International Medical Journal, Vol. 24., Iss. 4, 2018, P. 47−53.
MODERN DATA ON NEUROMYELITIS OPTICA, OR DEVIC’S DISEASE
Kharkiv Mediacl Academy of Postgraduate Education, Ukraine
Neuromyelitis optica (NMO) or Devic's disease is a chronic inflammatory demyelinating disease of the central nervous system characterized by selective involvement of optic nerve and spinal cord myelin in the pathological process, leading to severe disability. For a long time, the disease was considered as a variant of multiple sclerosis (MS) due to the similarity of symptoms. Detection of NMO−IgG antibodies (Neuromyelitis Optica, NMO) specific molecular biomarker aquaporin−4 (AQP4) in the blood allows to differentiate these two diseases. The purpose of the article is to expand and deepen doctors' knowledge in etiopathogenesis, pathomorphology, clinical course, diagnostic and treatment strategies of NMO, taking into account modern data. The disease is based on an autoimmune process with specific antibodies response on the periphery, its penetration through the blood brain barrier and protein aquaporin−4 (AQP4) binding, which leads to inflammation and demyelination, followed by necrosis of oligodendrocytes and neurons, its cystic degeneration. According to the new diagnostic criteria, the list of NMO Spectrum Disorder includes not only seropositive (with the presence of AQP4−IgG), but also seronegative cases. In patients without antibodies to AQP4, antibodies to myelin oligodendrogliocyte glycoprotein (MOG−IgG seropositive) are present. NMO has monophasic and recurrent flow types. NMO is clinically manifested by syndromes of optic neuritis and/or longitudinal−transverse myelitis. Diagnosing NMO is based on diagnostic criteria and data of neurological examination, neuroimaging, and laboratory (serological) tests. Treatment of NMO depends on stage and type of disease. The standard of care for the treatment of an acute attack is intravenous pulse therapy of corticosteroids (CS) and/or plasma exchange. For prevention of recurrence, the first−line drugs are azathioprine, mycophenolate mofetil and rituximab. Greater efficiency is shown by combined regimens of taking these drugs with low−dose CS. Intravenous immunoglobulin therapy is also possible. There are no clear recommendations regarding the duration of preventive treatment. The effect of complement inhibitors and neutrophil elastase, as well as AQP4−IgG binding blockers with AQP4, is being investigated. These include eculizumab, tocilizumab and a C1−esterase inhibitor. The outcomes of the disease vary from complete recovery and the presence of remission of the disease to the most unfavorable option with progressive deterioration and death of the patient.
Key words: neuromyelitis optica, Devic’s disease, aquaphorin−4, seropositive and seronegative cases, etiopathogenesis, pathomorphology, clinical findings, diagnostic criteria, treatment.