Schumacher G.A., Beebe G., Kibler R.F., Kurland L.T., Kurtzke J.F., McDowell F., Nagler B., Sibley W.A., Tourtellotte W.W., Willmon T.L. Lucchinetti, Department of Neurology, Mayo Clinic, Rochester, MN, USA Subfigure 5a,b: courtesy of dr Claudia F. Subfigures 1a,b, 2–4 and 6: from the collection of the Department of Neurology, Poznan University of Medical Sciences, Poznan, Poland. Abbreviations: MOGAD-MOG-antibody associated disease, ADEM-acute disseminated encephalomyelitis, FLAIR-Fluid Attenuated Inversion Recovery, NMOsd-neuromyelitis optica spectrum disorder, AQP4-aquaporin 4, LETM-longitudinally extensive transverse myelitis. The lesions are not visible on T1-weighted sequence and are non-enhancing. ( 6) MOGAD in a 34-year old female presenting with spastic paraparesis, which improved significantly after a 5-day intravenous methylprednisolone course: on a sagittal T2-weighted sequence multiple hyperintensive poorly demarcated lesions are visible throughout the spinal cord, including the cervical and thoracic regions, and affecting medullary conus, as well. On axial FLAIR images there are diffuse nonspecific white matter hyperintensities involving both cerebral hemispheres, corpus callosum ( 5a), posterior fossa ( 5b), but periventricular lesions are lacking. ( 5) MOGAD in a 47-year old male presenting with ADEM with confirmed MOG-ab seropositivity. ( 4) AQP4-ab positive NMOsd in a 43-year old female: on a sagittal T2-weighted sequence LETM is visualized from C1–C2 to C5.
( 3) Cortical involvement in a 22-year old female with MS: on axial FLAIR there is a 4 mm long linear hyperintensity that surrounds the cortical gyrus and involves both, the cortex and subcortical white matter. ( 2) Optic neuritis in a 26-year old female with AQP4-ab positive NMOsd: bilateral enhancement of the optic nerves on axial T1-weighted post-contrast sequence. On axial post-contrast T1-weighted image ( 1b) slight contrast enhancement is present in the latter. On axial FLAIR ( 1a), a periventricular lesion at the level of the left subcortical nuclei (17 × 11 mm) and the left ventricular triangle (15 × 10 mm) are visualized. ( 1) ADEM presentation in a 17-year old female with behavioral changes and transient right-sided paresis. Typical magnetic resonance findings in a spectrum of diseases overlapping with MOGAD.
NMO spectrum disorder myelin oligodendrocyte glycoprotein (MOG) myelin oligodendrocyte glycoprotein associated disease (MOGAD) neuroimmunology neuromyelitis optica (NMO). In this review, we present up-to-date clinical, immunological, radiographic, histopathological data concerning MOGAD and summarize the practical aspects of diagnosing and managing patients with this disease. Reports on randomised control trials are limited, but observational open-label experience suggests a role for high-dose steroids and plasma exchange in the treatment of acute attacks, and for immunosuppressive therapies, such as steroids, oral immunosuppressants and rituximab as maintenance treatment. MRI imaging can also help in differentiating MOGAD from other neuro-inflammatory disorders. A critical element of reliable diagnosis is detection of pathogenic serum antibodies MOG with accurate, specific and sensitive methods, preferably with optimized cell-based assay (CBA). Luckily, acute immunotherapy is very effective and severe disability (ambulatory and visual) is less frequent than in NMOSD. In children ADEM usually presents as a one-time incident. In adults, the disease course is multiphasic and subsequent relapses increase disability. Optic neuritis seems to be the most frequent presenting phenotype in adults and ADEM in children. The specific outmost location of myelin makes it a potential target for autoimmune antibodies and cell-mediated responses in demyelinating processes. Its function is not fully understood but this glycoprotein may act as a cell surface receptor or cell adhesion molecule. MOG is a molecule detected on the outer membrane of myelin sheaths and expressed primarily within the brain, spinal cord and also the optic nerves. Even though sometimes the clinical picture of this condition is similar to the presentation of neuromyelitis optica spectrum disorder (NMOSD), most experts consider MOGAD as a distinct entity with different immune system pathology. Myelin oligodendrocyte glycoprotein (MOG)-associated disease (MOGAD) is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system (CNS) with various phenotypes starting from optic neuritis, via transverse myelitis to acute demyelinating encephalomyelitis (ADEM) and cortical encephalitis.
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