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Neuromyelitis optica , also known as neuromyelitis optica spectrum disorder , is a rare inveterate redness of the central queasy system mediated by autoantibody . antecedently considered a discrepancy of multiple induration , it is now a trenchant disease . In most of the patients , antibodies are produced against astrocyte aquaporin 4 ( AQP4 ) water channel that leads to recurrent exacerbations of ocular neuritis and myelitis . Some patients may have liaison of the brain-stem and brain do postrema syndrome or diencephalic syndrome leading tohiccups , sickness , vomiting , narcolepsy , muscle affection . patient also suffer from symptom of fatigue , pain , headache , eternal rest disorders , anddepression.(1 )
The beggarly age of disease onset is around 39 twelvemonth and women are more frequently affected by neuromyelitis optica , specially in cases of AQP4 seropositivity , in which the female person to male proportion can be 10:1 . Even after discussion , there is incomplete recovery from relapsing and remission from the disease is rarefied . recidivate neuromyelitis optica answer for for about 80 - 85 % of all cases and each lapse leads to increasing neurologic handicap . The prognosis is poorer with increase death rate rate in patients with farsighted terminus immunosuppressive therapy.(1 )
What Is The Best Treatment For Neuromyelitis Optica?
The treatment of neuromyelitis optica bet on whether the treatment is for acute attack or for long term therapy of the patient . Acute attacks of neuromyelitis optica are finagle with systemic corticoid or therapeutic plasm interchange . High dose IV methylprednisolone ( 3 - 5 daytime ) is the first line of discussion when the diagnosis of neuromyelitis optica is confirmed or suspected . Early pulse therapy is of the essence for minimize the loss of axons during the approach . If no betterment or worsening symptom are find with corticosteroid therapy , then sanative blood plasma commutation ( 5 - 7 cycles ) is the handling of choice . Studies have show superior outcome with hyperkinetic syndrome on therapeutic neuromyelitis optica to glucocorticoids than with glucocorticoids alone.(2 )
Since the course of neuromyelitis optica is relapsing in nature , tenacious full term immunosuppressive therapy is important for disease management and good medical prognosis . The long term therapy for almost 5 years has been suggested for the bar of disease . However , the duration of treatment in each patient role should be individualize reckon on the treatment efficacy , the clinical course of instruction of the disease and associated complications . Low dose prednisolone monotherapy was recover in effect in trim back relapsing of the disease ; however , it is used as a combination therapy with Imuran or cyclosporine.(2 )
Azathioprine , a purine parallel , has an antiproliferative and immunosuppressive force . It take on 3 - 6 months to be fully effective , so ab initio , oral Meticorten has to be render and can be taper off when azathioprine is fully effectual . ivory marrow imprint and pancytopenia is a mutual side effect of the drug . Rituximab causes a step-down in antibody production and is instantly against CD20 and B lymphocytes . The side force include pruritus , rash , headache , or fever - related to IV extract . Azathioprine and rituximab are the two most commonly used drug for immunosuppressive therapy in neuromyelitis optica . Some relative cogitation have shown rituximab to be more superior to azathioprine ; therefore , rituximab is currently the most in force treatment for neuromyelitis optica.(1 )
Mycophenolate mofetil is another immunosuppressant that is used in the treatment of neuromyelitis optica . Mycophenolate mofetil has fewer side outcome than azathioprine with interchangeable efficaciousness . Intravenous immunoglobulin has also been feel effective in the treatment of neuromyelitis optica ; however , there are very few studies on the efficaciousness of the drug . Methotrexate has also been used as a intervention choice in patients who do not respond to first - line of products treatment and have gravid side personal effects with other therapies . Multiple sclerosis medications such as interferon genus Beta , fingolimod , alemtuzumab , natalizumab , glatiramer acetate , dimethyl fumarate are found ineffective in the management of neuromyelitis optica and have been known to exasperate the disease ; therefore , these are contraindicated in the treatment of neuromyelitis optica.(1 )
There are emerge therapies that are currently undergoing clinical test , such as eculizumab , tocilizumab , and C1 - esterase inhibitor . These drugs have show promising results in clinical test and hopefully , they will be available as new drugs for the treatment of neuromyelitis optica in future.(2 )
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