Here we report for the first time on a severe case of classic neuromyelitis optica i. Symptoms include rapid onset of eye pain and vision loss, myelitis muscle weakness, numbness, and occasionally paralysis of the arms and legs with sensory disturbances and loss of bowel and. Neuromyelitis optica spectrum disorder nord national. Neuromyelitis optica nmo is a heterogeneous condition consisting of the inflammation and demyelination of the optic nerve optic neuritis and the spinal cord myelitis. Life expectancy of people with devic syndrome nmo and recent progresses and researches in devic syndrome nmo. What is the life expectancy of someone with devic syndrome. Neuromyelitis optica is an autoimmune disease that affects the spinal cord and optic nerves nerves that carry visual messages to and from the brain. Devics disease diagnosis an early diagnosis of devics disease is critical for the management of this condition and for quality of life. This case shows that in some rare instances, a clinical presentation suggestive of a neuromyelitis optica can be of paraneoplastic origin. Currently at least three different kinds are proposed based on the presence of autoantibodies thought to produce the disease.
Neuromyelitis optica devic s disease is a rare clinical syndrome of unilateral or bilateral optic neuritis on and transverse myelitis tm occurring within an 8week time interval. Your childs doctor will start by conducting a thorough physical examination, asking about any symptoms your child is experiencing, including when they started and how theyve eased or progressed over time. What is the life expectancy of someone with devic syndrome nmo. Dec 15, 2017 neuromyelitis optica nmo is a central nervous system disorder that primarily affects the eye nerves optic neuritis and the spinal cord myelitis. Nmo is also known as neuromyelitis optica spectrum disorder or devics disease. A diagnostic dilemma neuromyelitis optica nmo, also known as devic syndrome or devic disease is a rare clinical entity. Neuromyelitis optica pathogenesis and aquaporin 4 david j graber1, michael levy2, douglas kerr2 and william f wade standardized method for the detection of antibodies to aquaporin4 based on a highly sensitive immunofluorescence assay. Individuals with devics syndrome may have one attack that lasts. Life expectancy of people with devic syndrome nmo and recent progresses and researches in devic syndrome nmo world map of devic syndrome nmo view more toggle navigation. Devics neuromyelitis optica nmo or devics disease is a severe idiopathic immunomediated inflammatory demyelinating and necrotizing disease that predominantly involves the optic nerves and spinal cord. Devic syndrome or neuromyelitis optica is an autoimmune neurological condition characterized by relapsing symptoms of optic neuritis and transverse myelitis. Classically, it was felt to be a monophasic illness, consisting of episodes of inflammation of one or both. Neuromyelitis optica nmo is a heterogeneous condition consisting of the inflammation and demyelination of the optic nerve optic neuritis and the spinal cord. Devic s disease diagnosis an early diagnosis of devic s disease is critical for the management of this condition and for quality of life.
Devic s disease, also called neuromyelitis optica or nmo, is an immunological disorder that may be confused with multiple sclerosis ms. Apr 26, 2020 devics syndrome in a woman with systemic lupus erythematosus. The case of a patient who presented with a necrotic myelopathy and bilateral optic neuritis in association with a thymoma and circulating anticv2crmp5 antibodies is reported. Devic s syndrome in systemic lupus erythematosus and probable antiphospholipid syndrome a. Facebook is showing information to help you better understand the purpose of a page. Recent immunopathological evidences suggest that the target antigen is aquaporin4, the dominant water channel in the central. Sep 30, 2014 despite recent development of direct acting antivirals for treatment of hepatitis c, the current standard of care may still include pegylatedinterferon, which is associated with frequent and, at times, serious adverse events. Neuromyelitis optica genetic and rare diseases information. In neuromyelitis optica, the bodys immune system mistakenly attacks healthy cells and a substance called myelin in the spinal cord and eyes. Treatment was commenced including azathiaprine, intragram and prednisolone with gradual recovery of function. Devic s disease is also called neuromyelitis optica or nmo.
Nov 06, 2017 neuromyelitis optica is an autoimmune disease that affects the spinal cord and optic nerves nerves that carry visual messages to and from the brain. Her first attack was rightsided optic neuritis at age 10 years. Devics syndrome is characterized by inflammation of the optic nerve of the eye andor the spinal cord due to attacks by the immune system. A case of classic neuromyelitis optica devics syndrome. Optic neuromyelitis syndrome in brazilian patients. Neuromyelitis optica nmo is a central nervous system disorder that primarily affects the eye nerves optic neuritis and the spinal cord myelitis.
Neuromyelitis optica of devic nmo is a syndrome which combines transverse myelitis and optic neuritis. Association of neuromyelitis optic nmo with autoimmune disorders. A 45yearold woman with a history of hashimoto thyroiditis presented with a 4. Devics syndromelike phenotype associated with thymoma. Primary sjogrens syndrome presenting as neuromyelitis optica. These will vary from person to person some may only have one attack of symptoms and recover. Devics syndromelike phenotype associated with thymoma and. There is no proven effective treatment although relapses are commonly treated with corticosteroids and people with recurrent attacks may be managed with chronic immune. The discovery of a novel serum autoantibody termed nmoigg or aqp4ab in a subset of patients in 2004 has revived interest in neuromyelitis optica nmo. Nmo is also known as neuromyelitis optica spectrum disorder or devic s disease. Neuromyelitis optica nmo, also known as devics disease, is a rare condition that affects the spinal cord and the nerves of the eyes optic nerves. Introduction devics neuromyelitis optica is an uncommon but severe form of demyelinating disease characterized by acute attacks of optic neuritis and myelitis. People with neuromyelitis optica develop optic neuritis and transverse myelitis. Devic disease with brainstem lesions demyelinating.
Devics neuromyelitis optica treated with intravenous. Its not very common only about 4,000 people in the united states have it. Please use one of the following formats to cite this article in your essay, paper or report. It can cause a wide range of symptoms, such as weakness, blindness, nerve pain and muscle spasms. May 14, 2010 neuromyelitis optica nmo is an uncommon disease of the central nervous system cns that affects the optic nerves and spinal cord due to myelin loss. A repeat mri 4 months later not shown demonstrated complete resolution of changes with a normal. Devics syndrome in a woman with systemic lupus erythematosus. To report the clinical features and outcome of 24 brazilian patients with optic neuromyelitis syndrome onm. S ir, dr ferreira and colleagues have given an excellent description of a lupus patient with devics syndrome neuromyelitis optica, nmo but it seems that a fundamental issue of this case has not been answered. Neuromyelitis optica spectrum disorder and antimog syndromes. It tends to have a poor prognosis, probably due to the occurrence of necrosis within lesions. Was this patient suffering from nmo with inflammatory demyelinating disease targeting the optic nerves and spinal cord as described, or a genuine. The full text of this article is available in pdf format.
Neuromyelitis optica, or nmo, is a disease that affects your eyes and spinal cord. Neuromyelitis optica spectrum disorder and antimog. Neuromyelitis optica devic s syndrome is a disease of the cns that affects the optic nerves and spinal cord. Devics disease is a type of inflammatory demyelinating disease that affects the optic nerve and spinal cord. Devics disease, also called neuromyelitis optica or nmo, is an immunological disorder that may be confused with multiple sclerosis ms. Devic s syndrome also known as devic s disease and neuromyelitis optica is a rare, chronic, inflammatory and demyelinating disease of the central nervous system cns which resembles multiple sclerosis in several ways. Neuromyelitis optica spectrum disorder nmosd, also known as devic disease, is a chronic disorder of the brain and spinal cord dominated by inflammation of the optic nerve optic neuritis and inflammation of the spinal cord myelitis. Despite recent development of direct acting antivirals for treatment of hepatitis c, the current standard of care may still include pegylatedinterferon, which is associated with frequent and, at times, serious adverse events. Devics syndrome in systemic lupus erythematosus and probable. Women with neuromyelitis optica suffer from adverse pregnancy outcomes and high relapse rates during pregnancy and the postpartum period. Neuromyelitis optica nmo is an uncommon disease of the central nervous system cns that affects the optic nerves and spinal cord due to myelin loss symptoms include rapid onset of eye pain and vision loss, myelitis muscle weakness, numbness, and occasionally paralysis of the. Neuromyelitis optica symptoms and causes mayo clinic. Devics syndrome in systemic lupus erythematosus and. It occurs when your bodys immune system reacts against its own cells in the central nervous system, mainly in the.
Devics syndrome neuromyelitis optica patient worthy. Devics disease is characterised by attacks of acute optic neuritis on, usually in both eyes. Liu, department of ophthalmology and visual sciences, the chinese university of hong kong, prince of wales hospital, shatin, hong kong. The optic nerve andor the spinal cord may be affected. Devic disease or neuromyelitis optica nmo is characterized by the association of an acute or subacute demyelinating myelitis and unilateral or bilateral optic neuritis. Wingerchuk dm, lennon va, lucchinetti cf, pittock sj, weinshenker bg. Neuromyelitis optica pathogenesis and aquaporin 4 david j graber1, michael levy2, douglas kerr2 and william f wade standardized method for the detection of antibodies to aquaporin4 based on a highly sensitive immunofluorescence assay employing recombinant. Devics syndrome, also known as neuromyelitis optica, is an immune disorder that is sometimes confused with multiple sclerosis ms. Neuromyelitis optica devics disease is a rare clinical syndrome of unilateral or bilateral optic neuritis on and transverse myelitis tm occurring within an 8week time interval. Devic s disease is characterised by attacks of acute optic neuritis on, usually in both eyes. The term devics neuromyelitis optica was subsequently extended to cases. Attacks involving both optic nerves and medulla spinalis were recorded during the ensuing years.
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