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Neuromyelitis optica is an autoimmune disease of the central nervous organization that predominantly affects the optic brass and the spinal cord . Neuromyelitis optica is now know as neuromyelitis optica spectrum disorder after the seropositivity of AQP4 - IgG ( aquaporin 4 immunoglobulin G ) antibodies . Earlier to that it was view to be a variant of multiple induration . Neuromyelitis optica spectrum disorder be roughly 20 % of all demyelinate disorders in India with a prevalence of 2.6 per 100,000 population . The age of onrush of the disease is in the third and fourth tenner of life story and is more prevalent in females than in males , particularly in the retrogress disease.(1 )
Approximately 70 - 80 % of all cases are powerfully linked to anti - AQP4 - IgG that are produce by plasma cellphone by an unsung mechanism . It is deserving noting that anti - AQP4 - IgG 33 % of attack occur after a inoculation or a fever . familial studies have picture that neuromyelitis optica is associated with an aberrance in HLA - DRB1(03 ) factor in Amerindic universe . Although most of the cases of neuromyelitis optic are sporadic , a few familial cases have also been noted.(1 )
How Dangerous Is Neuromyelitis Optica & Is It Contagious?
Neuromyelitis optica more and more causes vision , motor , sensory and bladder impairment due to recurrent approach . Most of the attacks worsen over days and convalescence takes place in several week to month with significant impairment left by each attack . The bad outcome is noted citizenry with a greater number of reversion within the first two yr of the disease , harshness of the first attack , disease onset at an older eld , seropositivity with AQP4 - IgG autoantibody , and when it is colligate with other autoimmune diseases.(2 )
Neuromyelitis optica is associated with high mortality rates due to neurogenic respiratory failure that happen when cervical wound put out into the brainstem or when primary brainstem lesions extend further . The deaths associated with neuromyelitis optica ranges from 25 - 50 % in North American , Brazilian and French West Indies people.(2 )
The greater awareness of the disease in the late yr , access code to anti - AQP4 - IgG testing and long term treatment with immunosuppressive has greatly improved the mortality rate of the disease that has improved from 30 % at 5 age to 9 % at 6 years.(1 )
The AQP4 - IgG autoantibody may be see a marking for the course of action of the disease and its prospect . Retrospective subject field have linked seropositivity of the antibody to the pitiable visual effect and disease growing . Some studies have suggested the antibody seropositivity to greater reversion within one year and some studies with no farseeing terminus sequelae associated with the seropositivity.(1 )
However , the disease is not contagious as it is an autoimmune disease .
Presentation Of Neuromyelitis Optica
The most uncouth feature of neuromyelitis optica include knifelike severe optic neuritis and lengthwise extensive transverse myelitis ( LETM ) . Longitudinally all-encompassing transverse myelitis is defined as longitudinal corduroy lesions present in more than 3 vertebral segment . Mostly the first attempt is either of ocular neuritis or transverse myelitis ; both the symptoms of optic neuritis and transverse myelitis are seen in about 15 - 40 % cases . Although it is difficult to speciate between optic neuritis in neuromyelitis optica and multiple sclerosis , the optic neuritis associated with neuromyelitis optica is severe with impaired sight , concurrent isobilateral affair of middle in rapid succession .
Spinal corduroy lesion lead to total paralysis of all the four extremities or of two extremities along with sensory amour and bladder dysfunction.(1 )
Other presenting symptoms include neuropathic pain , tonic spasm , Lhermitte ’s sign , seizure , hyposmia , encephalomeningitis , posterior reversible encephalopathy syndrome , cognitive disfunction , myeloradiculopathy , ophthalmoplegia , myocarditis , and brawny edema . brain stem involvement let in hiccough , vomiting , narcolepsy , increase daylight somnolence , hypothermia , and even respiratory failure . Non - neurological manifestations of neuromyelitis optica admit placentitis with the risk of miscarriage , internal otitis , and gastritis . Approximately 30 - 40 % of patients with neuromyelitis optica have associated autoimmune disorders includingSjogren ’s syndrome , systemic lupus erythematosus , myasthenia gravis , autoimmune thyroid disease , autoimmune encephalitis , autoimmune - mediate vitamin B12 deficiency , and others.(1 )
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