![]() Signs of brainstem affection like persistent hiccup, nausea or vomiting should explicitly be asked for as they are often attributed to other reasons and are therefore not reported spontaneously by the patient. In NMOSD and MOG-EM, most common symptoms are optic neuritis and longitudinally extensive transverse myelitis (LETM). To find all relevant publications, we did not restrict the year of publication however, most reports originate from the last 5 years. We used the search terms “neuromyelitis optica,” “neuromyelitis optica spectrum disorder,” “MOG,” aquaporin-4 antibodies,” “MRI,” “diagnostic criteria,” “therapy,” and combinations of these. To give an overview on diagnosis and treatment recommendations in NMOSD and MOG-EM, we here describe our own clinical experiences and give a review on the current literature using the Pubmed online database. To date, the relevance of MOG-Abs and their nosologic categorization is a topic of current discussion and under further investigation ( 47, 52, 53). Although ADEM can also be accompanied by MOG-Abs ( 51), in this manuscript we do not regard MOG-Ab positive patients with ADEM-phenotype as part of the “MOG-EM” due to their distinct clinical characteristics. Hereafter, we use the term “MOG-EM,” as it reflects the relevant symptoms of the disease and is used in several recent publications, e.g., ( 49). Various terms are used to describe the disease such as “MOG-antibody related disorder,” “MOG-associated disease,” “MOG antibody disease,” “MONEM” or “MOG-encephalomyelitis (MOG-EM)”( 40, 47– 50). ![]() Previous studies on NMOSD might have included patients with MOG-Abs and therefore overlapping features could have been reported in these studies. Although there are numerous overlaps in clinical presentation and imaging findings with NMOSD with and without AQP4-Ab, MOG-Ab-associated disease is more and more considered a disease entity in its own ( 47). According to the revised 2015 NMOSD diagnostic criteria ( 46), diseases with or without evidence of AQP4-Abs as well as disorders with MOG-Abs can be assigned to the NMO spectrum. MOG is a glycoprotein localized on the surface of the myelin sheath as well as of the cell body and processes of oligodendrocytes ( 44, 45). In the past, MOG-Abs were particularly described in acute disseminated encephalomyelitis (ADEM), an inflammatory CNS disorder that, if it has an pediatric onset, is mostly monophasic and has a favorable outcome in the majority of cases ( 42, 43). Recently, various publications described the detection of serum-Abs against myelin-oligodendrocyte-glycoprotein (MOG) in AQP4-Ab negative NMOSD patients including pediatric cohorts and few patients with MS ( 29– 41). Whether AQP4-Ab positive and AQP4-Ab negative diseases are varieties of the same disorder or rather reflect different disease entities is a topic of ongoing research ( 26– 28). In 20–30% of patients, depending on the assay used, AQP4-Abs are not detectable ( 24, 25). Women are disproportionately more often affected and, particularly in AQP4-seropositive patients, female to male-ratio can reach up to 10:1 ( 19, 22, 23). Disease onset ranges between 4 and 88 years with a mean age at onset of 39 years ( 18– 21). Patients without long-term immunosuppressive therapy have a worse prognosis with a higher mortality rate ( 17). Thus, in relapsing NMOSD, which account for approximately 80–85% of cases, neurologic deficits frequently accumulate during the disease course. Despite treatment, recovery from attacks is often incomplete and disease remission rarely occurs ( 15, 16). Patients also frequently suffer from burdensome symptoms like pain, headache, depression, fatigue, and sleep disorders ( 10– 14). In rarer cases, brainstem and brain involvement e.g., area postrema syndrome or diencephalic syndrome can occur ( 8, 9). In the majority of patients with NMOSD, autoantibodies (Abs) against the astrocyte aquaporin-4 (AQP4) water channel are detectable and patients typically suffer from recurrent attacks of severe optic neuritis or/and myelitis ( 3– 7). The French term “neuro-myélite optique aiguë,” which may be translated as “neuromyelitis optica acuta” was first used by Devic in 1894 ( 1, 2). ![]() Neuromyelitis optica spectrum disorders (NMOSD) are rare chronic inflammatory central nervous system diseases distinct from multiple sclerosis (MS).
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