7/5/2023 0 Comments Anti mog syndromeWhy autoimmunity develops is largely unknown. The cause of the remaining cases is still unknown, and it is likely heterogeneous. In more than 80% of cases, IgG autoantibodies against aquaporin-4 ( anti-AQP4+) are the cause, and in 10–40% of the remaining cases, IgG antibodies against MOG are the cause. NMOSD is caused by an autoimmune attack on the nervous system. Lesions may also affect the diencephalon, mostly in Aquaporin-4– Immunoglobulin-G (AQP4-IgG) NMOSD. Additional brain lesions are common but often asymptomatic (though cognitive deficits, as well as depression, may be underdiagnosed sequalae). Lesions in the area postrema of the medulla oblongata can cause vomiting or hiccups, as well as pain and tonic spasms. Lesions in the brain stem or upper cervical spinal cord can cause respiratory insufficiency. Less commonly than the spinal cord and optic nerve, NMOSD can affect the brain stem. However, with the discovery of disease-causing antibodies, a broader spectrum of disease manifestations has been grouped with NMO into the diagnosis of NMOSD. Ĭlassically, NMO included only symptoms of myelitis and ON. Compared to idiopathic ON and ON due to multiple sclerosis (MS), ON due to NMOSD more often results in severe visual loss at onset, with bilateral involvement, and permanent visual deficits. ON may lead to varying degrees of visual impairment with decreased visual acuity, although visual field defects, or loss of color vision, may occur in isolation or prior to formal loss of visual acuity. The second most common initial manifestation of the disease is inflammation of the optic nerve and/or optic chiasm ( optic neuritis, ON). The myelitis can be transverse, affecting an entire cross-section of the spinal cord, and showing bilateral symptoms. Myelitis causes spinal cord dysfunction, which can result in muscle weakness, paralysis in the limbs, lost or reduced sensation, spasms, loss of bladder and bowel control, or erectile dysfunction. The most common initial manifestation of the disease is inflammation of the spinal cord (myelitis). Deficits can be temporary or permanent, the latter especially in the absence of treatment. Signs and symptoms usually follow a relapsing and remitting course, but occasionally can be progressive (monophasic). The signs and symptoms of NMOSD depend on the neurologic structures the disease affects, and, to some extent, the antibodies involved. However, NMO is not related to MS in the vast majority of cases and differs from MS substantially in terms of pathogenesis, clinical presentation, magnetic resonance imaging, cerebrospinal fluid findings, disease course, and prognosis. In consequence, NMO was in the past wrongly considered a clinical variant of MS. in patients with long-standing MS resulting in confluent spinal cord lesions mimicking the long spinal cord lesions typically seen in MS). Multiple sclerosis (MS) and NMO can be similar in clinical and radiological presentation, and MS may very rarely present with an NMO-like phenotype (e.g. In some cases, the etiology remains unknown ( idiopathic NMO). connective tissue disorders, paraneoplastic syndromes) or infectious diseases. Rarely, NMO may occur in the context of other autoimmune diseases (e.g. A subset of anti-AQP4-negative cases is associated with antibodies against myelin oligodendrocyte glycoprotein ( anti-MOG). In more than 80% of cases, NMO is caused by immunoglobulin G autoantibodies to aquaporin 4 ( anti-AQP4), the most abundant water channel protein in the central nervous system. A relapsing disease course is common, especially in untreated patients. Episodes of ON and myelitis can be simultaneous or successive. Neuromyelitis optica spectrum disorders ( NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve ( optic neuritis, ON) and the spinal cord ( myelitis). Multiple sclerosis, various autoimmune disordersĮculizumab, inebilizumab, satralizumab, rituximab, methylprednisolone, azathioprine, cellCept, mitoxantrone, methotrexate, intravenous immunoglobulin, cyclophosphamide Median: age 40 for AQP4-IgG, age 31 for MOG-IgG ĪQP4-IgG-positive, MOG-IgG-positive (recurrent, monophasic) Vision loss, sensory loss, weakness, bladder dysfunction Neuromyelitis optica (NMO), Devic's disease, Devic's syndrome Medical condition Neuromyelitis optica spectrum disorders
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