| Abstract [eng] |
Neuromyelitis optica spectrum disorders are autoimmune inflammatory disorders of the central nervous system, leading to secondary demyelination and neurodegeneration. Due to their clinical similarity to multiple sclerosis, they are often undiagnosed and inadequately treated. The main pathogenetic factor is the interaction of the water channels of the astrocyte plasma membrane, aquaporins 4, with antibodies specific to them. The clinical presentation of the disease is highly variable and depends on the localization of the lesions. The hallmarks of this disorder are acute attacks of optic neuritis, causing bilateral and irreversible damage, longitudinal transverse myelitis, and a relapsing-remitting clinical course. Optic neuromyelitis spectrum disorders are diagnosed on the basis of clinical signs, specific MRI findings, and serum aquaporin-4 antibody detection. Intravenous glucocorticoid pulse therapy, plasmapheresis, and immunoglobulins are used for the treatment of acute episodes. Immunosuppressive drugs (azathioprine and mycophenolate mofetil), which have many side effects, or biological therapies (rituximab, satralizumab, eculizumab, inebilizumab, tocilizumab), which have a better outcome, are used for the prevention of exacerbations and progression of the disease. Optic neuromyelitis spectrum disorders are characterized by frequent relapses and rapid progression of neurological disability, with a mortality rate of 20- 25% in the first ten years after diagnosis if untreated. This paper presents a clinical case illustrating the clinical course and treatment of Optic Neuromyelitis Spectrum Disorder and reviews the literature on the pathogenetic features, clinical course, and treatment of this disorder. |
