NEUROMYELITIS OPTICA AND DIFFERENTIAL DIAGNOSIS OF NEUROMYELITIS OPTICA

Optički neuromijelitis (NMO, od engl. neuromyelitis optica) je autoimuna demijelinizacijska bolest središnjeg živčanog sustava koja zahvaća uglavnom očne živce i kralježničnu moždinu. Više rasprave o bolesti pokrenuto je 2004. godine kada su otkrivena ciljna protutijela, tzv. anti-akvaporinska protutijela (AQP4-IgG) koja imaju glavnu ulogu u samom mehanizmu nastanka. Za postavljanje dijagnoze potrebno je zadovoljiti dva glavna kriterija i dva od tri suportivna kriterija. Dva glavna kriterija uključuju: optički neuritis i mijelitis, a suportivni kriteriji uključuju: MR mozga koji ne ispunjava kriterije za dijagnozu multiple skleroze, prisutnost seropozitivnih AQP4-IgG (NMO-IgG) protutijela, longitudinalno ekstenzivni transverzalni mijelitis (LETM) u T2 vremenu na MR. Kako određena skupina pacijenata nije upotpunjavala sve kriterije za postavljanje dijagnoze NMO, 2007. godine uveden je pojam tzv. NMO-spektar poremećaja (NMOSD od engl. neuromyelitis optica spectrum disorder). Godine 2015. pokušalo se ujediniti termin NMO i NMOSD te su donijeti kriteriji za postavljanje dijagnoze NMOSD. Terapija koja se primjenjuje uglavnom je orijentirana na akutno zbrinjavanje simptoma dok je cilj dugoročne terapije sprječavanje egzacerbacija bolesti i novih relapsa. U radu je prikazana defi nicija, dijagnostika, diferencijalna dijagnostika te liječenje NMO/NMOSD.Introduction: Neuromyelitis optica (NMO) is an autoimmune demyelinating disease of the central nervous system that mainly affects optic nerve and spinal cord. The disease can be clinically presented similar to multiple sclerosis. Even though the disease was described in the late 19th century, discussion about the disease was initiated in 2004 when antibodies against aquaporin (AQP4-IgG) were discovered. They play the main role in the mechanism of the disease. Aim: Knowing that NMO can be presented similar to multiple sclerosis, our aim was to collect and review data about NMO and differential diagnosis of NMO. Methods: We performed MEDLINE search for NMO and included literature dating back from the year 2010. We also explored the Croatian scientifi c database Hrčak. Results: We found 334 review articles by exploring MEDLINE but chose only the most systemized ones. On Hrčak, we found 3 articles in Croatian language. Discussion: The prevalence of the disease is 1-4.4/100 000 in Western countries. In some Asian countries, NMO accounts for 50% of demyelinating disease diagnoses. Searching through the literature, NMO was found to be more common in women as compared with men (9:1 ratio). The disease can be associated with other autoimmune diseases such as systemic lupus erythematosus, sarcoidosis and Sjögren syndrome. The disease can be clinically presented by optic neuritis, symptoms of transverse myelitis, nausea, and other brain stem symptoms. At the beginning of defi ning criteria for diagnosing NMO, there were two main criteria and supportive criteria. The two main diagnostic criteria included optic neuritis and myelitis. Supportive criteria included brain magnetic resonance imaging (MRI) not meeting the criteria for multiple sclerosis (MS) diagnosis positive AQP4-IgG antibody and longitudinally extensive transverse myelitis (LETM) on T2-weighted imaging on MRI. A group of patients did not meet the criteria for NMO diagnosis, so in 2007 the term NMO spectrum disorder (NMOSD) was introduced. In 2015, there was a tendency to unite the NMO and NMOSD terms; so diagnostic criteria for NMOSD were developed by Wingerchuk et al. The criteria were extended to those with AQP4-IgG positive and AQP4-IgG negative or unknown status. In the article, were also mention the so called ‘red fl ags’ that help in differential diagnosing of NMO/NMOSD. Treatment is still limited to treating acute exacerbations by methylprednisolone or plasmapheresis. Long-term therapy is focused on reducing the frequency and intensity of exacerbations. Conclusions: Even though NMO is more common in some Asian countries, it can also occur in other countries. Clinically, it is similar to MS but treatment is different. There are some data that medications used in the treatment of MS can worsen the course of the disease in NMO. That is why it is important to make a correct diagnosis