Section	Original Articles
Authors	Melike Doğan Ünlü 0000-0002-4424-044X Serpil Demirci 0000-0003-1561-1296
Project Number	-
Early Pub Date	April 5, 2024
Publication Date	April 29, 2024
Published in Issue	Year 2024 Volume: 8 Issue: 1

Cite

APA	Doğan Ünlü, M., & Demirci, S. (2024). Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri. Ahi Evran Medical Journal, 8(1), 94-103. https://doi.org/10.46332/aemj.1303107
AMA	Doğan Ünlü M, Demirci S. Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri. Ahi Evran Med J. April 2024;8(1):94-103. doi:10.46332/aemj.1303107
Chicago	Doğan Ünlü, Melike, and Serpil Demirci. “Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik Ve Radyolojik Özellikleri”. Ahi Evran Medical Journal 8, no. 1 (April 2024): 94-103. https://doi.org/10.46332/aemj.1303107.
EndNote	Doğan Ünlü M, Demirci S (April 1, 2024) Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri. Ahi Evran Medical Journal 8 1 94–103.
IEEE	M. Doğan Ünlü and S. Demirci, “Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri”, Ahi Evran Med J, vol. 8, no. 1, pp. 94–103, 2024, doi: 10.46332/aemj.1303107.
ISNAD	Doğan Ünlü, Melike - Demirci, Serpil. “Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik Ve Radyolojik Özellikleri”. Ahi Evran Medical Journal 8/1 (April 2024), 94-103. https://doi.org/10.46332/aemj.1303107.
JAMA	Doğan Ünlü M, Demirci S. Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri. Ahi Evran Med J. 2024;8:94–103.
MLA	Doğan Ünlü, Melike and Serpil Demirci. “Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik Ve Radyolojik Özellikleri”. Ahi Evran Medical Journal, vol. 8, no. 1, 2024, pp. 94-103, doi:10.46332/aemj.1303107.
Vancouver	Doğan Ünlü M, Demirci S. Anti-Aquaporin 4 Ve Anti-Miyelin Oligodentrosit Glikoprotein Antikor İlişkili Nöromiyelitis Optika Spektrum Hastalığı Tanısı Alan Olguların Klinik ve Radyolojik Özellikleri. Ahi Evran Med J. 2024;8(1):94-103.

Download Cover Image

Article Files

Full Text

Ahi Evran Medical Journal is indexed in ULAKBIM TR Index, Turkish Medline, DOAJ, Index Copernicus, EBSCO and Turkey Citation Index. Ahi Evran Medical Journal is periodical scientific publication. Can not be cited without reference. Responsibility of the articles belong to the authors.

This journal is licensed under the Creative Commons Atıf-GayriTicari 4.0 Uluslararası Lisansı.