Prompt and accurate diagnosis and treatment of NMOSD aid in restoring patients’ visual and neurological functions in the acute stage of the disease. The novel strategies include the administration of biologics and monoclonal antibodies.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare chronic autoimmune disorder primarily based on the involvement of the optic nerves and spinal cord, resulting in the development of optic neuritis and transverse myelitis, respectively. The pathogenesis of this disease is based on the development of antibodies against aquaporin 4 (AQP4), a water channel protein found in the central nervous system (CNS) and other areas.
This study describes conventional treatment strategies and emergent therapeutic options for NMOSD. The study is published in the Taiwan Journal of Ophthalmology.
Diagnosis of Neuromyelitis Optical Spectrum Disorder and Optic Neuritis
The diagnosis of NMOSD is based on the presenting patients’ clinical findings, laboratory investigations, and imaging studies. Common clinical features include transverse myelitis and optic neuritis. The laboratory tests for NMOSD diagnosis include testing for antibodies against AQP4. Magnetic resonance imaging (MRI) aids in the confirmation of the diagnosis. The clinical presentation of optic neuritis in NMOSD patients includes acute and unilateral/bilateral vision loss.
Management of Acute Neuromyelitis Optica Spectrum Disorder Attacks
The management of acute NMOSD attacks includes the administration of high-dose 1000 mg intravenous methylprednisolone for 3–5 days. Early treatment aids in better clinical outcomes and reduces the loss of the retinal nerve fiber layer. In refractory cases, plasma exchange with or without steroids can be administered as escalation therapy. Intravenous immunoglobulin is also considered in acute NMOSD treatment.
Maintenance Therapy
The conventional treatment therapies include rituximab, mycophenolate mofetil, and azathioprine. Low-dose corticosteroids are also implicated in the reduction of NMOSD relapses. The administration of immunosuppressants and rituximab can achieve a significant reduction in the risk of relapse. The monoclonal antibodies approved for the treatment of NMOSD include satralizumab, eculizumab, and inebilizumab.
Emergent Therapies
Patients refractory to the treatment of NMOSD pose a challenge for therapeutic strategies. One of the more compelling therapeutic strategies for NMOSD patients is restoring immune tolerance. The potential therapeutic options include autologous hematopoietic stem cell transplantation, regulatory T cells, a DNA vaccine for myelin basic protein, and peptide-loaded tolerogenic dendritic cells. Other therapeutic targets include the complement cascade, B cells, the blood–brain barrier, and granulocytes. Monoclonal anti-AQP4 antibodies are also implicated in the treatment of NMOSD.
Prompt and accurate diagnosis and treatment of NMOSD aid in restoring patients’ visual and neurological functions in the acute stage of the disease.
Source
Huang, T., & Chu, Y. (2022). What’s new in neuromyelitis optica spectrum disorder treatment? Taiwan Journal of Ophthalmology, 12(3), 249–263. https://doi.org/10.4103/2211-5056.355617
