Neuromyelitis optica spectrum disorder diagnostic-therapeutic update. Systematic review

Abstract

Neuromyelitis Optica spectrum disorder (NMOSD) is a rare pathology characterized by recurrent inflammatory crises of the central nervous system focused on the level of the spinal cord and the optic chiasm. This disease can alter other regions of the CNS and its pathophysiology is immunologic and heterogeneous, which makes us think of a spectrum of the disease. It is believed that NMOSD is caused by the presence of two classes of antibodies called: Anti-aquaporin 4 NMO IgG and Anti-MOG-IgG, the disease can present with one of the antibodies or with both, in this last case the severity of the disease increases exponentially. The pathophysiology of this condition is not completely clear since it is known that the mentioned antibodies trigger inflammatory processes and demyelination of astrocytic cells, but it is not known by what mechanisms they do it. However, MRI studies can adequately delimit the lesions in the areas mentioned earlier and immunohistochemically localize the antibodies. The initial clinical picture is characterized by simultaneous involvement of both the optic chiasm and the medulla in the early stage of the disease, although in most cases there is a variable period of months and years to evidence lesions in the involved structures. The manifestations are severe and are characterized by a progressive decrease in a vision leading to blindness and total flaccid paraplegia. The prognosis of this disease is not encouraging, since in 18% of cases sufferers lose sight in both eyes; irreversible motor disability occurs in 34% of patients, and in 23% of cases the use of a wheelchair is permanent. This disease has a mortality rate of 9%. We conducted a systematic review of the therapeutic and diagnostic advances in the pathology of neuromyelitis optica using complete, updated articles written in the last 5 years, which will be obtained from digital databases such as PubMed, Scopus, Chrocane Library, UpToDate, and Scielo.