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

Yıl 2024, Cilt: 8 Sayı: 1, 94 - 103, 29.04.2024

Melike Doğan Ünlü , Serpil Demirci

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

Öz

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.

Anahtar Kelimeler

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

Proje Numarası

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

Öz

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.

Anahtar Kelimeler

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

Destekleyen Kurum

yoktur

Proje Numarası

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Teşekkür

Çalışmada yer alan hastalarımıza teşekkür ederiz.

Kaynakça

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