Clinical and Radiological Characteristics of Patients with Anti-Aquaporin 4 and Anti-Myelin Oligodentrocyte Glycoprotein Antibody-Associated Neuromyelitis Optica Spectrum Disease

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