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Devic disease


Other Names for this Disease

  • Devic syndrome
  • Devic's neuromyelitis optica
  • Neuromyelitis optica
  • NMO
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Devic disease?

What are the signs and symptoms of Devic disease?

What causes Devic disease?

Is Devic disease inherited?

How is Devic disease diagnosed?

How might Devic disease be treated?

What is the long-term outlook for people with Devic disease?

What is Devic disease?

Devic disease, is an autoimmune condition that affects the spinal cord and optic nerves (the nerves that carry information regarding sight from the eye). In Devic disease, the body's immune system attacks and destroys myelin, a fatty substance that surrounds nerves and helps nerve signals move from cell to cell.[1] Signs and symptoms worsen with time and include optic neuritistransverse myelitis; pain in spine and limbs; and bladder and bowel dysfunction. The exact cause of Devic disease is unknown. Most affected people do not have other family members with the condition.[2] Currently there is no cure for Devic disease, but there are therapies to treat an attack while it is happening, to reduce symptoms, and to prevent relapses.[1]
Last updated: 12/5/2014

What are the signs and symptoms of Devic disease?

The most common signs and symptoms of Devic disease are optic neuritis and/or transverse myelitis. Optic neuritis (inflammation of the optic nerve) can cause pain and sudden, reduced vision in the affected eye. In most cases, only one eye is affected; however, some people may develop optic neuritis in both eyes at the same time.[2][3]

Signs and symptoms of transverse myelitis (inflammation of the spinal cord) include pain in the spine or limbs (arms and legs); mild to severe paralysis of the legs; paresthesias (abnormal sensations such as burning, tickling, pricking, or tingling) in the legs; and loss of bowel or bladder control. Depending on which section of the spinal cord is affected, breathing problems may be present, as well. Some affected people may also experience muscle spasms, a stiff neck, a general feeling of discomfort, headache, fever, and loss of appetite. Progression of transverse myelitis leads to full paralysis of the legs, requiring the patient to use a wheelchair.[2][4]

In rare cases, Devic disease can also affect the brainstem (the part of the brain connected to the spinal cord). This can lead to symptoms such as uncontrollable vomiting and hiccups.[3][4]
Last updated: 12/5/2014

What causes Devic disease?

In most cases, the exact cause of Devic disease is unknown. However, studies of affected nerves have improved our understanding of the disease process. In Devic disease, certain immune proteins (autoantibodies) attach themselves to specialized proteins in the spinal cord and optical nerve called "water channel proteins." The autoantibodies signal immune cells to attack, resulting in damage to myelin and the breakdown of healthy nerves and tissues.[5]

There is a strong link between Devic disease and a personal or family history of other autoimmune conditions. In fact, 50% of people with Devic disease are also affected by other autoimmune conditions and/or have family members with an autoimmune condition.[2]

There have been a few cases of Devic disease occurring in association with certain infectious conditions (e.g., syphilis, HIV, chlamydia, varicella, cytomegalovirus, and Epstein Barr virus). The nature of this link isn't clear. It is possible that certain infections may trigger Devic disease in peoeple who are predisposed to the condition.[5]
Last updated: 12/5/2014

Is Devic disease inherited?

Devic disease is not thought to be inherited in most cases, and only 3% of affected people have a family member with the condition.[2]
Last updated: 12/5/2014

How is Devic disease diagnosed?

A diagnosis of Devic disease is based on a physical examination; identification of characteristic signs and symptoms; and a variety of specialized imaging and laboratory tests. For example, magnetic resonance imaging (MRI scan) or computed tomography (CT scan) of the brain and spinal cord and optical coherence tomography (specialized pictures of the retina) can be used to locate areas of nerve damage. Special blood tests that are used to detect autoimmune conditions and/or examination of the cerebrospinal fluid may also be necessary to confirm a diagnosis.[3][2]  
Last updated: 12/5/2014

How might Devic disease be treated?

There is no cure for Devic disease, but there are therapies to treat an attack while it is happening, to reduce symptoms, and to prevent relapses. Doctors usually treat an initial attack of Devic disease with a combination of corticosteroid drugs to stop the attack and immunosuppressive drugs to prevent additional attacks. If frequent relapses occur, some people may need to continue a low dose of steroids for longer periods. Plasma exchange (plasmapheresis) is a technique that separates antibodies out of the blood stream and is used with people who do not respond to corticosteroid therapy. Pain, stiffness, muscle spasms, and bladder and bowel control problems can be managed with the appropriate medications and therapies. People with Devic disease may require the combined efforts of occupational therapists, physical therapists, and social services professionals to address their complex rehabilitation needs.[4]

The Transverse Myelitis Association provides additional information on the treatment options available for Devic disease. http://myelitis.org/symptoms-conditions/neuromyelitis-optica/acute-treatments-nmo/
Last updated: 12/5/2014

What is the long-term outlook for people with Devic disease?

The onset of Devic disease varies from childhood to adulthood, with two peaks: one in childhood and the other in adults in their 40s. Most people with Devic disease have a relapsing form of the disease and experience clusters of attacks months or years apart, followed by partial recovery during periods of remission (a decrease or disappearance of symptoms). Disability is cumulative, the result of each attack damaging new areas of myelin. In rare cases, Devic disease is characterized by a single, severe attack extending over a month or two, with little recurrence after the initial onset of symptoms. Some people are severely affected by Devic disease and can lose vision in both eyes and the use of their arms and legs. Most people experience a moderate degree of permanent limb weakness from myelitis. Muscle weakness can cause breathing difficulties and may require the use of artificial ventilation. The death of an individual with Devic disease is most often caused by respiratory (breathing) complications from myelitis attacks.[4]
Last updated: 12/5/2014

References
  1. NINDS Neuromyelitis Optica Information Page. National Institute of Neurological Disorders and Stroke (NINDS). 2007; http://www.ninds.nih.gov/disorders/neuromyelitis_optica/neuromyelitis_optica.htm. Accessed 11/18/2009.
  2. Neuromyelitis Optica. NORD. March 2012; http://rarediseases.org/rare-disease-information/rare-diseases/byID/479/viewFullReport.
  3. Christopher C Glisson, DO, MS. Neuromyelitis optica (NMO) and NMO spectrum disorders. UpToDate. November 2014;
  4. NINDS Neuromyelitis Optica Information Page. National Institute of Neurological Disorders and Stroke. June 2014; http://www.ninds.nih.gov/disorders/neuromyelitis_optica/neuromyelitis_optica.htm.
  5. Hazin H, Khan F, Bhatti MT. Neuromyeliits optica: Current concepts and prospects for future management. Current opinion in opthalmology. 2009;


Other Names for this Disease
  • Devic syndrome
  • Devic's neuromyelitis optica
  • Neuromyelitis optica
  • NMO
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.