Consensus Recommendations for the Diagnosis and Treatment of Neuromyelitis Optica Spectrum Disorders (NMOSD): The MENACTRIMS Guidelines
摘要
Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune disorder affecting the central nervous system, often misdiagnosed as multiple sclerosis. The identification of aquaporin-4–IgG (AQP4-IgG) has improved diagnostic precision and enabled targeted therapies. Given the unique regional challenges in healthcare delivery across the Middle East and North Africa (MENA) region, the Middle East and North Africa Committee for Treatment and Research in Multiple Sclerosis (MENACTRIMS) convened an expert panel to develop evidence-based, region-specific consensus recommendations for diagnosis and management. These guidelines endorse the 2015 International Panel for NMO Diagnosis (IPND) criteria, emphasizing AQP4-IgG testing via cell-based assays. Differential diagnosis should consider multiple sclerosis, myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and acute disseminated encephalomyelitis (ADEM). For acute treatment: initiate high-dose intravenous methylprednisolone promptly and use plasma exchange early for severe or steroid-refractory attacks. For long-term immunotherapy, monoclonal antibodies (rituximab, inebilizumab, eculizumab, ravulizumab, satralizumab, or tocilizumab) are recommended according to availability and patient factors; conventional immunosuppressants remain alternatives when biologics are inaccessible. Guidance is provided for pediatric patients and for pregnancy and breastfeeding, including planning after ≥ 12 months of disease stability and early postpartum treatment resumption. These MENACTRIMS guidelines aim to improve NMOSD outcomes across the region by promoting accurate diagnosis and timely, effective therapy.