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Definition of Devic Syndrome
Devic's syndrome is a very rare autoimmune central nervous system disorder characterized by transverse myelitis and optic neuritis (in which inflammation of the optic nerve causes loss of vision and eye pain). It is thought about a special type of multiple sclerosis (MS) with a extreme and rapid coursework. The disorder affects the optic nerve and the nerves in the spinal cord. In Devic's syndrome, the fatty sheath that protects these nerves is lost. Patient may experience vision loss and various degrees of paralysis, as well as incontinence. The disorder is closely linked with MS and lupus, but usually appears before any signs of MS are noted. If an isolated illness episode affecting the spinal cord and optic nerve occurs after an infection or common cold, it is thought about a post-infectious acute demyelinated encephalomyelitis (ADE) than Devic's syndrome.
Symptoms of Devic Syndrome
Patients with Devic Syndrome 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. Devic Syndrome 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 Devic syndrome, 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.
Causes of Devic Syndrome
Devic's disease has been associated with lots of systemic diseases, based on historial proof of some Devic's disease patients with a comorbid condition. Devic's disease conditions include: collagen vascular diseases, autoantibody syndromes, infections with varicella-zoster virus, Epstein-Barr virus, and HIV.
?Diagnosis of Devic Syndrome
The Mayo Clinic proposed a revised set of criteria for diagnosis of Devic's disease in 2006. The new guidelines require two absolute criteria plus at least two of three supportive criteria.
Absolute criteria: Optic neuritis, Acute myelitis
Supportive criteria: Brain MRI not meeting criteria for MS at disease onset, Spinal cord MRI with contiguous T2-weighted signal abnormality extending over three or more vertebral segments, indicating a relatively large lesion in the spinal cord.
?Treatment of Devic Syndrome
Currently no standard treatment for Devic's syndrome is available. Usually, treatment is symptomatic and supportive. Corticosteroids may be advised. Treatment for ADE may include corticosteroids, intravenous immunoglobulin, and intravenous methylprednisolone??
?Prognosis of Devic Syndrome?Devic's syndrome is deadly in lots of patients. Some ADE patients accomplish complete or very complete recovery while others may have residual deficits. Some extreme cases of ADE may be deadly.??
?Prevention of Devic Syndrome?Consult with your doctor.
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Last Updated on Monday, 24 December 2012 18:31