Abstract
Neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system that frequently involve the optic nerves and the spinal cord. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Some agents used for MS treatment may be deleterious to NMOSD. NMOSD is frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, located in the astrocytic processes at the blood-brain barrier (BBB). On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization.
Highlights
Neuromyelitis optica spectrum disorders (NMOSD) and anti-myelin oligodendrocyte glycoprotein syndromes are immune-mediated inflammatory conditions of the central nervous system (CNS) that frequently involve the optic nerves and the spinal cord
Whereas NMOSD is most frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, expressed in the astrocytic processes at the blood-brain barrier (BBB) [8], anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), a membrane protein expressed on oligodendrocyte cell surfaces and on the Biomedicines 2019, 7, 42; doi:10.3390/biomedicines7020042
Histopathological features such as a marked loss of astrocytes and accumulation of IgG and IgM around blood vessels, the site of AQP4 expression; spare of myelin and axons in some lesions suggesting that astrocytes are the initial cell target in the disease, whereas in more recent lesions there may be preservation of glial fibrillary acidic protein (GFAP), a marker of astrocyte damage, suggesting that AQP4 is the primary target of the immune attack
Summary
Neuromyelitis optica spectrum disorders (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system (CNS) that frequently involve the optic nerves and the spinal cord. Because of their clinical manifestations and habitual relapsing course, they are frequently confounded with multiple sclerosis (MS). Whereas NMOSD is most frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, expressed in the astrocytic processes at the blood-brain barrier (BBB) [8], anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), a membrane protein expressed on oligodendrocyte cell surfaces and on the Biomedicines 2019, 7, 42; doi:10.3390/biomedicines7020042 www.mdpi.com/journal/biomedicines. N-methyl-D-aspartate receptor-IgG and CV2/CRMP5-IgG have been described in association with NMOSD phenotype [16,17,18,19]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
