![]() 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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