Devic's Syndrome (cont.)
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What are the symptoms of neuromyelitis optica?
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Individuals with neuromyelitis optica develop optic neuritis, which causes pain in the eye and vision loss, and transverse myelitis, which causes weakness, numbness, and sometimes paralysis of the arms and legs, along with sensory disturbances and loss of bladder and bowel control. Neuromyelitis optica leads to loss of myelin, which is a fatty substance that surrounds nerve fibers and helps nerve signals move from cell to cell. The syndrome can also damage nerve fibers and leave areas of broken-down tissue. In the disease process of neuromyelitis optica, for reasons that aren't yet clear, immune system cells and antibodies attack and destroy myelin cells in the optic nerves and the spinal cord.
How is neuromyelitis optica diagnosed?
Historically, neuromyelitis optica was diagnosed in patients who experienced a rapid onset of blindness in one or both eyes, followed within days or weeks by varying degrees of paralysis in the arms and legs. In most cases, however, the interval between optic neuritis and transverse myelitis is significantly longer, sometimes as long as several years. After the initial attack, neuromyelitis optica follows an unpredictable course. Most individuals with the syndrome experience clusters of attacks months or years apart, followed by partial recovery during periods of remission. This relapsing form of neuromyelitis optica primarily affects women. The female to male ratio is greater than 4:1. Another form of neuromyelitis optica, in which an individual only has a single, severe attack extending over a month or two, is most likely a distinct disease that affects men and women with equal frequency. The onset of neuromyelitis optica varies from childhood to adulthood, with two peaks, one in childhood and the other in adults in their 40s.
In the past, neuromyelitis optica was considered to be a severe variant of multiple sclerosis (MS) because both can cause attacks of optic neuritis and myelitis. Recent discoveries, however, suggest it is a separate disease. Neuromyelitis optica is different from MS in the severity of its attacks and its tendency to solely strike the optic nerves and spinal cord at the beginning of the disease. Symptoms outside of the optic nerves and spinal cord are rare, although certain symptoms, including uncontrollable vomiting and hiccups, are now recognized as relatively specific symptoms of neuromyelitis optica that are due to brainstem involvement.
The recent discovery of an antibody in the blood of individuals with neuromyelitis optica gives doctors a reliable biomarker to distinguish neuromyelitis optica from MS. The antibody, known as NMO-IgG, seems to be present in about 70 percent of those with neuromyelitis optica and is not found in people with MS or other similar conditions.
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