![]() There was demyelination of the optic nerves, but a gross examination of the brain was unrevealing. Autopsy disclosed severe demyelinating and necrotic lesions extending 4–5 cm length in the lower thoracic and lumbar spinal cord. One month later, she developed urinary retention, complete paraplegia and blindness, and died few weeks later. Devic reported the case of a 45-year-old French woman who was seen at the Hôtel-Dieu hospital of Lyon because of an intractable headache and depression in addition to general asthenia. The term neuromyelitis optica was introduced by Eugène Devic and Fernand Gault in 1894, who first recognized the association of amaurosis and myelitis as a new clinical entity. In line with that, N-methyl-D-aspartate receptor-IgG and CV2/CRMP5-IgG have been described in association with NMOSD phenotype. On the other hand, failure to identify either AQP4-IgG or MOG-IgG in a proportion of patients with NMOSD phenotype supports the view that other autoantibodies or factors may also play a role in NMOSD pathogenesis. The lack of coexistence of AQP4-IgG and MOG-IgG in the serum of a same patient further suggests that AQP4-IgG NMOSD and anti-MOG syndromes are distinct diseases. Moreover, three fourths of NMOSD patients test positive for AQP4 antibody, while serum MOG antibody is only detected in a minority of seronegative AQP4-IgG NMOSD patients. Because of this particular location, MOG is a good antigen candidate for autoimmune demyelination. Whereas NMOSD is most frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, particularly expressed in the astrocytic processes at the blood-brain barrier (BBB), anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), a membrane protein expressed on oligodendrocyte cell surfaces and on the outermost surface of myelin sheaths. Īlthough NMOSD and anti-MOG syndromes share a number of clinical manifestations, they are independent nosological entities with distinct pathophysiological mechanisms and histopathological features. Even a more important reason is the observation that some agents used in MS treatment may be deleterious to patients with NMOSD. Early and accurate diagnosis of these distinct conditions is very relevant as they have different therapeutic approaches. Because of their clinical manifestations and habitual relapsing course, they are frequently confounded with multiple sclerosis (MS). 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. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. 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). ![]() Some agents used for MS treatment may be deleterious to NMOSD. ![]() Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). 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. ![]()
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