Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) are autoantibody mediated chronic inflammatory diseases. Serum antibodies (Abs) against the aquaporin-4 water channel lead to recurrent attacks of optic neuritis, myelitis and/or brainstem syndromes. In some patients with symptoms of NMOSD, no AQP4-Abs but Abs against myelin-oligodendrocyte-glycoprotein (MOG) are detectable. These clinical syndromes are now frequently referred to as “MOG-encephalomyelitis” (MOG-EM). Here we give an overview on current recommendations concerning diagnosis of NMOSD and MOG-EM. These include antibody and further laboratory testing, MR imaging and optical coherence tomography. We discuss therapeutic options of acute attacks as well as longterm immunosuppressive treatment, including azathioprine, rituximab, and immunoglobulins.

Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

“…Neuromyelitis optica (NMO, also known as Devic’s disease) is a rare chronic inflammatory autoimmune disorder of the central nervous system that preferentially targets the spinal cord and optic nerve, with rarer cases involving the brain stem and diencephalon . This severe, immune‐mediated disorder is characterized by longitudinally extensive transverse myelitis (LETM, myelitis extending three or more vertebral segments in length) and unilateral or bilateral optic neuritis.…”

Vitamin D and Demyelinating Diseases: Neuromyelitis Optica (NMO) and Multiple Sclerosis (MS)

“…NMO spectrum disorders present with fatigue, pain, depression, and sleep problems, painting a very similar clinical picture to MS [24]. Relapses and recovery however are much more severe in NMO relative to MS [22].…”

“…Treatment for NMO spectrum disorders and MS are both aimed at immunosuppression and still very experimental. Both utilize corticosteroids for acute exacerbations [24,25], but the MS disease modifying drugs (DMDs) such as interferons can worsen NMO [22]. Vitamin D serum levels of patients with MS have been of interest and supplementation has had mixed results as is discussed further below.…”

Autoantibody Diagnostics in Neuroimmunology: Experience From the 2018 Italian Neuroimmunology Association External Quality Assessment Program

“…Such autoantibodies preferentially bind antigens when their tertiary structure is preserved. This has revolutionized the neuroimmunology diagnostics, with the diffusion of "conformational" tests, such as cell-based assays (CBAs) and immunohistochemistry on lightly-fixed brain tissues for the diagnosis of autoimmune encephalitis (4), and for the differential diagnosis of the acquired demyelinating diseases of the CNS, including multiple sclerosis (5).…”