Many patients with NMOSD are misdiagnosed as having MS. Some patients may present with acute disseminated encephalomyelitis (ADEM). Neuromyelitis optica (NMO), sometimes called Devic disease or opticospinal multiple sclerosis (MS) is a severe, relapsing, autoimmune, inflammatory and demyelinating central nervous system disease (IDD) that predominantly affects optic nerves and spinal cord.(1) The disorder is now recognized as a spectrum of autoimmunity (termed NMO spectrum disorders: NMOSD).(1-3) Brain lesions are observed in more than 60% of patients with NMOSD and approximately 10% will be MS-like.(4) Children tend to have greater brain involvement than adults, and brain lesions are more symptomatic than is typical for adult patients.(3) The clinical course is characterized by relapses of optic neuritis or transverse myelitis, or both. Seropositivity for MOG-IgG in the setting of a severe relapse of central nervous system (CNS) demyelination warrants aggressive therapy with intravenous methylprednisolone or plasmapheresis. Seropositivity for MOG-IgG, in NMOSD like disorders, including optic neuritis (ON), transverse myelitis (TM), and acute disseminated encephalomyelitis (ADEM), predicts relapse and warrants consideration for maintenance immunosuppression. MS therapies may worsen MOG-IgG associated IDDs, so correct diagnosis is important. Detection of MOG-IgG implies an inflammatory demyelinating disorder distinct from MS. Patients seropositive for MOG-IgG are commonly misdiagnosed as MS. There is currently no biomarker specific for MS (multiple sclerosis). Patients only rarely harbor both antibodies. Seropositivity predicts a relapsing phenotype and warrants immunosuppressive therapy. Of the remaining 20%, one-third harbor MOG-IgG. Myelin oligodendrocyte glycoprotein (MOG)-IgG with an NMO spectrum disorder like phenotype is now recognized as a sensitive and specific diagnostic antibody biomarker of inflammatory demyelinating disorders (IDDs).Īpproximately 80% of patients fulfilling 2006 Wingerchuk criteria for neuromyelitis optica are seropositive for aquaporin-4 (AQP4)-IgG.
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